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Evaluation of the Child with Cerebral Palsy
Abstract
Cerebral palsy (CP) is a common problem, occurring in about 2 to 2.5 per 1000 live births. The diagnosis of CP is based upon a history of abnormal motor development that is not progressive coupled with an examination (e.g. hypertonicity, increased reflexes, clonus) "placing" the lesion in the brain. In order to establish that a brain abnormality exists in children with CP that may, in turn, suggest an etiology and prognosis, neuroimaging is recommended with magnetic resonance imaging preferred to computed tomography. Metabolic and genetic studies should be obtained if there are atypical features in the history or on the examination. Detection of a brain malformation in a child with CP might suggest an underlying genetic or metabolic etiology. As cerebral infarction is high in children with hemiplegic CP, diagnostic testing for coagulation disorders should be considered. However, there is insufficient evidence at present to be precise as to what studies should be ordered. An electroencephalogram is not recommended unless there are features suggestive of epilepsy or a specific epileptic syndrome. As children with CP may have associated deficits of mental retardation, ophthalmologic and hearing impairments, speech and language disorders and oral-motor dysfunction, screening for these conditions should be part of the initial assessment.
... Early diagnosis is involved with the use of neuroimaging, and standardized neurological and motor assessments that can indicate congruent abnormal findings revealing cerebral palsy [4]. Cerebral palsy diagnosis is on the basis of a history of abnormal motor development and an examination "placing" lesions in the brain [5]. ...
... As previously reported, MRI is more recommended to utilized combined with neuroimaging in contrast with CT [5]. MRI benefits in high-risk term infants [20]. ...
Objective
This study focused on analyzing the clinical value and effect of magnetic resonance imaging plus computed tomography (MRCT) and CT in the clinical diagnosis of cerebral palsy in children.
Methods
From February 2021 to April 2023, 94 children diagnosed with cerebral palsy were selected from our hospital for study subjects. These patients were divided into CT and MRI groups, with CT examination given to the CT group and MRI examination given to the MRI group. The positive rate of the two examination methods in the diagnosis of cerebral palsy was compared, different imaging signs in two groups of children with cerebral palsy were compared, and the diagnostic test typing results between two groups were further analyzed.
Results
The diagnostic positivity rate of the children in the MRI group was 91.49%, which was significantly higher than that of the children in the CT group (70.21%) (P < 0.05). In both groups, encephalomalacia, bilateral frontal subdural effusions, and gray-white matter atrophy of the brain were the main signs, and the difference in the proportion of these three imaging signs between the two groups was not significant (P > 0.05). Differences between the two groups examined for cerebral palsy subtypes were not significant (P > 0.05).
Conclusion
The positive rate of pediatric cerebral palsy examined by MRI is higher than that of CT diagnosis, but the clinic should organically combine the two to further improve the detection validity and accuracy.
... CP has been classified by number and location of affected limb, its either monoplegic, diplegic, or quadriplegic, it's also classified according to the type of motor disability, such as hypotonia, hypertonia, dystonia, and ataxia (7,8).The diagnosis of CP depends on the clinical information about abnormal motor development that is not progressive in nature, associated with (e.g. hyper tonicity, increased reflexes, clonus) on examination pointing in to an intracranial location (9,10). The nature of brain lesion may be vascular, infective, inflammatory, or traumatic and the time of insult may be in prenatal, perinatal or postnatal period (9), in developing countries, 50% of cases occur in preterm infants, while in western countries the prevalence of CP in premature infants declined over the past decade (12). ...
... hyper tonicity, increased reflexes, clonus) on examination pointing in to an intracranial location (9,10). The nature of brain lesion may be vascular, infective, inflammatory, or traumatic and the time of insult may be in prenatal, perinatal or postnatal period (9), in developing countries, 50% of cases occur in preterm infants, while in western countries the prevalence of CP in premature infants declined over the past decade (12). ...
Background
Cerebral palsy (CP) is a major cause of the disability in children. It is considered a neurological disease occurs due to a non-progressive brain injury or anomaly that occurs while the brain is developing.
Intracranial imaging provides a window to see the brain lesion and potentially, provide an insight into the pathogenesis of CP.
Objectives: To reveal the radiological changes of brain using CT scan in different clinical types of CP and in those CP children with functional impairments.
Material and methods
Sixty eight children with previous diagnosis of cerebral palsy were scanned with computed tomography (Philips, brilliance 64) for brain without contrast at Azadi Teaching Hospital/Kirkuk city, from February 2013-July 2016. Clinical information was obtained from the documentation of pediatricians. The images were reviewed by two board certificate radiologists with at least 6 years of experience.
Result: Sixty-eight patients were included in the study, 42 (61.8%) females and 26 (38.2%) males with female to male ratio 1.6:1 and an overall mean age at presentation was 12 months. Most (79.3%) children with cerebral palsy had abnormal neuroradiological findings, Diffuse brain atrophy predominantly involving the cortical –subcortical grey matter with and without white matter hypodensities which indicate grey matter injury (35.4%) was the most common finding and it was more in the pyramidal CP (100%), followed by white matter atrophy and hypodensities with or without ventriculomegaly which indicate white matter injury in (23.5 %) which was the most common CT finding in Cerebeller CP (50%), congenital malformations found in (8.8 %) which include pachygyria, and Dandy-Walker malformation, entirely seen in quadriplegic type, the focal vascular brain insult seen in (5.9%) occurred only in diplegic type, and the least was ventriculomegaly labeled as miscellaneus ( 2.9 %) occurred only in quadriplegic CP. CT scan was normal in (20.7%), predominantly in Cerebeller type. There were significantly more patients with abnormal CT findings among CP children suffering from convulsion (p>0.05) than those without convulsion, this was not true in those with microcephaly (p<0.05).
Conclusion:
CT scan brain is a good modality for detection of structural brain abnormality in cerebral palsy (CP) cases. There was significant correlation between the topographic distribution of motor deficit and brain CT findings. There were significantly more patients with abnormal CT findings among CP children suffering from convulsion but not microcephaly.
... Cognitive disability is seen more frequently in children with severe CP, and is also accompanied with evidence of cortical abnormalities on imaging. 17 Our finding is supported by evidence presented by Dourado et al, 18 who found that the risk of having dental caries increases with the severity of intellectual impairment. This is proven to affect negatively the quality of life of patients with CP. 19 The main reason seems to be that cognitive disability makes the child's cooperation in brushing and caring for their teeth and oral health more challenging for the parents. ...
Background: Epilepsy is a common neurological disorder in childhood. However, there have been limited studies on its impact on the oral health of affected children. Our study aimed to assess the oral health of children with epilepsy in the city of Jeddah, Saudi Arabia, as perceived by their mothers.
Methods: We conducted a cross-sectional study in three hospitals. We included children 2–18 years old with physician-confirmed epilepsy diagnosis. We assessed parental perception of dental status and need for dental care using a standardized questionnaire that was completed by the mothers. To adjust for potential confounding variables, we used univariate and multivariate logistic regression.
Results: We included 96 children with epilepsy in our study. Their mean age was 6.4±3.4 years. In 55.2% (n=53), dental status was rated as bad, and in 84.4% (n=81) a need for dental care was expressed. Cerebral palsy (OR 5.06, 95% CI 1.28–19.99; P=0.021), motor disability (OR 6.41, 95% CI 1.12–36.73; P=0.037), referral from a pediatric neurology clinic to a dentist (OR 10.755, 95% CI 3.290–35.151; P<0.001), and irregular brushing of teeth (OR 5.397, 95% CI 1.536–18.961; P=0.009) were significantly associated with increased risk of perceived bad dental status. Perception of the child as being overweight (OR 0.117, 95% CI 0.034–0.400; P=0.001) was significantly associated with decreased risk of perceived bad dental status. Motor disability (OR 5.73, 95% CI 1.64–20.04; P=0.006) was significantly associated with increased parental expression of need for dental care.
Conclusion: In most children with epilepsy, perceived dental status was bad and there was a high expressed need for dental care. Interventions to improve the dental health of children with epilepsy should focus on those with cerebral palsy and motor disability.
... While the symptoms may get more noticeable over the first few years of life, the underlying problems do not worsen over time (18) According to characteristics of the studied children, the current study revealed that, more than half of children were boys while the rest of them were girls. This finding was in an agreement with (19) in his study of coping parents toward their child with cerebral palsy, there high percent for boys than girls and contradicting with (20) who mentioned that cerebral palsy occurs in both sexes equally. ...
... The more severe the degree of neurological stroke was and, as a result, cognitive and motor deficits, the higher the risk of developing dental diseases (8)(9)(10)(11). Inability to maintain oral hygiene, difficulty in chewing and swallowing, excessive drooling (sialorrhea), recurrent regurgitation and vomiting, insufficient calcium intake, vitamin D deficiency, use of antiepileptic medications are only a part of the factors leading to the onset and development of dental pathologies in children with CP (12)(13)(14)(15)(16). Along with caries, gingivitis is observed in the vast majority of children with CP (17)(18)(19). ...
Dental diseases occur in children with cerebral palsy three times higher than in healthy children. Low values of the unstimulated salivation rate (<0.3 ml per minute), pH and buffer capacity, changes in enzyme activity and sialic acid concentration, as well as increased saliva osmolarity and total protein concentration, which indicates impaired hydration, are the factors in the development of a gingiva disease in case of cerebral palsy. This leads to increased bacterial agglutination and the formation of acquired pellicle and biofilm, leading to the formation of dental plaque. There is a tendency toward an increase in the concentration of hemoglobin and a decrease in the degree of hemoglobin oxygenation, as well as an increase in the generation of reactive oxygen and nitrogen species. Photodynamic therapy (PDT) with the use of photosensitizer methylene blue improves blood circulation and the degree of oxygenation in periodontal tissues, as well as eliminates a bacterial biofilm. Analysis of back diffuse reflection spectra makes it possible to conduct non-invasive monitoring determine tissue areas with a low level of hemoglobin oxygenation for precision photodynamic exposure.
Aim
To improve the effectiveness of phototheranostics methods using, namely PDT with simultaneous optical-spectral control, for the treatment of gingivitis in children with complex dental and somatic status (cerebral palsy).
Methods
The study involved 15 children (6-18 y.o.) with various forms of cerebral palsy, in particular, spastic diplegia and atonic-astatic form and with gingivitis. The degree of hemoglobin oxygenation was measured in tissues before PDT and on the 12th day. PDT was performed using laser radiation (λ = 660 nm) with a power density of 150 mW/cm ² with a five-minute application of 0.01% MB. The total light dose was 45 ± 15 J/cm ² . For statistical evaluation of the results, a paired Student's t-test was used.
Results
The paper presents the results of phototheranostics using methylene blue in children with cerebral palsy. An increase in the level of hemoglobin oxygenation from 50 to 67% ( p < 0.001) and a decrease in blood volume in the microcirculatory bed of periodontal tissues were shown.
Conclusion
Photodynamic therapy methods with application of methylene blue make it possible to assess the state of the gingival mucosa tissue diseases objectively in real time, and to provide effective targeted therapy for gingivitis in children with cerebral palsy. There is a prospect that they can become widely used clinical methods.
... (14) If the patient faces difficulties in skills of communicating that make use of organizing, problem-solving, memory skills as well as perception then it is termed as cognitive communication disorder. (15) (16) For help with these disorders and problems, the patient should be referred to a speech language pathologist who works in order to improve their vocabulary, conversation, fluency, pronunciation, listening, word formation, conversation and stimulating their oral and neck muscles. (17) (18) This study aims to describe the development patterns of language in young children aged 2-4 years, suffering from cerebral palsy. ...
One of the frequently occurring disabilities of early age is Cerebral Palsy. Impairments in speech, language and communication usually co-occur with CP. These arise from the auditory, sensory and visual disorders that accompany CP. Delay or impairments in the development of language in cerebral palsy can lead to different speech and language disorders. Objectives: Evaluating the development of language in children with cerebral palsy between 2-4 years of age. Methods: A cross-sectional study design was utilized. Data was collected from different healthcare centers, clinics and hospitals. Convenient-based sampling technique was used. Children with co-morbid conditions with CP were not included in the study sample. The assessment tool used for evaluation of language development was ‘PORTAGE – early education program.’ The language related questions of PEEP were included as the tool for data collection. The data for 108 patients of cerebral palsy between 2-4 years of age was compiled. The results have been divided into demographics and the levels of portage i.e., 0-1 years, 1-2 years, 2-3 years and 3-4 years. The receptive as well as expressive language has been evaluated for these children. Total 70 questions have been added from the portage guide for up to 4 years of age. Results: It is observed that a little more than half of the patients included in the study experience varying degrees of language difficulties. These difficulties are an accumulation of receptive as well as expressive impairments. On the contrary, a little under half of the patients (46.38%) have language abilities developed according to their age. Early identification of language impairment in children with Cerebral Palsy can lead to early intervention. This study evaluates the language development in young patients with CP. The results reveal the percentage of patients with language impairments. Hence, the study encourages early evaluation and treatment of language disorders in this population. Conclusion: The most vital period of language development ranges till 5 years of age. So, 2-4 years is an important period of time for language development in an individual. An in-depth assessment of speech, language, cognitive and motor skills should be conducted during this time for CP children. Keywords: Cerebral Palsy, Portage Early Education Program, Language Development, Language Impairment.
... According to Stokes (1998), abnormalities could date from before birth (prenatal), or during birth (perinatal) but occurs after birth (postnatal) in the majority of CP cases. The diagnosis of CP is based on a history of abnormal motor development that is not progressive coupled with examination that helps in locating the specific site of the lesion in the brain (Russman and Ashwal, 2004). The observation of the form of CP, medical history of the mother and child and onset of the disorder can reveal the cause of CP (Nucleus Catalog Medical Reference Library, 2003). ...
