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Recovery in Gross Motor Function Classification Scale (GMFCS) and Gross Motor Function Measure (GMFM) of 12 children
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Using the International Classification of Functioning Disability and Health (ICF) (WHO, 2001), impairments, activities and social participation are reported in 12 children (mean age at surgery 5.9 years) who were investigated before and three times over a 2-year period after hemispherectomy. Impairments were assessed (i) in terms of seizure frequen...
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Context 1
... GMFCS scores had improved statistically significantly 2 years after surgery compared with before surgery (P < 0.05) ( Table 2). ...
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Citations
... We suppose that short-time periods are not enough to reach gains in QOL. Therefore, QOL assessments are, in addition to seizure control, an indirect approach to verify the effectiveness of surgery [8, 9,13,[19][20][21]. ...
Purpose: To evaluate QOL and caregiver burden of children and teenagers submitted to hemispherotomy for pharmacoresistant epilepsy, by comparing pre and post surgical intervention data.
Materials and methods: Retrospective analysis of pediatric patients submitted to surgical hemispherotomy before intervention (preOP) and their follow-up at 6 months (6M PO) and 2 years (2Y PO) after surgery. QOL was evaluated through the Quality of Life in Childhood Epilepsy (QVCE-50) questionnaire and caregiver burden, through the Zarit Burden Interview (ZBI) tool.
Results: Twenty-two patients were included in the study. Sixteen patients (72%) were classified as Engel I at 2Y PO follow-up. QVCE-50 scale showed improvement of total QOL at 2Y PO. In relation to QVCE-50 specific domains, there was an improvement in the physical domain and in the cognitive-education; a decrease in psychological and a stabilization in social/familiar domain scores. The majority of caregivers classified their burden as mild to moderate, with no PO improvement.
Conclusions: Hemispherotomy represents an effective seizure control treatment, as well as it contributes to improvement of QOL, particularly in the physical domain and in spite of children's physical and cognitive limitations. However, no improvement in caregiver burden was observed, probably due to the chronic condition of these patients, which might be worsened by social issues.
... This permitted an examination of the approximate competence of a single hemisphere across the two groups by comparing accuracy of a patient's single hemisphere to a control's single hemisphere. Trial accuracy (the binary response) was the primary variable of interest, as reaction time (RT) may be confounded by motor impairments in the patients (35,36). Furthermore, throughout the text, patients with a preserved LH will be referred to as "LH patients" and patients with a preserved RH as "RH patients." ...
... English-speaking patients with extensive cortical resection [childhood hemispherectomy or hemispherotomy, per participant and/or guardian report; see Kim et al. (35) for distinctions between surgery types] were recruited primarily with the assistance of the Brain Recovery Project: Childhood Epilepsy Surgery Foundation. While right handers typically evince more reliable LH language lateralization than left handers (62), native handedness could not be established in patients given their contralesional hemiparesis (35,36). The sample (including all participants from experiments 1 and 2) included a total of 40 patients, 16 with a preserved left hemisphere (LH patients; median age = 17.9 y, MAD of age = 6.1 y; 12 females, 4 males) and 24 with a preserved right hemisphere (RH patients; median age = 15.3, ...
The right and left cerebral hemispheres are important for face and word recognition, respectively—a specialization that emerges over human development. The question is whether this bilateral distribution is necessary or whether a single hemisphere, be it left or right, can support both face and word recognition. Here, face and word recognition accuracy in patients (median age 16.7 y) with a single hemisphere following childhood hemispherectomy was compared against matched typical controls. In experiment 1, participants viewed stimuli in central vision. Across both face and word tasks, accuracy of both left and right hemispherectomy patients, while significantly lower than controls' accuracy, averaged above 80% and did not differ from each other. To compare patients' single hemisphere more directly to one hemisphere of controls, in experiment 2, participants viewed stimuli in one visual field to constrain initial processing chiefly to a single (contralateral) hemisphere. Whereas controls had higher word accuracy when words were presented to the right than to the left visual field, there was no field/hemispheric difference for faces. In contrast, left and right hemispherectomy patients, again, showed comparable performance to one another on both face and word recognition, albeit significantly lower than controls. Altogether, the findings indicate that a single developing hemisphere, either left or right, may be sufficiently plastic for comparable representation of faces and words. However, perhaps due to increased competition or “neural crowding,” constraining cortical representations to one hemisphere may collectively hamper face and word recognition, relative to that observed in typical development with two hemispheres.
... Additionally, because nearly all patients will develop spastic hemiplegia on the contralateral side of the resected hemisphere, HS is preferred for patients with hemiparesis contrary to the side of the intracranial lesion. [55][56][57] However, the decision to perform surgery for patients without apparent motor defects or cognitive decline should be made cautiously. 5,46 For 25 patients without motor defects or cognitive decline, a prolonged period (24 months) of AED medication treatment was provided to ensure its refractory nature. ...
Few studies have reported the clinical presentation, surgical treatment, outcomes, and influential factors for patients with epilepsy and Sturge-Weber syndrome.
This large-scale retrospective study continuously enrolled 132 patients with Sturge-Weber syndrome and epilepsy from January 2008 to December 2018 at our hospital to analyze their characteristics. Among these patients, 90 underwent epilepsy surgery, and their postoperative 2-year follow-up seizure, cognitive, and motor functional outcomes were assessed and analyzed. Univariable and multivariable logistic analyses were conducted to explore the influential factors.
Among the Sturge-Weber syndrome patients for whom characteristics were analyzed (