Background: Cerebral palsy (CP) is a major cause of childhood disability. The objective of this study was to review the causes, types, complications, demographic and clinical characteristics of children with CP over a period of ten years as seen in the physiotherapy (PT) department of the University of Nigeria Teaching Hospital, Ituku-Ozalla Enugu, Nigeria. Methods: A retrospective study that reviewed a total of 497 case folders of children with CP. Ethical clearance was obtained from the Health Research Ethics Committee of the University of Nigeria Teaching Hospital, Enugu. The case records were randomly selected, a data retrieval form developed through a two-stage process was used to obtain the required information. Data was analyzed using descriptive statistics and Chi-square tests. Level of significance was set at 0.05. Results: Four hundred and ninety-seven cases of children with CP were reviewed. Birth asphyxia (29.9%) and jaundice (28.7%) were the leading causes of CP while spastic CP (56.7%) was the most common type. Motor disorder (67.0%) was the predominant clinical presentation. There was a significant association between type of CP and each of cause (X2 =28.303, p = 0.001), and duration of PT treatment (X2 =31.784, p < 0.0001). Conclusion: It was concluded that Birth asphyxia and jaundice are the major cause of CP in this environment. There is a significant association between type of CP and the cause of CP as well as the duration of PT treatment received.Keywords: Review; Cerebral Palsy; Hospital
... Cerebral palsy (CP) is the major developmental disability limiting function in children due to a defect or lesion that occurs while the brain is under development in the prenatal, perinatal, or postnatal period (Russman & Ashwal, 2004). CP describes a group of movement disorders with various types of motor dysfunctions causing functional activity limitations that are attributed to non-progressive disturbances (Burtner, Woollacott, Craft, & Roncesvalles, 2007;Woollacott & Shumway-Cook, 2005). ...
The present study aimed to verify whether hippotherapy once or twice a week has a different effect on gross motor function and functional performance in children with cerebral palsy. This trial had 20 children (age 2 to 5 years and 11 months) with cerebral palsy who were assigned to the groups once a week (n= 9) or twice a week (n= 11) and underwent 30 minute hippotherapy sessions for 16 weeks. The instruments used were the Gross Motor Function Measure and the Pediatric Evaluation of Disability Inventory, assessed at baseline and after 16 weeks. A significant time effect was observed for both groups without significant interactions between groups. Hippotherapy improved the gross motor function and functional performance of children with cerebral palsy, regardless of the weekly frequency of the sessions. This result has relevant clinical implications since hippotherapy has a high cost, and the prescription of weekly sessions could make this treatment more available for this population.
... La epilepsia de niños con PC tiene unas características diferenciales: mayor incidencia de epilepsia con inicio dentro del primer año de edad, antecedentes de convulsiones neonatales, estatus epiléptico, necesidad de politerapia y tratamiento con fármacos antiepilépticos de segunda línea. 9 Además, estos pacientes tienen con más frecuencia crisis focales, crisis reflejas, síndromes epilépticos graves y diferentes tipos de convulsiones a lo largo de la evolución de su epilepsia. 10 La realización de un EEG estaría indicado ante la sospecha de crisis de posible origen epiléptico, y el inicio de tratamiento se valorará de forma individualizada en cada caso en función del riesgo de recurrencia de las mismas. ...
Introducción:
La parálisis cerebral es la causa más frecuente de discapacidad infantil. Los niños con PC van a asociar, en la mayoría de los casos, además de los problemas neurológicos, otros trastornos no neurológicos que serán más frecuentes a mayor grado de afectación de la PC.
Objetivo:
Abordar las manifestaciones clínicas del niño con parálisis cerebral y su manejo diagnóstico y terapéutico con el fin de facilitar en un único documento un enfoque integral de estos pacientes.
Desarrollo:
Se realiza una revisión de las manifestaciones clínicas del niño con parálisis cerebral incluyendo trastornos neurológicos, problemas ortopédicos, trastornos digestivos, problemas respiratorios, salud ósea, problemas visuales y auditivos, trastornos urológicos y sexuales, salud bucodental, sialorrea, trastorno del sueño y dolor.
Conclusiones:
El abordaje del paciente con PC ha cambiado en los últimos años siendo fundamental la atención por un equipo multidisciplinar especializado en los diferentes problemas que presentan estos niños. Sin embargo, desde nuestra experiencia parece fundamental la figura de un pediatra conocedor de todos los problemas asociados que coordine todo el seguimiento.
... gestasyonel haftadan daha erken doğum öyküsü varsa ve muayenede nörolojik patoloji saptanırsa görüntüleme yapılmalıdır. [21] Görüntüleme çalışmaları, asfiktik yenidoğanlarda doku hasarlanması, intrakranial kanamaların varlığı ve derecesi, santral sinir sistemi (SSS) gelişimsel anomalilerinin varlığı hakkında bilgi verir. ...
... Children with milder cases of CP may simply need some physical therapy to treat their situation. [16] In more severe cases, it may be utilized next to other management or medications. Start physical therapy as early as possible typically provides children the greatest probability of progress. ...
Background: To assess the effects of injection of citicoline in combination with physiotherapy versus physiotherapy alone, to advance the functional outcome in paediatric patients with cerebral palsy. Subjects & Methods: A total of 150 paediatric patients diagnosed with cerebral palsy were incorporated into the study. The average age of the patient included in the study was found to be 36 months. All the patients enrolled in the study were referred to the paediatric neurological department. Results: The mean age of the patient was 36 10 months. Out of the 150 total patients, 60% were boys and 40% were girls. When the percentage of improvement was compared between the groups, the study group was established to be statistically significant. (P = 0.02). Conclusion: Citicoline is efficient and secure in the treatment of gross motor function combination with physiotherapy in patients with cerebral palsy. Physical therapy is frequently the first move in managing cerebral palsy.
... The observed dysfunctions in the present sample are likely related to spasticity and oral motor difficulties, which are linked to pyramidal tract damage and are associated with reduced nutritional intake [43][44][45][46][47][48]. Therefore, it is not surprising that the children in the SCP group were observed to display compensatory strategies while eating. ...
Dysphagia and resulting pulmonary sequelae are frequently observed in children with spastic cerebral palsy (SCP). However, physiological evidence regarding airway protective behaviors (specifically swallowing and cough function) in these children is sparse. The aim of this investigation was to quantify specific feeding, swallowing, and cough impairments in children with SCP compared to controls. Eleven children with SCP (mean age: 7 ± 2 years; GMFCS: I–V; MACS: I–V) and 10 age-matched controls participated. Clinical feeding and swallowing performance was evaluated with the dysphagia disorder survey (DDS) using standardized liquid, puree, and chewable solid consistencies. Suprahyoid muscle activity and respiratory–swallow patterns were assessed with simultaneous surface electromyography and respiratory inductance plethysmography as children swallowed the various consistencies. Voluntary cough airflow measures were also obtained. Nonparametric tests were used for group comparisons and correlational analyses. Compared to controls, children with SCP demonstrated more signs of clinical feeding and swallowing impairment (p < 0.0001, η² = 0.771), heightened suprahyoid muscle activity for puree (p = 0.014, η² = 0.305) and chewable solids (p = 0.001, η² = 0.528), more frequent post-swallow inhalation across liquid (p = 0.005, η² = 0.401), puree (p = 0.014, η² = 0.304), and chewable solids (p = 0.035, η² = 0.223), and lower cough volume acceleration (p = 0.019, η² = 0.289). Post-swallow inhalation for chewable solids was correlated with the DDS Part 1 (rs = 0.734, p = 0.010), DDS Part 2 (rs = 0.610, p = 0.046) and the DDS Total scores (rs = 0.673, p = 0.023). This study is the first to provide evidence of specific physiological deficits of both swallowing and voluntary cough in children with SCP. Potential hypotheses explaining these deficits and implications for physiologically driven management are explored.
... : Type of cerebral seizures.Because ofbilateral corticobulbar dysfunction in many CP syndromes, anarthritic or dysarthric speech caused by oromotor dysfunction is common[11]. Here mutism was considered as an autisticfeature when it was accompanied by no compensatory alternative modes ofcommunication such as gestures. ...
Background and Aim: Cerebral Palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that is attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain. Hence the present study was planned with the sole objective to study the clinical profile of patients of cerebral palsy and to assess the spectrum of functional disability. Materials and Methods: A total of 100 consecutive children diagnosed as cerebral palsy attending the pediatric department and Rehabilitation center were included in the study. Participants were examined forbehavioral problems like Pervasive Developmental Disorders (PDD) and ADHD. Complete evaluation of a child with CP included an assessment of associated deficits like vision, speech and hearing, oromotor evaluation, epilepsy, and cognitive functioning. Results:Out of 1000 cases studied, spastic cerebral palsy was the commonest physiological type of cerebral palsy (82%). atonic, athetoid, and dystonic types formed the rest of the 18% of cases. Among the spastic group, quadriplegia was the commonest topographical type (48%) followed by diplegia (24%). The study showed 96% of patients were mentally retarded. In the study, 48% ofchildren with CP had a seizure disorder. Conclusion: Spastic cerebral palsy was the commonest clinical type of cerebral palsy withquadriplegia being the most common topographical type of spastic cerebral palsyfollowed by diplegia as observed in this study. The prevalenceof behavioral problems like autism spectrum disorder and ADHD in the clinical sampleof children with CP was found to be 10%.PDD is not rare in CP and should be considered in patients with other comorbid conditions.
... The observed dysfunctions in the present sample are likely related to spasticity and oral motor difficulties, which are linked to pyramidal tract damage and are associated with reduced nutritional intake [43][44][45][46][47][48]. Therefore, it is not surprising that the children in the SCP group were observed to display compensatory strategies while eating. ...
Dysphagia and resulting pulmonary sequelae are frequently observed in children with spastic cerebral palsy (SCP). However, physiological evidence regarding airway protective behaviors (specifically swallowing and cough function) in these children is sparse. The aim of this investigation was to quantify specific feeding, swallowing, and cough impairments in children with SCP compared to controls. Eleven children with SCP (mean age: 7 ± 2 years; GMFCS: I-V; MACS: I-V) and 10 age-matched controls participated. Clinical feeding and swallowing performance was evaluated with the dysphagia disorder survey (DDS) using standardized liquid, puree, and chewable solid consistencies. Suprahyoid muscle activity and respiratory-swallow patterns were assessed with simultaneous surface electromyography and respiratory inductance plethysmography as children swallowed the various consistencies. Voluntary cough airflow measures were also obtained. Nonparametric tests were used for group comparisons and correlational analyses. Compared to controls, children with SCP demonstrated more signs of clinical feeding and swallowing impairment (p < 0.0001, η2 = 0.771), heightened suprahyoid muscle activity for puree (p = 0.014, η2 = 0.305) and chewable solids (p = 0.001, η2 = 0.528), more frequent post-swallow inhalation across liquid (p = 0.005, η2 = 0.401), puree (p = 0.014, η2 = 0.304), and chewable solids (p = 0.035, η2 = 0.223), and lower cough volume acceleration (p = 0.019, η2 = 0.289). Post-swallow inhalation for chewable solids was correlated with the DDS Part 1 (rs = 0.734, p = 0.010), DDS Part 2 (rs = 0.610, p = 0.046) and the DDS Total scores (rs = 0.673, p = 0.023). This study is the first to provide evidence of specific physiological deficits of both swallowing and voluntary cough in children with SCP. Potential hypotheses explaining these deficits and implications for physiologically driven management are explored.
... Despite considerable advances in medical care of affected infants, the overall incidence of CP has remained unchanged within the recent decades (3,4). This clinical event could limit children's daily functional abilities and disturb their health-related quality of life, and lead to death in case of deterioration of clinical conditions as well as appearing concurrent clinical complications, such as neurological, cardiovascular, or respiratory complications (5,6). Furthermore, this disability could be accompanied by mental retardation because of reduced oxygen and also further decrease of supply to neural pathways and neuronal structure (7). ...
... Entretanto devido as dificuldades motoras grosseiras, finas e oromotoras a comunicação nesses lactentes pode estar prejudicada e a expressão da capacidade intelectual será limitada. Portanto se a Paralisia Cerebral for tratada de uma maneira multidisciplinar com terapia nutricional, ocupacional e física para maximizar os esforços de reabilitação, os pacientes podem ser mais integrados academicamente e socialmente 21,22 . ...
Brazil has made a wide development and contribution in the field of Public Health. These contributions have maximized public health decision-making, which is a factor of great importance for the maintenance of health of a given population, either in the prevention of disease, as is the case of immunizations or with actions in Health Promotion, improving the quality of life of the affected population. Thus, the Journal of Human Growth and Development has contributed enormously to the dissemination of knowledge, not only in Brazil but also in the world making a major effort with its publications in English which is the preferred language of the modern scientific world. It was evidenced the importance of research in the investigation of better ways to obtain the public health of a given community, bringing discussion of themes that involve aspects of human growth and development such as nutritional aspects, sexuality, motor development, covering situations and diseases as obesity, cerebral palsy, dyslexia and violence. The Journal of Human Growth and Development has maintained the tradition of approaching the different aspects that involve clinical practice for people and for Public Health.
... The aetiology of CP in these particular cases could thus be determined by the imaging examination. 53 Spoken language comprehension performance was moderately to severely delayed in all children with malformations. However, a more specific analyses of these underlying brain abnormalities in relation to spoken language comprehension was not possible since brain malformations constitute i) a different developmental sequel of the brain and ii) cannot be scored with an adapted scoring system such as according to Cioni et al. 19 Despite adjustments to optimize test circumstances to the individual child's conditions, we excluded children with (severe) neurological visual impairment (severe CVI or cortical blindness) or whose vision abilities were uncertain. ...
Patients with severe cerebral palsy (CP) often have poor speech ability but potentially better language comprehension. The arcuate fasciculus and the extreme capsule are two important language tracts between the Wernicke and Broca areas. Using diffusion tensor imaging, we visualized language tracts and pyramidal tracts in both hemispheres in 10 controls (5 to 18 years) and 5 patients (5 to 23 years) with severe CP. Language comprehension was assessed with a recently designed instrument (the Computer-Based instrument for Low motor Language Testing [C-BiLLT]). The language tracts were visualized in all control children and in four CP patients. In one CP patient without any objective language comprehension skills, no language tract could be visualized. Both language and pyramidal tracts were smaller in patients than in controls. These preliminary data indicate a relation between language tracts and language skills. Further research is necessary to study the value of structural integrity of language tracts in predicting language comprehension in CP patients.
... Quanto ao tipo de tônus a classificação corresponde a espástico, discinético e atáxico. O tipo espástico é o mais encontrado, em torno de 75% (Souza, 1998); os discinéticos representam 14% a 20% dos indivíduos com sequelas de paralisia cerebral (Gianni, 2003;Schwartzman, 2004;Russman, Ashwal, 2004). ...
... A growing body of research focuses instead on infants at high risk for CP, as up to two-thirds of infants born extremely preterm with encephalopathy of prematurity or neonates with perinatal brain insults receive a later diagnosis of CP. 5 These infants often have abnormalities on neurological examinations, the General Movements Assessment and/ or neuroimaging findings that together confer >90% risks of developing CP. [6][7][8][9][10] Some pediatric populations with other developmental disorders can also have a higher risk of coexisting CP, even though they show no clear perinatal insults. 11 In addition to their motor impairments, infants at high risk for CP have frequent early speech and language problems. ...
Aim:
We evaluated the level of evidence of speech, language, and communication interventions for infants at high-risk for, or with a diagnosis of, cerebral palsy (CP) from 0 to 2 years old.
Method:
We performed a systematic review of relevant terms. Articles were evaluated based on the level of methodological quality and evidence according to A Measurement Tool to Assess Systematic Reviews (AMSTAR) and Grading of Recommendations Assessment, Development and Evaluation (GRADE) guidelines.
Results:
The search terms provided 17 publications consisting of speech or language interventions. There were no interventions in the high level of evidence category. The overall level of evidence was very low. Promising interventions included Responsivity and Prelinguistic Milieu Teaching and other parent-infant transaction frameworks.
Interpretation:
There are few evidence-based interventions addressing speech, language, and communication needs of infants and toddlers at high risk for CP, and none for infants diagnosed with CP. Recommendation guidelines include parent-infant transaction programs.
... The diagnosis of CP is based on a history of abnormal motor development that is not progressive coupled with examination that helps in locating the specific site of the lesion in the brain (Russman and Ashwal, 2004). The observation of the form of CP, medical history of the mother and child and onset of the disorder can reveal the cause of CP (Nucleus Catalog Medical Reference Library, 2003). ...
Cerebral palsy (CP) is a major cause of childhood disability. The objective of this study was to investigate the causes, types, severity, history of pregnancy, delivery, maternal care, demographic and clinical characteristics of children with CP and their parents as seen in outpatient physiotherapy clinics of the selected hospitals in Southwest Nigeria.A cross-sectional survey of children with CP and their parents was conducted in twelve hospitals in Southwest Nigeria. Information about the participants was obtained from hospital case records, physical examination and interviews. Data were summarized using descriptive and Chi-square tests with Alpha level put at 0. 05.Two hundred and thirteen children with CP were seen, aged 18 months to 12 years, and most of them were male (59.2%). The majority (41.0%) of the mothers were in the age range of 28 to 33 years. Jaundice (39.9%), asphyxia (26.8%) and infection (17.4%) were the leading causes of CP and spastic CP was the most common type (81.7%). Quadriplegic CP presentation was predominant (67.1%), and leading co-morbidities were mental retardation (31%) and speech impairment (26.3%). About 50% of the children severely affected by CP fell within Levels 4 and 5 of the Gross Motor Function Classification System (GMFCS) of CP severity.It was concluded that cerebral palsy in Southwest Nigeria is mainly associated with jaundice, asphyxia and infections. Spastic cerebral palsy was most common and quadriplegic affectation was predominant. It is recommended that factors promoting perinatal problems should be curtailed.
... Clinically, the presence and degree of spasticity is assessed in the upper and lower limbs, but it is known that it can also be present in the muscles of other parts of the body, for example in the jaw muscles. This is the reason for a high prevalence of speech and other oral-motor dysfunctions (approximately 38% of CP patients have impaired speech) [7]. The clinical tests used for spasticity assessment (Ashworth, Tardieu) are subjective tests, with low accuracy [2]. ...
Purpose:
Patients with spasticity suffer not only from neurological problems but also from various dentistry problems due to spasticity of the jaw muscles. Measurements of motion in temporomandibular joints should reflect the amount of abnormal muscle tone of these muscles. The aim of this study was to find out if the measurements of temporomandibular joint movements performed with the ultrasound Zebris device are different in cerebral palsy patients than in healthy subjects; and to find out if the information on the degree of spasticity in the lower legs provided by the Wartenberg test could be used to predict the degree of spasticty in the jaw muscles.
Method:
Twenty five healthy subjects and 25 cerebral palsy patients participated in the study. Two types of measurements were performed: temporomandibular movements measured with Zebris device, and instrumented Wartenberg test.
Results:
The laterotrusion and opening movements are different in CP patients than in healthy subjects. Laterotrusion movement correlates with velocity measured during the Wartenberg test.
Conclusion:
This finding suggests that high spasticity in the lower legs could indicate jaw movement restrictions in CP patients.
... There is a rising incidence of childhood epilepsy, where it reached up to 11.29 per 1000 in Africa. [5][6][7] Despite this, neurology as a pediatric subspecialty rarely attracts attention and priority in the cramped health budget in Sudan and other African countries. 8 The available neurology services are mainly found in big cities. ...
Background: There is no available data from Sudan reflecting the magnitude of the neurological disorders and disabilities in the pediatric age-group. This study aims to evaluate the pattern of neurological disorders among Sudanese children. Patients and Methods: This is a retrospective survey of children with epilepsy and other neurodisability disorders seen at pediatric neurology outpatient clinic, during the period from January 2007 to August 2013. The data of 9600 patients were analyzed. Results: A total of 6019 patients were included in the study. The majority of the patients had epilepsy that amounted to 52.8%, followed by cerebral palsy (19.1%), congenital anomalies of the central nervous system (6.2%), neuromuscular disorders (3.2%), stroke (2.4%), ataxia and movement disorders (1.9%), assumed genetic syndromes (1.2%), and others. Conclusion: Neurological disorders constitute a major cause of chronic morbidity in pediatric age-group.
... There is a rising incidence of childhood epilepsy, where it reached up to 11.29 per 1000 in Africa. [5][6][7] Despite this, neurology as a pediatric subspecialty rarely attracts attention and priority in the cramped health budget in Sudan and other African countries. 8 The available neurology services are mainly found in big cities. ...
Background
There is no available data from Sudan reflecting the magnitude of the neurological disorders and disabilities in the pediatric age-group. This study aims to evaluate the pattern of neurological disorders among Sudanese children.
Patients and Methods
This is a retrospective survey of children with epilepsy and other neurodisability disorders seen at pediatric neurology outpatient clinic, during the period from January 2007 to August 2013. The data of 9600 patients were analyzed.
Results
A total of 6019 patients were included in the study. The majority of the patients had epilepsy that amounted to 52.8%, followed by cerebral palsy (19.1%), congenital anomalies of the central nervous system (6.2%), neuromuscular disorders (3.2%), stroke (2.4%), ataxia and movement disorders (1.9%), assumed genetic syndromes (1.2%), and others.
Conclusion
Neurological disorders constitute a major cause of chronic morbidity in pediatric age-group.
... All cases of CP seen over the study period were recruited into the study, having obtained informed consent from the caregivers. The diagnosis of CP was clinical, based on a history of abnormal motor development that was not progressive, coupled with the presence of abnormal neurological signs that localized the lesion to the brain [14]. All the diagnoses were made by the Pediatric Neurologist and all the children who satisfied the diagnostic criteria were enrolled into the study. ...
Although cerebral palsy (CP) is primarily a motor disorder, a significant proportion of individuals with CP have associated neurocognitive and sensory impairments that adversely affect the prognosis of the disease. This study was undertaken to determine the pattern and burden of associated neurocognitive and sensory deficits in Nigerian children with CP. All consecutive cases of CP seen at the Pediatric Neurology clinic of the University College Hospital, Ibadan, Nigeria over a period of 3 years were evaluated for severity of motor impairment and any accompanying neurocognitive and sensory deficits. A total of 303 children, 187 males and 116 females were studied. Severe perinatal asphyxia (42.2%), bilirubin encephalopathy (23.4%) and intracranial infections (15.8%) were the leading risk factors for CP. Associated neurocognitive/sensory impairments were present in 269 (88.8%) of the cases. The most frequent deficits, which are associated with day to day functioning, were epilepsy (40.3%), hearing impairment (33.0%), visual impairment (32.0%), growth impairment (29.0%) and speech disorders (24.8%). Cognitive impairment was present in 30 (83.3%) of the 36 children aged 6 years and above. Deficits were multiple in 51.5% of the cases. The study showed a statistically increased risk of multiple neurocognitive and sensory deficits with presence of spastic quadriparesis (P< 0.001, odds ratio 2.553, 95% confidence interval 1.55-4.25) and microcephaly (P< 0.001, odds ratio 3.83, 95% confidence interval 2.38-6.18). Neurocognitive and sensory impairments are common in Nigerian children with CP. These can further compromise the quality of life and increase the burden of care on affected children and their families.
... It was indicated that like other disorders included in neurodevelopmental disorders, the frequency of speech-language disorders increased in children who had been diagnosed with CP. In their study, Yalçınkaya et al. (29) indicated the frequency of speech-language disorders in children diagnosed with CP was 32% in the age group between 2 and 18 years; Russman and Ashwal (30) found this frequency to be 38%. In our study, the relationship between the intellectual level and speech-language disorders in children with CP was related to the fact that common etiological factors affect these two neurodevelopmental disorders (31,32). ...
Introduction:
Cerebral palsy (CP) is the most common significant motor impairment in childhood. CP is defined as a primary disorder of posture and movement; however, intellectual impairment is prevalent in children with CP. The purpose of this study was to examine the intelligence level associated with gross motor function and hand function, type of CP, the presence of comorbid disorders such as epilepsy, and other factors.
Methods:
In total, 107 children with CP were included. Age, gender, prenatal/natal/postnatal risk factors, type of CP, and presence of other neurodevelopmental disorders were recorded as demographic findings. Intellectual functions of the patients were determined by clinical assessment, adaptive function of daily life, and individualized, standardized intelligence testing. The gross motor function and hand function of the patients were classified using the "Gross Motor Function Classification System" and "Bimanual Fine Motor Function" measurements, respectively.
Results:
The mean age of the patients was 8.10±3.43 years (2-16 years). The study included 63 (58.9%) male patients and 44 (41.1%) female patients. During clinical typing, 80.4% of the patients were spastic, 11.2% were mixed, 4.7% were dyskinetic, and 3.7% were ataxic. Intellectual functioning tests found 26.2% of the children within the intellectual norm and that 10% of the children had a borderline intellectual disability, 16% of them had a mild intellectual disability, 17% of them had a moderate intellectual disability, and 30.8% of them had a severe intellectual disability. No significant relationship was determined between the CP type and intellectual functioning (p>0.05). Intellectual functioning was found to be significantly correlated with hand functions and motor levels (p<0.001). Factors related with intellectual functioning were neonatal convulsion, epilepsy, and speech disorders.
Conclusion:
Intelligence assessment should be an essential part of CP evaluation and research. There is not enough reliable knowledge, unanimity regarding validity data, and population-specific norms in the intelligence assessments of children with CP. Research is required to assess properly intelligence for children with CP.
Cerebral palsy (CP), the most common disability in childhood, is adevastating non-progressive ailment of the infants' brain with lifelongsequelae, e.g., spastic paresis, chronic pain, inability to walk, intellectualdisability, behavioral disorders, for which there is no cure at present.CP may develop after pediatric brain damage caused, e.g., by hypoxic-ischemia, periventricular leukomalacia, intracranial hemorrhage, hypoxic-ischemicencephalopathy, trauma, stroke, and infection. About 17 million peopleworldwide live with cerebral palsy as a result of pediatric brain damage.This reflects both the magnitude of the personal, medical, andsocioeconomic global burden of this brain disorder and the overt unmettherapeutic needs of the pediatric population.This review will focus on recent preclinical, clinical, and regulatorydevelopments in cell therapy for infantile cerebral palsy bytransplantation of cord blood derived mononuclear cells from bench tobedside. The body of evidence suggests that cord blood cell therapy ofcerebral palsy in the autologous setting is feasible, effective, and safe,however, adequately powered phase 3 trials are overdue.
https://mc.manuscriptcentral.com/jpmedJournal of Perinatal Medicine
https://www.researchgate.net/publication/365303033_2022_10_27_JPMed20220505_Accept_in_its_present_form [accessed Nov 11 2022].
Infantile cerebral palsy is one of the most prevalent diseases and the most frequent cause of disability in paediatrics. Children with cerebral palsy have complex health care needs and often require the care of a multidisciplinary team. However, in many cases there is no paediatrician with overall responsibility for coordinating follow-up.
We have produced a support document intended for paediatricians coordinating the care of children with cerebral palsy. Our aim is to provide an ordered compilation of the main issues these patients may develop, to know how to identify and address them if necessary, and to establish criteria for referring these patients to other specialists.
Resumen
La parálisis cerebral infantil es una de las enfermedades más prevalentes y la causa de discapacidad más frecuente en pediatría. Los niños con parálisis cerebral tienen necesidades de atención médica complejas y a menudo requieren atención por un equipo multidisciplinar, sin embargo, en muchas ocasiones no existe la figura de un pediatra responsable que coordine todo el seguimiento.
Realizamos un documento de ayuda en el abordaje de niños con parálisis cerebral dirigido a pediatras que sean coordinadores en la atención de estos pacientes. Nuestra finalidad es la de recopilar de forma ordenada los principales problemas que pueden desarrollar estos pacientes, saber cómo identificarlos y abordarlos en caso necesario, y establecer criterios para la derivación de estos pacientes a otros especialistas.
This paper presents results on a study which used the Gross Motor Function Classification System-Expanded and Revised (GMFCS-E&R), Manual Ability Classification System (MACS) and adapted Pictorial Test of Intelligence, Second Edition (PTI-2) to assess the gross motor skills, fine motor skills and intellectual ability of pupils with cerebral palsy respectively. Additionally, the potential relationship between motor skills and intellectual ability of pupils with cerebral palsy were explored. One-hundred and thirty three (133) pupils with cerebral palsy (79 males; 54 females) completed the study. Majority of pupils with cerebral palsy were classified in the higher levels of the GMFCS-E&R and MACS (levels I/II). Applying a linear regression model, both gross and fine motor skills statistically significantly predicted intellectual ability of pupils with cerebral palsy. While the relation between motor skills and intellectual ability of persons with cerebral palsy has typically been viewed from the perspective of gross motor skills, it may be useful to emphasize fine motor activities as well.
Keywords: Gross motor, fine motor skills, intellectual ability, pupils with cerebral palsy
Background:
Cerebral palsy affects the general neurological development of those involved. This usually culminates into various muscular deficits some of which are amenable to physiotherapy intervention and rehabilitation.
Method:
A 6 year retrospective study was conducted to find out the clinical presentation and physiotherapy management of cerebral palsy patients in ESUT Teaching Hospital Enugu, Nigeria, between June 2009 and May, 2015.
Result:
146(30.2%) cases of cerebral palsy were noted out of 483 children seen within the period. There was male predominance n- 86(58.9%) and the commonest cause of cerebral palsy was birth asphyxia n- 56(38.4%).Several physiotherapy treatment modalities were applied, the common ones being neurodevelopment therapy, trans-cutaneous electric nerve stimulation, and passive and active exercise modules.Majority of the patients' accessed treatment less than 5 times n- 86(59%) and were lost to follow up subsequently. Slightly more than 10% of the patients accessed treatment consistently for 3 months.
Conclusion:
High level of abandonment of treatment and lack of more modern physiotherapy modalities were noted. There is need to emphasize better health education of parents/guardians of the patients and improve social welfare benefits for the patients.
Background
Cerebral palsy is a common pediatric problem encountered in about 1:3 per 1000 born children and causing variable mental, motor and behavioral s dilemmas. Newly introduced trials of neurogenesis with different agents are now extensively evaluated.
Objective
Our study was conducted to evaluate the neurotrophic response to B12 vitamin and omega-3 fatty acids in children diagnosed early with variable forms of cerebral palsy. The response was monitored both clinically and with C.T Scan as being a highly predictive tool for assessing cerebral palsy.
Design
The study was carried out on 40 cerebral palsy patients; 26 (65%) out of them were girls, and 14 of them were boys, aged from 0 to 5 years old; from outpatient clinic at Zakho/Duhok General Hospital in Kurdistan Region-Iraq. Patients were treated and followed up for 6 month to one year. They were represented and adjusted by full history taking and clinical examination. Brain C.T scans was done for every patient to assess the degree of brain atrophy before starting this combined therapy, and every month for six months to one year.
Results
The study revealed that early intervention of both omega 3 and B12 vitamin in children under 5 with cerebral palsy (cp) shows great response based on clinical examination and CT scan findings. after combined therapy, 80% of children with delayed speech delay have very good response and improvement, 77% of children with delayed milestone and hypertonia, and 87% with delayed walking have positive clinical outcomes. Both sexes have equal response to combined therapy. Such findings were obtained as a result of early treatment and diagnosis of children with (CP). In addition, improvement in CT scan results was obtained. 84% of treated children have great improvement in their neuroimaging results from moderate/severe forms of brain atrophy to mild form of brain atrophy after being treated and followed up for 6 months- 1 year.
Conclusions
The damaged brain sites based on CT scan results, showed progressive improvement in response to B12 and omega-3 fatty acids upon daily supplement throughout 6 months to one year. B12 vitamin and omega- 3 fatty acids are valuable therapy for children with various forms of cerebral palsy particularly when being linked. The greatest improvement in speech and motor development was significantly observed in about 32 patients (80%) of treated children with combined therapy. Others have less response to combine therapy as being presented and diagnosed beyond 1 year of age (16%)
There are presented results of own clinical studies of instrumental characteristics of children with hereditary diseases, initially observed with a diagnosis of cerebral palsy. Under the supervision, there were 44 children aged 2 to 16 years, including 23 boys and 21 girls. All patients were diagnosed with cerebral palsy at different age periods of life. At the same time, another type of cerebral palsy (G80.8) was established in 34 patients, spastic diplegia (G80.1) - in 5 patients, spastic cerebral palsy (G80.0) - in 4 children, pediatric hemiplegia (G80.2) - in 1 child. Prospective observation of patients revealed they to have separate phenotypic features not typical of cerebral palsy and brain MRI data, which determined the need to continue the diagnostic search with the involvement of molecular genetic studies and allowed us verifying a variety of genetically determined diseases. There is presented a comparative analysis of clinical symptoms and results of examinations of patients with genetically determined diseases and children with cerebral palsy.
Background Cerebral palsy is a common pediatric problem encountered in about 1:3 per
Background Cerebral palsy is a common pediatric problem encountered in about 1:3 per
Background: Cerebral palsy is non-progressive disorder of posture or movement due to a lesion of the developing brain. It is the commonest physical disability in childhood. Objective: To study the clinical, neurological abnormalities, prevalence of convulsion (epilepsy) & to asses the value of CT scans of brain in patients with cerebral palsy. Patients& Methods: It is a cross-sectional hospital based study, carried at Al-Kindy Teaching hospital outpatient pediatrics unites for the period from first of January through June 2005. A total of 91 cases (52 boys, 39 girls) with over all mean age 25.6-month range (1 month-84 month) were collected. Data were collected from their parents about age, sex, main clinical presentation, and prenatal, perinatal, postnatal history, history of convulsion. They all had clinical examination. All patients were sent to radiology department at same hospital for CT scan of brain. Results: The study revealed that boys affected more than girls (52 boys, 39 girls) with male to female ratio 1.33:1,thecommonest age group affected between 7 month-12 month (30.8%), followed by the age group between 13-24 month (20.9%). delayed milestone with hypertonia & convulsion was the commonest clinical presentation, each represent (48.3%) followed by delayed milestone with hypotonia (34.0 %), microcephaly (29.6%) & speech delay (27.4%). The commonest type of cerebral palsy was spastic form (53. 9%), followed by hypotonic form (34.0%). Convulsion was predominant in quadriplegic cerebral palsy (50%). cortical brain atrophy was commonest CT scan finding (41.7%), followed by sub cortical brain atrophy (28.6%). Cortical brain atrophy was predominant in quadriplegic cerebral palsy (27.5%).CT scan was normal in (19.8%), predominantly in hypotonic cerebral palsy (13.2%). Conclusion: The pattern of clinical presentation & prevalence of convulsion in patients with cerebral palsy in the present study are comparable to the result from studies in other clinical settings with slight variation. CT scan of brain is highly valuable in patients with cerebral palsy.
The focus on studies regarding heart rate variability and sympathetic-parasympathetic balance is increasing
along the time, and this behavior in individuals with Cerebral Palsy (CP) is important for
determining the possibility of heart diseases in this population. The objective of this study was to evaluate
the variability of heart rate and cardiac autonomic control in individuals with CP. This study was conducted
in a nonprofit organization in the city of Carapicuíba that houses individuals with severe CP. The
Polar RSX800CS heart rate was used to determine the behavior of the CP group and the control-group.
The CP group was assessed three times at intervals of 30 days between each evaluation, while the control
group was assessed only once. Twelve individuals with PC and 13 control individuals (mean age of 16.2
± 3.7 years and 15.9 ± 4.1 years, respectively) were assessed. There was no statistical difference between
groups for both time and frequency. Young individuals with severe CP diagnosis didn´t differ from typically
developed individuals with regard to cardiac autonomic behavior.
Мета. Розробити діагностичні критерії і вдосконалити техніку виконання селективної задньої ризотомії (СЗР) у хворих з приводу інкурабельної локальної спастичності нижніх кінцівок.Матеріали і методи. У 12 хворих з приводу інкурабельної локальної спастичності нижніх кінцівок здійснено СЗР корінців LII–SI (у 6 — при дитячому церебральному паралічу (ДЦП), у 6 — з наслідками перенесеного мієліту на грудному рівні і спинномозкової травми). Тонус в окремих групах м’язів становив 3,8–4 бала (за шкалою Ashworth), сила м’язів — 2,2–2,6 бала (за шкалою Asia), обмеження обсягу пасивних рухів 3–4 ступеня. Тяжкість моторної неспроможності оцінювали за Gross Motor Function Classification System (GMFCS). Для виявлення контрактури перед операцією проводили тест параневральної блокади з використанням 1% розчину бупівакаїну. Хворим на ДЦП здійснено кістково-пластичну ламінотомію. Тривалість спостереження 4 роки.Результати. В усіх хворих після операції відзначали достовірне зниження тонусу м’язів до 1,2–1,6 бала (р<0,05), збільшення обсягу рухів у суглобах (р<0,001), недостовірне збільшення сили м’язів. У 8 хворих у віддаленому періоді виявлене покращення моторних функцій за шкалою GMFCS на 1 пункт (відмінний результат); у 2 хворих тонус знизився на 1–1,5 бала без змін локомоторного статусу (хороший результат); у 2 — тонус знизився на 1 бал без змін за шкалою GMFCS (задовільний результат). Рецидив спастичності, деформацію хребта, розлади чутливості не спостерігали.Висновки. СЗР на поперековому рівні є ефективним методом лікування інкурабельної локальної спастичності нижніх кінцівок, що забезпечує тривале зниження тонусу м’язів, збільшення функціональних можливостей, полегшує догляд за хворими.Провідникова блокада відповідних нервів з використанням бупівакаїну до операції дозволяє виявити необоротні зміни в м’язах і суглобах, за яких виконання СЗР протипоказане.Здійснення СЗР протипоказане при змішаних порушеннях тонусу м’язів (ригідність, дистонія), використанні спастичності для опори і ходьби.Виконання у дітей кістково-пластичної ламінотомії дозволяє попередити виникнення деформації хребта у віддаленому післяопераційному періоді.СЗР слід виконувати з обов’язковим використанням мікрохірургічної техніки, мікроскопа та електрофізіологічного моніторингу.
Background
Cerebral palsy is a common paediatric problem encountered in about 1:3 per 1000 born children and causing variable mental, motor and behavioural s dilemmas. Newly introduced trials of neurogenesis with different agents are now extensively evaluated.
Objective
Our study was conducted to evaluate the neurotrophic response to B12 vitamin and omega-3 fatty acids in children diagnosed early with variable forms of cerebral palsy. The response was monitored both clinically and with C.T Scan as being a highly predictive tool for assessing cerebral palsy.
Design
The study was carried out on 40 cerebral palsy pa1ents; 26 (65%) out of them were girls, and 14 of them were boys, aged from 0 to 5 years old; from outpa1ent clinic at Zakho/Duhok General Hospital in Kurdistan Region-Iraq. Patients were treated and followed up for 6 month to one year. They were represented and adjusted by full history taking and clinical examination. Brain C.T scans was done for every patient to assess the degree of brain atrophy before starting this combined therapy, and every month for six months to one year. There was an improvement in general health of children after interventional therapy.
Results
The study revealed that early interven1on of both omega 3 and B12 vitamin in children under 5 with cerebral palsy (cp) shows great response based on clinical examination and CT scan findings. Almost, after combined therapy, 80% of children with delayed speech delay have very good response and improvement, 77% of children with delayed milestone and hypertonia, and 87% with delayed walking have posi1ve clinical outcomes. Bothe sexes have equal response to combined therapy. Such findings were obtained as a result of early treatment and diagnosis of children with (CP). In addition, among the treated children with CP, improvement in CT scan results was obtained. 84% of treated children have great improvement in their neuroimaging results from moderate/severe forms of brain atrophy to mild form of brain atrophy after being treated and followed up for 6 month- 1 year.
Conclusions
The damaged brain sites based on CT scan results, showed progressive improvement in response to B12 and omega-3 fa?y acids upon daily supplement throughout 6 months to one year. However, combining these 2 drugs showed preservative synergistic consequences. B12 vitamin and omega- 3 fa?y acids are valuable therapy for children with various forms of cerebral palsy particularly when being linked. The greatest improvement in speech and motor development was significantly observed in about 32 patients (80%) of treated children with B12 vitamin and omega- 3 fa?y acids. Others have less response to combine therapy as being presented and diagnosed beyond 1 year of age (16%)
Cerebral palsy (CP) is currently considered as a clinically defined symptom complex applied to individuals with a static, nonprogressive motor impairment of early onset that is cerebral in origin. It describes a group of motor impairment syndromes secondary to genetic and acquired disorders of the developing brain. Therefore, it is not a single entity with respect to pathogenesis but rather an etiologically heterogeneous entity with multiple possible causes that result in an aberration or injury to the maturing central nervous system. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior and/or by a seizure disorder. The prevalence is estimated between 1.5 and 2.5 per 1,000 live births (Ferriero 1999).
This chapter examines common features of physical and sensory disabilities often encountered in children and adolescents and discusses ways to foster quality of life for children and adolescents with these disabilities. These disorders often affect the lives of children and their families in profound ways; therefore they need healthcare and social services from specialists with a range of expertise. The chapter discusses the etiology, signs and symptoms, and prognosis of cerebral palsy (CP), neural tube defects, muscular dystrophy (MD), traumatic brain injury (TBI), spinal cord injuries (SCI), and hearing and visual impairment in children. The common pharmacologic and non-pharmacologic therapies, the role of nursing, occupational and physical therapy, and speech and language therapy for these disabilities are also discussed.
Cerebral palsy (CP) is a common chronic motor disorder with associated cognitive, communicative, and seizure disorders. Children with CP have a higher risk of dental problems creating signi cant morbidity that can further a ect their wellbeing and negatively impact their quality of life. Screening for dental disease should be part of the initial assessment of any child with CP. e objective of this article is to present an updated overview of dental health issues in children with CP and outline important preventative and practical strategies to the management of this common comorbidity. Providing adequate oral care requires adaptation of special dental skills to help families manage the ongoing health issues that may arise. As oral health is increasingly recognized as a foundation for general wellbeing, caregivers for CP patients should be considered an important component of the oral health team and must become knowledgeable and competent in home oral health practices.
Cerebral palsy (CP) is currently considered as a clinically defined symptom complex applied to individuals with a static, nonprogressive motor impairment of early onset that is cerebral in origin. It describes a group of motor impairment syndromes secondary to genetic and acquired disorders of the developing brain. Therefore, it is not a single entity with respect to pathogenesis but rather an etiologically heterogeneous entity with multiple possible causes that result in an aberration or injury to the maturing central nervous system. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior and/or by a seizure disorder. The prevalence is estimated between 1.5 and 2.5 per 1,000 live births (Ferriero 1999).
Visual unresponsiveness in an otherwise healthy baby is an alarming finding. Parents are staring down the cannon at a lifetime of potential blindness in their baby and are understandably anxious and inquisitive about the cause, severity, and prognosis of the condition. Depending on the underlying cause, the visual outcome may range from normal vision to complete blindness. The importance of establishing an accurate diagnosis in this setting is obvious. This chapter emphasizes the congenital visual disorders in infancy but includes some discussion of other visual system disorders that may manifest later in childhood.
Infants and children who manifest motor dysfunction early in life have decreased ability to interact with their environment which results in less learning opportunities; hence, the consequent cognitive impairment often seen in these circumstances. During this period of brain growth, the brain plasticity allows the rewiring and the creation of new synapses that would compensate for the motor and cognitive deficits [1]. Therefore, early intervention to limit the physical disability and encourage motility, especially before the age of three has the potential to increase the chances of limiting or overcoming further developmental disabilities and promoting alternative learning methods to accomplish challenging tasks.
The causal disease Definitions and epidemiology Cerebral palsy (CP) affects between 2 and 3 per 1000 live births and is thought to be the most common cause of serious physical disability in childhood (SCPE 2000). The definition and classification of cerebral palsy is not straightforward and remains an area of debate and controversy. Martin Bax's elegant 1964 definition describes cerebral palsy as “a dynamic disorder of posture and movement caused by a non-progressive defect or lesion of the developing brain.” A developmental perspective is key to understanding cerebral palsy and one of its strongest associations, epilepsy. William Little in his 1843 lecture to the Orthopaedic Institution of London on “The deformities of the human frame” noted that epilepsy could adversely affect the already vulnerable child with CP. Recent studies have emphasized that epileptic seizures can impact on behavior and cognition, and impair the ability to thrive, learn, and develop in individuals with CP (Carlsson et al. 2008). Optimal seizure management is therefore very important to allow an individual to achieve their full developmental potential and improve their quality of life.
Cerebral palsy (CP) is one of the leading causes of childhood disability worldwide, with the greatest burden found in the developing countries of the world. A longitudinal study was carried out on all consecutive new cases of CP who presented at the neuro-developmental clinic in Nigeria over a period of 6 months to evaluate the continuum of care available to children with CP following diagnosis. At the end of 1-year post-enrolment into the study, all cases were evaluated for survival, on-going rehabilitative care, functional improvement and reasons for default. Eighty-two children were enrolled into the study. Mean age was 25.3 ± (SD = 25.5) months and the leading causes of CP were birth asphyxia (39.0%), bilirubin encephalopathy (24.4%) and post infectious brain damage (18.3%). By the end of 1 year, 61 (74.4%) had defaulted from follow up, 11 (13.4%) had died and only 21 (25.6%) were still receiving rehabilitative care. The major reasons for default were financial constraints, no improvement in condition and the hospital being too far from place of residence. Presence of severe functional impairment and absence of epilepsy were associated with an increased risk of default from medical care. In conclusion, CP remains a major cause of neurodisability in Nigeria. There is a high default rate with majority of children lacking ready access to medical care and education. There is an urgent need for improvement in medical services in the country and social support for children with CP and their families.
Definition and classification of cerebral palsy (CP) are constantly reconsidered. The previous definitions have not emphasised non-motoric neuro developmental problems that often accompany CP, as well as the progression of musculoskeletal problems. Today, the limitations of person with CP in performing activities of daily living, are increasingly assessing. Thus, the level of functional limitation becomes a key criteria in the redefinition ofCP The concept of unique definition and classification ofCP is still evolving. New parameters in the definition and classification of this clinical entity should meet the needs of clinicians and scientists, but also families and the public, in order to create a common, international language and to improve mutual communication. Simplified SCPE (Surveillance of Cerebral Palsy in Europe) classification based on neurological symptoms, and classification systems for classification of gross (GMFCS - Gross Motor Function Classification System) and fine motor function (BFMF - Bimanual Fine Motor Function and MACS - Manual Ability Classification System) have become universally accepted instruments in working with people with CP. Although the one of the proposed dimensions of classification is cause and time of occurrence ofCP, the classification by the cause would be currently unrealistic. However, the latest findings in developmental neurobiology and neuroradiology play an important role in revealing the pathogenesis ofCP, and thus facilitates planning and developing of the most appropriate therapy treatments and (rehabilitation intervention. The considerations behind the process of redefining and reclassifing CP are aimed towards unambiguously leading of national registries, easier monitoring of prevalence and comparison of the effects of interventions, and to more productive collaboration between experts in different fields.
A subgroup of children with arterial ischemic stroke in the pre- or perinatal period present with delayed diagnosis. We identified 22 children who met the following criteria: (1) normal neonatal neurological history, (2) hemiparesis and/or seizures first recognized after 2 months of age, and (3) computed tomography or magnetic resonance imaging showing remote cerebral infarct. Laboratory evaluations included protein C, protein S, antithrombin, activated protein C resistance screen (APCR), Factor V Leiden (FVL), prothrombin gene defect, methylene tetrahydrofolate reductase variant (MTHFR), anticardiolipin antibody (ACLA), and lupus anticoagulant. Not all children received all tests. Age at last visit ranged from 8 months to 16.5 years (median 4 years). Twelve were boys. Fourteen had left hemisphere infarcts. Median age at presentation was 6 months. Eighteen had gestational complications. Fourteen children had at least transient coagulation abnormalities (ACLA = 11, ACLA + APCR = 1, APCR = 2 with FVL + MTHFR = 1); 6 of these children had family histories suggestive of thrombosis. Cardiac echocardiogram was unremarkable in the 15 tested. Outcomes included persistent hemiparesis in 22; speech, behavior, or learning problems in 12; and persistent seizures in 5, with no evidence of further stroke in any patient. The persistence and importance of coagulation abnormalities in this group need further study.
Prosody is that quality of speech that imparts meaning by changes in intonation, pitch, and stress. The right hemisphere (RH) appears to be dominant for affective prosody in adults, while the left hemisphere (LH) mediates the more linguistic aspects of nonverbal communication. Few similar studies have been reported of individuals who suffered early unilateral brain damage, when brain reorganization or plasticity might be expected to play a role in ameliorating the adverse effects of focal brain damage. In this study, comprehension and expression of affective and linguistic prosody were tested in subjects with documented unilateral brain damage of pre- or perinatal onset and in matched controls. Both RH- and LH-lesion groups demonstrated difficulty on tasks involving expression of affective prosody, and on tests of linguistic prosody, compared with controls. Only the RH-lesion group was impaired on an affective comprehension task. The results indicate that even after very early unilateral brain damage, prosodic deficits may be present. However, only for affective comprehension does the side of the lesion appear to determine such deficits. The findings suggest that during brain development there is not clear brain lateralization for prosody and there may be bilateral representation for these skills during early development. There may be limitations to the ability of the developing brain to reorganize after early injury.
Objectives:
To further define the clinical spectrum of the disease for pediatric and metabolic specialists, and to suggest that the general pediatrician and pediatric neurologist consider succinic semialdehyde dehydrogenase (SSADH) deficiency in the differential diagnosis of patients with (idiopathic) mental retardation and emphasize the need for accurate, quantitative organic acid analysis in such patients.
Patients:
The clinical features of 23 patients (20 families) with SSADH deficiency (4-hydroxybutyric acid-uria) are presented. The age at diagnosis ranged from 3 months to 25 years in the 11 male and 12 female patients; consanguinity was noted in 39% of families.
Outcome measurements:
The following abnormalities were observed (frequency in 23 patients): motor delay, including fine-motor skills, 78%; language delay, 78%; hypotonia, 74%; mental delay, 74%; seizures, 48%; decreased or absent reflexes, 39%; ataxia, 30%; behavioral problems, 30%; hyperkinesis, 30%; neonatal problems, 26%; and electroencephalographic abnormalities, 26%. Associated findings included psychoses, cranial magnetic resonance or computed tomographic abnormalities, and ocular problems in 22% or less of patients. Therapy with vigabatrin proved beneficial to varying degrees in 35% of the patients. Normal early development was noted in 30% of patients.
Conclusions:
Our data imply that two groups of patients with SSADH deficiency exist, differentiated by the course of early development. Our recommendation would be that accurate, quantitative organic acid analysis in an appropriate specialist laboratory be requested for any patients presenting with two or more features of mental, motor, or language delay and hypotonia of unknown cause. Such analyses are the only definitive way to diagnose SSADH deficiency; the diagnosis can be confirmed by determination of enzyme activity in white cells from whole blood. We think that increased use of organic acid determination will lead to increased diagnosis of SSADH deficiency and a more accurate representation of disease frequency. As additional patients are identified, we should have a better understanding of both the metabolic and clinical profiles of SSADH deficiency.
Glutaric aciduria type I (GA1) is a preventable cause of acute brain damage in early childhood, leading to a severe dystonic‐dyskinetic disorder that is similar to cerebral palsy and ranges from extreme hypotonia to choreoathetosis to rigidity with spasticity. Degeneration of the putamen and caudate typically occurs between 6 and 18 months of age and is probably linked to changes in metabolic demand caused by normal maturational changes and superimposed catabolic stress. Recognition of this biochemical disorder before the brain has been injured is essential to outcome. Diagnosis depends upon the recognition of relatively nonspecific physical findings such as hypotonia, irritability and macrocephaly, and on performance of urine organic acid quantification by gas chromatography–mass spectrometry or selective searches of urine or blood specimens by tandem mass spectrometry for glutarylcarnitine. The diagnosis may also be suggested by characteristic findings on neuroimaging. In selected patients diagnosis can only be reached by enzyme assay. Specific current management by the authors of this paper includes pharmacological doses of L‐carnitine, as well as dietary protein restriction. Metabolic decompensation must be treated aggressively to avoid permanent brain damage. Multicentre studies are needed to establish best methods of diagnosis and optimal therapy of this disorder.
The importance of early diagnosis of this condition is stressed. During the initial stage various signs may suggest a cerebral lesion: apathy, coma, agitation, convulsions, signs of raised intracranial pressure, cyanosis, apnoea, feeding disturbances; the newborn must be examined for attitude, muscle tone, primitive reflexes and response to stimuli. During the second stage (free interval) signs suggestive of cerebral lesion in lactation include hypotonia, delayed psychomotor development, alterations in posture, spontaneous and reflex movements. During the last stage (starting about the end of the first year of life) the illness is established and diagnosis is simple. Additional investigations are : skull radiograms, funduscopy, electroencephalogram, air encephalography and measurement of intelligence.
The aim of this study was to estimate the prevalence and severity of feeding and nutritional problems in children with neurological impairment within a defined geographical area. In a cross-sectional study, a validated questionnaire was sent to 377 parents of children (aged 4 to 13 years) on the Oxford Register of Early Childhood Impairments with oromotor dysfunction. The return rate was 72%. Of these, 93% had cerebral palsy; 47% were unable to walk; 78% had speech difficulty; and 28% continuous drooling of saliva. Gastrointestinal problems were commonly encountered: 59% were constipated; 22% had significant problems with vomiting, and 31% had suffered at least one chest infection in the previous 6 months. Feeding problems were prevalent: 89% needed help with feeding and 56% choked with food; 20% of parents described feeding as stressful and unenjoyable. Prolonged feeding times (3h/day) were reported by 28%. Only 8% of participants received caloric supplements and 8% were fed via gastrostomy tube. Even though 38% of respondents considered their child to be underweight, 64% of children had never had their feeding and nutrition assessed. The results highlight that feeding problems in children with neurological impairment are common and severe, causing parental concern. Many of these children would benefit from nutritional assessment and management as part of their overall care.
Growth failure and nutritional status were evaluated in 141 children with quadriplegic cerebral palsy, aged between two and 18 years. Linear growth was assessed by upper‐arm and lower‐leg lengths: both means were significantly reduced. Mean bodyweight and triceps skinfold thickness fat stores were reduced to 65 per cent medians and subscapular skinfold fat stores to 81 per cent median. Muscle stores were the most preserved and reduced to 88 per cent median. Examination of the sample by age‐group showed significant reductions in growth and nutrition status indicators, even at two to four years, except for muscle area. TO determine the degree to which nutritional status affected linear growth, a set of two‐step regression analyses was conducted. The linear growth measures were significantly correlated with the measures of nutritional status.
RÉSUMÉ
Défaur de croissance d'origine alintentaire chet les en fants présentant une forme tétraplegique’ d ‘IMC
Le défaut de croissance et létat nutritional ont Aeté appréciés chez 142 enfants presentant une forme tétraplégique d'IMC, Pges de deux à 18 ans. La taille (croissance linéaire) fut déduite des longueurs du bras et de la jambe: les deux moyennes étaient significativement basses. Le poids total moyen et I‘épaisseur moyenne du pli cutané au triceps (appréciant la charge en graisse) étaient réduits à 65 pour cent du médian et le pli cutané sous scapulaire à 81 pour cent du médian. La masse musculaire était la mieux préservée, réduite á 88 pour cent du médian. L'examen de I‘échantillon par groupe d'ǎges démontrait des rAeductions significatives de la croissance et des indicateurs de I‘état nutritional. měme entre deux et quatre ans, sauf pour la masse musculaire. Pour déterminer le degré selon lequel létat nutritional affectait la taille, un ensemble d'analyses de régression à deux échelons a étéétabli. Les mesures de la taille étaient significativement corrélées aux mesures de l‘état nutritionnel.
ZUSAMMENFASSUNG
Ernährungsbedingte Wachstumsverzögerrrng bei Kindern mit Terraplegie
Bei 142 Kindern im Alter zwischen zwei und 18 Jahren mit tetraplegischer Cerebralparese wurden Wachstumverzögerung und Ernährungszustand beurteilt. Das lineare Wachstum wurde anhand von Unterarm‐ und Unterschenkellängen bestimmt: beide Manβe waren signifikant vermindert. Das mittlere Körpergewicht und die Hautfattendicke über dem Triceps (Fettspeicher) waren im Mittel auf 65 Prozent und die subscapularen Hautfaltenfettspeicher auf 81 Prozent reduziert. Die Muskelspeicher waren am besten erhalten und im Mittel auf 88 Prozent reduziert. Die Untersuchung einzelner Altersgruppen zeigte selbst bei zweibis vier‐jáhrigen eine signifikante Reduktion der Parameter für Wachstum und Ernährung auβer im muskulären Bereich. Um festzustellen, in welchum Maβe der Ernährungszustand das lineare Wachstum beeinfluβte, wurden Regressionsanalysen durchgeführt. Das lineare Wachstum korrelierte signifikant mit dem Ernährungszustand.
RESUMEN
Deficiencia en el crecimiento en relaciön con la nutrición en ninCos con parálisis cerebral cuadripléjica
Se evaluö el fallo en el crecimiento y el estado nutritivo en 142 ninCos con parálisis cerebral espástica, d de dos a 18 años de edad. El crecimiento lineal se evaluö utilizando la longitud de la extremidad superior y de la inferior, ambas reducidas significativamente. El peso corporal medio y el groso del pliegue cutáneo estaban reducidos a un 65 por ciento de promedio y el grosor del plieque subescapular (almacenamiento de grasa) lo estaba a un 81 por ciento de promedio. Los depositos musculares eran los más preservados y reducidos a un 88 por ciento de promedio. El examen de la muestra por edades mostró una reducción significativa en el crecimiento y estado de nutrición. incluso entre los dos y cuatro antos de edad, excrpto en el area muscular. Para determinar el grado en que el cstado nutritivo afectaba el crecimiento lineal se realizaron una serie de análisis de regresión en dos etams. Las medidas de crecimiento lineal tenian una correlaciön significativa con las mediciones del eitado nutritivo.
The incidence of epilepsy in 323 patients with cerebral palsy (CP) was 41.8%. Almost half of the patients with spastic tetraplegia and hemiplegia had epilepsy. The incidence was lower in patients with spastic diplegia. No sex differences were observed. Partial seizures were by far the most common form of epilepsy in spastic hemiplegia, while generalized tonic-clonic episodes predominated in all other forms of CP. A very high incidence of West syndrome was observed in patients with spastic tetraplegia. Most of the patients with spastic tetraplegia had their first seizure in the first year of life. In patients with spastic hemiplegia the onset of epilepsy was often delayed for several years. A high rate of polytherapy was recorded, but two-thirds of the patients remained seizure-free for long periods. In just over one-fifth of the patients successful withdrawal of medication was achieved.
It is generally accepted that patients with cerebral palsy suffer from a static encephalopathy causing a non-progressive disorder of posture and/or movement. We describe 7 patients from 5 families who were initially diagnosed with cerebral palsy. Eventually, excessive excretion of urinary 3-methyl glutaconic acid (3-MGA) was found. The data of our 7 patients are quite similar to the clinical description of Costeff et al (1989): Jewish-Iraqi origin (7/7), consanguinity (2/7), involuntary movements (5/7), ataxia (6/7), pyramidal involvement (6/7) and optic atrophy (6/7). The cognitive functions were intact in 5/7 and 2/7 showed mild to moderate mental retardation. The mean delay in the definitive diagnosis was 9 years. Cerebral palsy-like symptoms accompanied by optic atrophy and extrapyramidal signs should call for extensive metabolic evaluation including the determination of urinary 3-MGA.
One of the disabilities in patients with cerebral palsy (CP) is dysphagia. To establish the prevalence of dysphagia in a population of children with CP, and to determine if any factors are related to dysphagia, we studied 56 CP patients, 5-21 years, enrolled in a primary school for the disabled. Fifteen patients (27%) had either radiographic or clinical evidence of dysphagia. These 15 patients were compared to the remaining 41 patients without dysphagia. Using data obtained from chart review and interviews with speech pathologists, several factors that contributed to dysphagia were found. These included: bite reflexes, slowness of oral intake, poor trunk control, inability to feed independently, anticonvulsant medication, coughing with meals, choking, and pneumonia. We also noted trends in the following factors: presence of tongue thrusting, presence of drooling, severity of CP, poor head control, severity of mental retardation, seizures, and speech disorders. Factors not related to the presence of dysphagia include: subject age, cause of CP, and type of CP. Early, aggressive work-up and identification in CP patients with the risk factors outlined above can reduce the associated pulmonary complications.
We used MR to retrospectively analyze the brains of patients suffering from cerebral palsy, our aim being to determine MR's role in the assessment of brain damage and the relationship of pre-, peri-, and post-natal events to cerebral palsy.
Forty patients (aged 1 month to 41 years) underwent MR scanning and findings were correlated with clinical histories in all cases.
Review of MR scans of 11 patients who had been born prematurely revealed findings of periventricular white matter damage, indicative of hypoxic-ischemic brain injury (82%), the chronology of which was difficult to determine. Among 29 patients who had been born at term, three major patterns emerged: (1), gyral anomalies, suggestive of polymicrogyria, consistent with mid-second trimester injury; (2), isolated periventricular leukomalacia reflecting late second- or early third-trimester injury; and (3), watershed cortical or deep gray nuclear damage, consistent with late third-trimester, perinatal or postnatal injury. In 16 (55%) of 29 patients born at term, MR findings of intrauterine brain damage were observed; in over half of these cases MR revealed developmental anomalies, which is nearly twice the rate reported in prior studies employing CT.
Our results support a growing consensus that cerebral palsy in term infants is often the result of prenatal factors, and less commonly related to the perinatal period.
Assessment of visual acuity using the visual acuity card procedure in 164 children with cerebral palsy revealed low visual acuity in 71 per cent. Results of ophthalmological examination were available for 74 of these patients, but could not explain adequately the low visual acuity of 36 of the 43 patients (84 per cent) assessed by both the acuity card procedure and other techniques. There is a high probability that cerebral visual disturbance is present in these patients. Awareness of visual disability when compiling a programme of visual and neurodevelopmental stimulation for children with cerebral palsy is essential.
RÉSUMÉ
La prévalence des troubles centraux de la vision dans l'IMC
L'appréciation de l'acuité visuelle à partir de tableux d'acuité visuelle chez 164 sujets IMC a révélé une acuité visuelle basse dans 71 pour cent des cas. Les résultats des examerts ophtalmologiques étaient disponibles chez 74 sujets mais ne pouvaient expliquer l'acuité visuelle basse de 36 des 43 sujets (84 pour cent) évalués à la fois par les tableaux d'acuité visuelle et d'autres techniques. Il y a une haute probabilité qu'il existait un trouble visuel central chez ces sujets. La reconnaissance d'un déficit visuel en établissant un programme de stimulation visuelle et neurodéveloppementale chez les IMC est essentielle.
ZUSAMMENFASSUNG
Die Häufigkeit der cerebralen Sehstörung bei Cerebralparese
Die Beurteilung der Sehschärfe mit dem Acuity Card Verfahren bei 164 Patienten mit Cerebralpares zeigte bei 71 Prozent eine verminderte Sehschärfe. Von 74 dieser Patienten waren die Ergebnisse der augenärztlichen Untersuchung verfügbar, sie konnten aber bei 36 der 43 Patienten (84 Prozent), die sowohl mit dem Acuity Card Verfahren als auch mit anderen Methoden untersucht wurden waren, die verminderte Sehschärfe nicht hinreichend erklären. Wenn man ein Programm zur visuellen und entwicklungsneurologischen Stimulation für Kinder mit Cerebralparese zusammenstellt, ist es sehr wichtig auf das Vorliegen einer Sehstöbrung zu achten.
RESUMEN
Prevalencia de la alteración visual cerebral en la parálisis cerebral
La evaluación de la agudeza visual utilizando el procedimiento de las tarjetas de agudeza visual en 164 pacientes con parálisis cerebral reveló una agudeza visual baja en el 71 por ciento de los casos. En 74 des estos pacientes se pudo obtener un examenoftalmológico, pero ello no pudo explicar adecuadamente la disminución visual de 36 de los 43 pacientes (84 por ciento) evaluados al mismo tiempo con las cartas de agudeza y con otros técnicas. Existe una alta probabilidad de que en estos casos la alteración visual sea de origen cerebral. Es esencial el darse cuenta de la presencia de la alteración visual cuando se crea un programa de estimulación visual y neuroevolutiva en niños con parálisis cerebral.
The prevalence of dental disease, the types and quality of dental care, and the provision of services were assessed for 150 cerebral palsied children (mean age 10.25 years) attending special schools in Leeds and compared with a matched control group of 191 children (mean age 10.39 years). Similar dental caries experience existed in the two groups, but study children had more extracted and unrestored teeth, and fewer and poorer quality restorations than control children. Oral hygiene and gingival health were worse in the study group, which also exhibited delayed eruption and higher levels of tooth wear. Significantly greater overjet (5.1 mm compared with 2.5 mm) and less crowding occurred in the study group than in the control group. All parents had favorable attitudes toward dentistry and were satisfied with their children's dental care. More study children received treatment from the community dental service, while the general dental services were used more commonly by the control group.
CT brain scans were used to evaluate the most likely etiology of hemiplegic cerebral palsy in a group of children born at term. The population based study group comprised 83 children. The CT findings were classified to reflect the phase of brain maturation during which the injury causing hemiplegia most likely had occurred. The most common finding (37%) was periventricular atrophy considered to represent periventricular leukomalacia (PVL). PVL is a form of hypoxic-ischemic damage typical of the immature brain and most commonly seen as a complication of preterm birth. As this lesion was found in children born at term it was considered to reflect a cerebral injury that had occurred in utero. Cerebral maldevelopment, due to a very early intrauterine lesion, was detected in 17%, while cortical-/subcortical lesions, previously considered to be of major importance in hemiplegia, were seen in just 16% of the children. Altogether the CT findings indicated a prenatal origin of hemiplegia in 57%. Assessing the time of injury based on clinical records concurred with prenatal origin in 32% of the children thought to have prenatal origin of hemiplegia by CT. The objective information available from careful review of imaging information such as CT brain scans, is an important adjunct to clinical data. We propose that assessment of brain morphology, by means of imaging, should be integrated with clinical assessment when evaluating the etiology of brain lesions in children with congenital motor impairment.
The magnetic resonance findings in 22 children with athetotic cerebral palsy were studied. Sixteen had perinatal asphyxia, two had neonatal jaundice, and four had no association with predisposing conditions. In six of the children, symmetrical high intensity areas were found in both the thalamus and putamen in T2-weighted images. In five children they were seen only in the thalamus, and in another one only in the putamen. In six children, symmetrical periventricular high intensity areas were seen. In seven of the subjects, no abnormal magnetic resonance findings were seen. Magnetic resonance lesions, possibly caused by asphyxia, were found in the basal ganglia, thalamus and/or cerebral white matter in 14 of 16 children. Three children with lesions only in the cerebral white matter had mild motor abnormality, and six children with lesions in both the thalamus and putamen did not have mild abnormality.
We describe 14 patients with glutaric aciduria type 1 in five Canadian Indian kindreds living in Manitoba and northwest Ontario. The patients had marked clinical variability of the disease, even within families. Eight followed the typical clinical course of normal early growth and development until the onset of neurologic abnormalities, often precipitated by infection, between 6 weeks and 7 1/2 months of age. Five patients had early developmental delay; one was thought to be normal until 8 years of age. Three patients died, seven are severely mentally and physically handicapped, and four have only mild mental retardation or incoordination. Six patients had macrocephaly in the neonatal period. Computed tomography was done for 12 patients, and findings were abnormal in 11. Glutaric acid and 3-hydroxyglutaric acid were detected in increased amounts in the urine of all patients, but the concentrations were much lower than those in most other reported patients. Glutaryl coenzyme A dehydrogenase activity in skin fibroblasts, interleukin-2-dependent lymphocytes, or both, ranged from 0% to 13% of control values. There was no correlation between clinical severity and urine glutaric acid concentration or level of residual enzyme activity. We recommend that organic acid analysis of the urine be done in patients with unexplained cerebral palsy-like disorders, especially if the computed tomographic scan is abnormal. If there is suspicion of glutaric aciduria, glutaryl-coenzyme A dehydrogenase should be measured in fibroblasts or lymphocytes even if glutaric acid is not increased in the urine.
Computed tomography (CT) of the brain has been used to evaluate potential etiologies of hemiplegic cerebral palsy in a population-based group of 28 children born preterm, in whom an obvious postnatal cause of hemiplegia could be excluded. The CT findings were classified according to a system so constructed to reflect the phase of brain maturation during which the insult causing hemiplegia likely had occurred. We found that 50% had evidence of periventricular leukomalacia the lesion typical of hypoxic-ischemic injury to the immature brain. However, it was not possible, neither from the CT scans nor from retrospective review of available clinical data, to date the injury more precisely to the pre- or perinatal period. Analysis of CT findings further showed that 18% of the children had cerebral maldevelopment, indicating an injury during early fetal life as the cause of hemiplegia, earlier than clinically suspected. The CT scans were normal in 25% of the children. It is concluded that the objective information obtained by studying the morphology of brain lesions using CT provides important information in addition to clinical data, and should be integrated with clinical assessment when attempts are being made to evaluate the etiology of cerebral palsy in children.
The CT findings in 120 cerebral palsied children are analysed. The 72.5% positive findings are correlated with the clinical types, as well as the aetiological basis for the cerebral palsy. The spastic type, 83.3% of the total number of children, had the highest positive findings. The yield was increased in children with seizures (91.3%) and those in the postnatal group (90%), as well as those with birth trauma and neonatal asphyxia (94%). The findings were those of atrophy in 30.8%, hydrocephalus, in 10%, infarct in 11.6%, porencephaly in 8.3% and others. The atropic changes and their patterns are explained. Treatable lesions, such as tumour, hydrocephalus, subdural haematoma, porencephaly and hygroma were identified in 22.5% of cases. It is concluded that CT scan is definitely efficacious in the management of cerebral palsied children.
Retrospective analysis of computed tomographic (CT) examinations in 76 children with cerebral palsy without any severe intellectual handicap has revealed abnormalities in 63% of these children; abnormalities were present in 88% of former pre-term infants. This study highlights the significant pathogenic role of periventricular leucomalacia in cerebral palsy, since in 45% of the cases of this series this diagnosis had been reached as a result of a characteristic CT appearance associated with a history of premature birth and very specific clinical deficits. The CT abnormalities, mostly posterior and generally bilateral and symmetrical, affect the contours of the lateral ventricles, which become straight and angular, and often involve ventriculomegaly (85%). Localized juxtaventricular hypodensities were only found in a few cases (6%). The cognitive and intellectual sequelae in these children were more severe than in those children whose CT examination was normal, and were also more pronounced when the longitudinal fissure of the cerebrum was enlarged.
Clinico-radiologic correlations, using CT, were studied in 29 patients with ataxic cerebral palsy. The scans were normal or only slightly abnormal in 38%, posterior fossa abnormalities occurred in 28%, and 55% had obvious cerebral abnormalities which always involved the parietal lobes. There were only two cases where the changes were confined to the posterior fossa. Hydrocephalus was present in four, and there was one case of a Dandy-Walker malformation. An absolute association with any clinical subtype was unusual. All cases of simple ataxia had an obviously abnormal scan, but the changes were widespread. Where dysequilibrium was present the scans were either normal or showed widespread change. Only 25% involved the vermis. Although there was some association between hydrocephalus and ataxic diplegia, the majority of this subtype had a normal or only slightly abnormal scan. Most were hypotonic docile infants with delayed speech and motor skills, particularly those with posterior fossa abnormality. Although CT did not predict development well, those with lower IQ's were more likely to have obvious CT change. Where obvious supratentorial change was present, 75% had a history of epilepsy. In ataxic cerebral palsy CT findings are common, but variable. CT change correlates better with IQ and epilepsy than clinical subtype.
In order to examine if specific findings from CT correlate with specific clinical or neurological findings, 30 children, 5 to 16 years old, born at term, affected by congenital hemiparesis without intellectual impairment, were submitted to neurofunctional and psychological assessment and examined by CT. 28 of the 30 children had pathological CT. Two morphological CT patterns were found: A) Cavity in the cortex and underlying subcortical white matter (11 cases); B) Unilateral ventricular enlargement and paraventricular lesions (17 cases). CT was normal in two cases. In our children, born at term, no statistical correlation between CT patterns and anamnestic data was found as described in other studies, although in the patients with cortical-subcortical lesions, there was a slight prevalence of a history of perinatal complications, and in the patients with paraventricular lesions there was an uneventful history or abnormal pregnancy history.
The relation between specific CT patterns (type A and B) and specific clinical dysfunction is not statistically evident, (unless for astereognosis and type A CT pattern). However, the patients with cortical-subcortical lesions showed a slight prevalence of a lower function of the impaired hand, and a higher percentage of an I.Q. of < 90, than the group with unilateral ventricular enlargement. No relation was found between CT lesional pattern and epilepsy.
The absence of correlation between morphological aspects and clinical findings could be explained by the complexity of structural changes and remodelling properties of the central nervous system, following prenatal and perinatal brain damage.
In a 1-year birth cohort from the two northernmost provinces in Finland, Oulu and Lapland which comprised of 12 058 liveborn infants, the total number of children affected with cerebral palsy (CP) was 69. The cumulative incidence up to the age of 14 years was 5.7 per thousand. A prenatal aetiology was present in 32%, a perinatal aetiology in 36% and a postnatal aetiology in 19% whereas in 13.0% of the cases the cause remained untraceable. A total of 50 children (73%), had 1 or more additional handicaps. Mental retardation (IQ less than 85) was present in 70%, epilepsy in 48%, a visual defect in 19% and impaired hearing in 7%. The impact of the total handicaps is also illustrated by the fact that only 33% of the children were able to attend a normal school. The incidence of 5.7 per thousand recorded here is clearly higher than in studies from several other countries, as the cumulative incidence is usually reported to vary between 2 and 3 per thousand.
After the introduction of cranial computed tomography (CT) it is now possible by an atraumatic procedure to evaluate the pathophysiological findings in children suffering from cerebral palsy (CP). The aim of this study is to describe the cranial CT findings in children with CP and relate these to CP-type, grade of handicap, aetiology, and presence of other functional cerebral defects.
The CT-examination was performed in 83 children with spastic CP (44 boys and 39 girls). Fifty-seven children (67 %) had pathological CT. There was no statistically significant difference between the frequencies of pathological CT findings in the groups with tetraplegia, diplegia, and paraplegia. The frequency of pathological CT findings was increasing with increasing severity of the motor handicap (p < 0.05). There were significantly more children with pathological CT findings among CP children suffering from epilepsy, than among CP children without epilepsy (p < 0.05). The CP children with the lowest IQ, had numerical more pathological CT findings but there was no significant difference among pathological CT findings in CP children with oligophrenia compared to the rest of the group. Infarction, its sequelae and hemiatrophy were much more frequent in patients with hemiplegia (p < 0.001) compared to the other CP-types.
The most frequent pathological CT finding was atrophy (44 cases among 56 pathological CT). Central atrophy with enlargement of the ventricular system or parts of this was found in 39 children. A special kind of central atrophy in this paper called isolated atrophy around cella media, was seen more frequently in patients with paraplegia, and in premature children with CP.
Athetotic cerebral palsy was diagnosed in a 6-month-old boy with no history of perinatal trauma. Lesch-Nyhan syndrome (i.e., complete deficiency of hypoxanthine-guanine phosphoribosyltransferase [HGPRT] ) was diagnosed only when the boy began biting his lower lip at the age of 10 years. It is suggested, on the basis of this case and others like it in the literature, that the delayed onset or absence of self-mutilation in patients with Lesch-Nyhan syndrome may be more common than has been previously suspected. In all males said to have cerebral palsy, HGPRT deficiency must be ruled out, preferably by measuring the ratio of uric acid to creatinine in a random urine specimen.
Two hundred and eight-two children with cerebral palsy (C.P.) and thirty-seven normal children were studied by computerized tomography (C.T.) of the head for finding out the correlations between the organic damage of the brain and the motor developmental disturbance. The abnormal findings of C.T. were: enlargement of the ventricular system, high density area, low density area and porencephalus, enlargement of the sulcus and anomaly of the medial structure. Enlargement of the ventricular system seemed to have correlation with spasticity; the portion and the extent of the enlargement corresponded to the affected extremities and the severity of the spasticity. Children of other types also showed various abnormal C.T. findings but, in general, less than that of spastic types. The prognosis of the motor development of C.P. children cannot be predicted by serial C.T. examinations strictly, because early treatment could cause improvement to that of these children. However, it is of worthy notice that C.T. is an effective method of helping to diagnose the motor developmental disturbance in earlier childhood.
In an attempt to define prognostic indicators of intelligence and seizures in the hemiparetic cerebral palsy population, birth histories, electroencephalograms, and computerized tomographic (CT) scans were reviewed in 52 children with hemiparetic cerebral palsy. Cases were excluded when the hemiparesis might have been related to events beyond the neonatal period, such as meningitis, trauma, focal seizures, tumor, or vascular insults. Detailed neurological histories and examinations were done in all patients. Intellectual evaluations, electroencephalograms, and CT scans were obtained. There was no significant relationship of birth history to subsequent seizure development or mental retardation. In contrast, findings in electroencephalograms and CT scans correlated well with the development of seizures and abnormal intelligence. Children who shared anatomical abnormalities of commissural pathways, association pathways, or cerebral cortex were found to have a much higher incidence of seizures and abnormal intelligence.
The adequacy of biting, sucking, swallowing, and chewing as well as the presence or absence of nine infantile oral reflexes were assessed in 60 cerebral-palsied individuals. The effect of the asymmetric tonic neck reflex and the Moro reflex on the infantile oral reflexes was also studied. There was a trend for subjects with more adequate feeding skills to achieve higher levels of overall speech proficiency and articulatory competency, but this trend was not completely systematic. Fifteen subjects displayed abnormal oral reflexes, but these reflexes could not be elicited consistently. The effect of the ATNR and Moro reflex on the oral reflexes seemed limited. Results generally supported the value of the widespread prescription of improving feeding in cerebral palsy, but the need to modify abnormal oral reflexes received less support. The question of using prespeech oromotor training to reduce possible future dysarthria is discussed in terms of the findings.
The present study attempted to investigate the relationship between judged speech proficiency of cerebral palsied speakers and the following quantitative speech parameters: 1) the number of articulatory errors; 2) the intelligibility of the speech of the cerebral palsied; 3) mean vocal fundamental frequency; 4) variation of vocal fundamental frequency; 5) mean speech-sound pressure level; 6) variation of speech-sound pressure level; 7) word-per-minute speech rate. Secondarily, the study investigated the differences in the speech of the spastic and athetoid populations in terms of the parameters listed above.
Cranial magnetic resonance imaging (MRI) was performed prospectively in 45 children (ages 3-27 months) with clinically documented motor delay to evaluate the ability of MRI to determine etiologic factors, to determine whether myelination correlated with motor delay, and whether the clinical category corresponded with the imaging findings. Of the 22 children diagnosed clinically as having major motor delay (i.e., cerebral palsy), 77% had magnetic resonance imaging abnormalities. In 23%, etiologic associations were established from MRI alone and in 32% a clinically suspected etiology was supported. No children had myelination delay as the sole abnormality. In 23 children with minor motor delay, only 17% had abnormal scans. Clearly, MRI provided useful information in the majority of children with cerebral palsy; therefore, a classification system is proposed in which MRI can be used in conjunction with clinical assessment to specify more precisely the etiologic factors in cerebral palsy.
The authors used MRI to analyse retrospectively the brain images of patients with cerebral palsy (CP) to evaluate its the role in the assessment of brain abnormalities and injury, and the relationship of pre‐, peri‐ and postnatal events to CP. 70 patients with CP aged two to 16 years who underwent MRI were divided into four groups: group 1 (26 patients) comprised subjects whose CP was considered to have been caused by neuronal migration disorders in the embryonal stage; group 2 (30 patients) contained subjects whose cause was vascular disorders; in group 3 patients (five) the cause was intra‐uterine infection; and CP clearly attributable to birth asphyxia (group 4) was noted in only nine patients. The results indicate that CP of term infants is often the result of prenatal factors, and their MRI findings indicated migration and cerebral infarction. Brain MRI is an essential examination in identifying the factors causing brain damage in CP.
RÉSUMÉ
Quand les anomalies cérébrates de l'IMC survienncnt‐elles? Etude des IRM de 70 patients Les auteurs ont utilisé l'IRM pour analyser rétrospectivement les images cérébrales d' IMC, pour apprécier dans la genése, les anomalies et lésions cérébrales, et la relation avec des événcments pré‐, péri‐ et post nataux sur l' IMC 70 LM.C âgés de deux à 16 ans ayant bénéficié d' une IRM furent répartis en cjuatre groupes: le groupe 1 (26 patients) comprenait des sujels dont l' IMC était considéréé comme causée par un trouble de la migration neuronale durant le développcment embryologique; le groupe 2 (30 patients) comprenait des sujets chez qui était invoqué un trouble vasculaire; dans le groupe 3 (cinq patients), la cause était une infection intra‐utérine et une LM.C. manifestement atlribuable à une asphyxie à la naissance (groupe 4) ne Cut observée que pour neuf patients. Ces résultats indiquent que l'IMC d'enfants nés à terme est souvent le résultat de facteurs prénalaux, et leurs IRM indiquent alors un trouble de migration ou un infarctus cérébral. l' IRM est un examen essentiel pour identifier les facteurs causant le dommage cérábral dans l' IMC
ZUSAMMENFASSUNG
Wann treten llirnanomalicn bei Cerebralparese auf? Eine MRI sludie bei 70 Patienlen
Die Autoren haben retrospektiv die MRI Aufnahmcn von Patienten mil Cerebralparese (CP) analysicrt, urn ihre Bedeutung für die Beurteilung von Hirnänonialien und ‐verletzungen, sowie die Relatiun von prä‐ peri‐ und postnatalen Eintlüssen auf die CP zu untersuchen. 70 Patienten mit Cerebralparese. im Alter von zwei bis 16 Jahren, bei denen ein MRI gemacht worden war, wurden in vier Gruppen eingeteilt: Gruppe 1 unifaßte 26 Putienien, deren CP durch neuronale Migrationsstörungen im Umbryonalstadium bedingt war: Gruppe 2 hatte 30 Patienten, bei denen die Ur. sache für die CP Gefäßerkrankungen waren; Gruppe 3 umfaßte fünf Patienten, bei denen eine intrauterine Infektion vorlag; und in der Gruppe 4 waren neun Patienten. deren CP eindeutig auf eine Geburtsasphyxie zurückzuführen war. Die Hrgcbnissc zeigen, daß CP bei reifen Neugeborenen oft durch pränatule Faktoren bedingt ist und auf thren MRI Aufnahmen fanden sich Migration und Hirninfarkt. Das MRI des Gehirns ist eine wesentliche Untersuchung zur Differenzierung der Ursachen von Himschädigungen bei CP.
RESUMEN
Cuandu tienen lunar las anomalias cerebrates en la pardslisis cerebral? Estudio can IRM del cerebro de 70 pacientes
Los autores usaron la IRM para analizar restrospectivamente las imágenes cerebrates de pacientes con parálisis cerebral (PC) para evaluar su papel en la evaluación de las anomalías y lesiones cerebrales y las rclaciones de los acontccimientos pre‐ peri‐ y postnatales con la PC. 70 pacientes con PC de dos a 16 años de edad examinados con IRM, se dividieron en cuatro grupos: grupo 1 (25 pacientes), comprendió los sujetos cuya PC se consideró debida a alteraciones de la migración cerebral en el estadio embrionario; grupo 2 (30 pacientes), con sujetos cuya causa era vascular; grupo 3 (cinco) cuya causa era una infección intrauterina; y un grupo 4 en que la PC era claramente atribuible a una usfixia del parto (sólo en 9 casos). Los resultados indican que la PC en lactantcs a término es a menudo el resultado de factores prenatales y los hallazgos en sus IRM indican alteraciones en la migración e infarto cerebral. La IRM cerebral es una exploración esencial para identificar los factores causantes de una alteraeión cerebral.
MRI of the brain was performed on 56 children with bilateral spastic cerebral palsy (CP) at a mean age of 10.7 years. Specific pathology was found in 91 per cent; periventricular leukomalacia was present in 42 per cent of term‐ and H7 per cent of preterm‐born children. Parasagittal subeortico‐cortical injury, multicystie encephalomalacia and basal ganglia lesions were identified in 16 per cent, in all but one associated with severe peri‐/neonatal events at term or near term. Maldevelopment composed 9 per cent, all but one found in term‐born children. MRI morphology correlated strikingly with outcome. Periventricular leukomalacia was associated with more severe disability in term‐ than preterm‐born children.
RÉSUMÉ
Une forme spasticpie bilatérale: pathologic IRM el olivine. Analyse d' une série représentative de 56 cas
Une IRM du ccrveau a été effectuée chez 56 enfants IMC présentant une forme spastique bilatérale, á l'àge moyen de 10.7 ans. Une imagerie pathologique spécifique fut observée dans 91 pour cent des cas; une leucomalacie était présente chez 42 pour cent des enfants nés à terme et 87 pour cent des enfants nés prémalurément. Une lésion parasagittal sous‐cortico‐corticale. une encéphalomalacie multicystique el des lésions des noyaux gris furent identifyées dans 16 pour cent des cas, associées dans tous les cas sauf un, à des événements graves péri‐néonataux à terme ou près du term. Un mauvais développement fut trouvé dans neuf pour cent des cas. toujours sauf une fois, chez des enfants nés à terme. La morphologic IRM était corrélée de façon frappante avec le devenir. La leucomalacie périventriculairc était associée à une incapacité plus sévère chez. les enfants nés à terme, que chez les prématurés.
ZUSAMMENFASSUNG
Bilaterale spastischc Cercbralparvse: pathologische MRl‐Befunde und tlcrcn Ursachc. Analyse einer repräsenlativen Uniersuchungsreihe von 56 Fällen
Bei 56 Kinderen mit bilateraler spastischer Cerebralparese (CP) wurde im mittleren Alter von 10.7 Jahren ein MRI des Gehirns gemacht. 91 Prozent wiesen cine spezifische Pathologie auf; 42 Prozent der rcitgeborcnen Kinder und 87 Prozent der Frühgehorenen hatten eine periventrikuläre Leukomalazie. Parasagittal subcortico‐corticale Schädigungen, multizystische Enzephalomalazie und Läsionen der Basalganglien wurden bei 16 Prozent nachgewiesen und waren bei alien außer einem Patienten mit schweren peri‐ und/oder neonatalen Störungen in Verbindung zu bringen. Eine Fehlentwicklung fand sich in neun Prozent der Fälle. die alle außer einem zum Termin geboren worden waren. Die MRI‐Befunde korrelierten auffallend mit dem Outcome. Die periventrikuläre Leukumaiazic war bei den reifgeborenen Kindern mit schwcroren Behinderungen verbunden als bei den Frühgeborenen.
RESUMEN
Parálisis cerebral espástica bilateral: palologia IRM y arisen. Aná lists de una serie reprcsentativa de 56 casos
Se realizó una IRM cerebral en 56 niños con parálisis cerebral espastica bilateral (PC) con una edad de promedio de 10.7 años. Fn el 91 por ciento de los casos se halló una patología especifíca; la leucomalacia periventricular estaba presente en el 42 por ciento de los casos a término y en el 87 por ciento de los pretermino. En el 16 por ciento se identificó una lesión parasagital subcorticocortical. encefalomalacia multiquistica y lesiones en los ganglios basales, asociados en todos los casos menos uno con graves acontecimientos peri‐neonalales al término o cerca de él. Las alteraeiones de dcsarrollo comprendieron el 9 por ciento, todos los casos exeepto uno en niños a término. La morfología de la IRM se correlacionaba notableiuente con el curso. La leucomalacia periventricular estaba asociada a una mayor incapacidad en los niños a término, que en los pretérmino.
The hypothesis of unilateral left hemisphere damage as an explanatory model for the neurological basis of specific language impairment (SLI) does not appear to be sufficient for most children with SLI. Children with unilateral brain lesions have been shown to function significantly lower than their neurologically intact peers on a variety of language measures, yet few of the deficits noted are as persistent or severe as those seen in SLI. In at least two instances, however, language symptomatology following unilateral lesions in children does parallel some types of SLI. The first occurs following subcortical damage to anterior grey and white matter structures that typically results in pronounced language and learning disorders. The second parallel lies in the similar developmental course shared by children with "delayed" language and children with known unilateral lesions, whereby language onset and development is slow in the preschool years but normalizes by school age, with minimal long-term language-learning deficits.
Glutaric aciduria type I (GA-I) is an inborn error in the degradation of lysine, hydroxylysine, and tryptophan due to a deficiency of glutaryl-CoA dehydrogenase. Glutaric, 3-OH-glutaric, and glutaconic acids are excreted in the urine, particularly during intercurrent illness. The enzyme may be assayed in leukocytes, cultured fibroblasts and chorionic villi. Twelve new cases, 9 months-16 years of age, are reported, comprising all known cases of GA-I in Sweden and Norway. Ten had a severe dystonic-dyskinetic disorder, one had a mild hyperkinetic disorder, and one was asymptomatic. Two children died in a state of hyperthermia. Carnitine deficiency and malnutrition developed in patients with severe dystonia and dysphagia, which necessitated substitution and gastrostomy. A slowly progressive dyskinetic disorder developed in spite of adequate early dietary treatment in one subject. Macrocephaly was found in three. Computed tomography and magnetic resonance investigations in 10 showed deep bitemporal spaces in 7. Neuropsychological testing of 8 of 12 subjects demonstrated receptive language function to be superior to expressive language and motor function. Cognitive functions were obviously less affected than motor functions. A review of 57 pooled cases showed that a severe dystonic syndrome developed in 77%, a mild extrapyramidal syndrome in 10%, and 12% were asymptomatic. This disorder may pass undetected in the cerebral palsy and mentally retarded child and adult populations. Repeated urine examinations of organic acids in the urine and enzyme assay may be necessary to confirm GA-I.
The Metropolitan Atlanta Developmental Disabilities Study was a population-based study (1985 through 1987) to determine the prevalence of five developmental disabilities among 10-year-old children. The disabilities included cerebral palsy, mental retardation, visual impairment, hearing impairment, and epilepsy. The prevalence of cerebral palsy (CP) and a description of the children with CP are reported here. Using a record review approach, we identified 204 10-year-old children with CP (resulting in a prevalence of 2.3 per 1000). The rate of CP was significantly higher among boys (prevalence odds ratio = 1.5; 95% confidence interval = 1.1, 2.0), and the rate was also higher among black children than white children (prevalence odds ratio = 1.3; 95% confidence interval = 1.0, 1.7). Thirty-three of the children (16%) acquired CP postnatally; these children were more likely to be black or male. The gender and racial differences found for acquired CP were greater than those for congenital CP. Approximately 75% of the children had one of the other four disabilities studied; 65% of the children were mentally retarded, 46% had epilepsy, and 15% had a sensory impairment. Our multiple-source method of identifying children with CP gave us a population-based sample from which to determine the prevalence of the condition and to study factors that are associated with CP.
The magnetic resonance examination was performed in 38 patients with severe cerebral palsy (CP; 15 males and 23 females) who had both motor delay (unable to move anywhere) and mental retardation (I. Q or D. Q below 30). Neuroimaging findings were compared with the CP type, etiology, and grade of understanding of language. Cranial magnetic resonance imagings (MRI) in CP were divided into five types. Type 1 : nine predominantly showed cyst-liked ventricles and periventricular hyperintensity on T2-weighted imaging (PVH) and only scarred basal ganglia and thalamus were visible. All suffered from neonatal asphyxia and the clinical type was rigospastic tetraplegia (RST). Type 2: eleven predominantly showed PVH and hyperintensity on T2-weighted (HT2) in basal ganglia and thalamus. All suffered from neonatal asphyxia and the clinical type was RST or rigospastic diplegia. Type 3: five showed PVH and three had cortical atrophy. All suffered from neonatal asphyxia and the clinical type was spastic diplegia. Type 4: four predominantly showed HT 2 in putamen and thalamus. Three had cortical atrophy. All suffered from neonatal asphyxia. The clinical type was athetotic CP (ATH). Type 5: nine predominantly showed HT 2 in globus pallidus. Four had cortical atrophy and two had hippocampal atrophy. All suffered from neonatal jaundice and the clinical type was ATH. All patients who suffered from neonatal asphyxia and spastic CP had MRI in PVH. All patients who suffered from neonatal asphyxia and ATH showed HT 2 in putamen and thalamus. Almost patients who suffered neonatal jaundice and ATH showed HT 2 in globus pallidus. With athetotic CP, cases with atrophy of the cerebral cortex or/and hippocampus were lower grade of understanding of language than no atrophy of both. The result of studies of MRI are in agreement with neuropathological findings.
A girl with ornithine transcarbamylase deficiency had a history of recurrent strokelike episodes. The differential diagnosis of unexplained stroke should include primary urea cycle defects.
The MR findings in patients with spastic diplegia were investigated and the role of MR imaging in assessing the extent of brain injury was evaluated.
39 male and 24 female patients (preterm/term 43/20) were imaged using a 0.5 T MR system.
The MR findings in term patients were quite different from those in preterm patients; 55% of the term patients showed normal and minimal changes on MR, whereas 90.7% of the 43 preterm children had periventricular leucomalacia. The deep cerebral white matter was the most frequently involved site. Objective measurements revealed significant reductions of the entire sagittal area of corpus callosum in diplegic patients in comparison with normal controls. The motor palsy severity correlated well with the extent of corpus callosum involvement.
The corpus callosum appears to be a sensitive marker site for the assessment of the extent of white matter injury.
To determine the prevalence and nature of feeding difficulties and oral motor dysfunction among a representative sample of 49 children with cerebral palsy (12 to 72 months of age).
A population survey was undertaken by means of a combination of interview and home observational measures.
Sucking (57%) and swallowing (38%) problems in the first 12 months of life were common, and 80% had been fed nonorally on at least one occasion. More than 90% had clinically significant oral motor dysfunction. One in three (36.2%) was severely impaired and therefore at high risk of chronic undernourishment. There was a substantial discrepancy between the lengthy duration of mealtimes reported by mothers and those actually observed in the home (mean, 19 minutes 21 seconds; range, 5 minutes 21 seconds to 41 minutes 39 seconds). In 60% of the children, severe feeding problems preceded the diagnosis of cerebral palsy.
Using a standardized assessment of oral motor function, we found the majority of children to have clinically significant oral motor dysfunction. Contrary to maternal report, mealtimes were relatively brief, and this, combined with the severity of oral motor dysfunction, made it difficult for some children to achieve a satisfactory nutritional intake. The study illustrates the importance of observing feeding, preferably in the home.
The patterns of motor and sensory impairments, disabilities and handicap were assessed in 217 children and adolescents with epilepsy. Motor and sensory dysfunctions were found to be common even in children without major additional neurodisorders such as cerebral palsy and mental retardation. Handicap was most severe in the dimensions of physical independence and orientation and increased significantly with duration of epilepsy. It was more severe when the onset of seizures was early and when secondary generalized seizures were present. Handicap was significantly reduced after epilepsy surgery.
The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. Eighty-one patients had diplegia, 45 had quadriplegia, and 26 had hemiplegia. Of patients with diplegia, 72 had periventricular leukomalacia (PVL) and very few had other types of lesions. In patients with quadriplegia, three main types of brain lesions were observed: PVL in 12 patients, term-type brain injury in 22, and brain anomaly in 10. In the 26 patients with hemiplegia, 17 had a unilateral lesion (rare in patients with diplegia and quadriplegia), and bilateral lesions were seen in seven others.
Activated protein C resistance caused by an Arg506Gln mutation in the factor V gene (factor V Leiden mutation) is the most common cause of familial thrombosis. This mutation is associated with arterial and venous thromboembolic disease in neonates, infants, and children, but is not a significant risk factor for ischemic stroke in adults. We report on 3 babies with different neonatal cerebrovascular disorders including ischemic infarction and hemorrhagic stroke who are heterozygous for factor V Leiden mutation. One infant had multiple thrombi in the fetal placental vasculature. This is the first reported association between hemiplegic cerebral palsy, placental thrombosis, and factor V Leiden mutation. We suspect that activated protein C resistance may be an important cause of in utero cerebrovascular disease and hemiplegic cerebral palsy.
Argininemia, a rare autosomal recessive urea cycle disorder, is caused by a deficiency of arginase, with resulting elevated plasma arginine and ammonia levels. Reports to date have focused little on the neurology of this disorder or the efficacy of treatments. A MEDLINE search revealed 25 previously reported cases, to which we have added two brothers who presented with late onset progressive spastic diplegia. Though their degree of enzyme deficiency was comparable, the severity of their phenotypic abnormalities differed substantially. With dietary therapy, both showed improved cognitive and motor function. Late metabolic crises occurred in both, resulting in death of the less severely affected brother. Based on analysis of our clinical database, we report on the full spectrum of neurologic abnormalities seen in argininemia with particular focus on the accompanying progressive spastic diplegia and its response to treatment; progressive decline in head growth; distinctive neuroradiologic findings; and life-threatening later complications. Current and potential future therapies and long-term outcome are summarized.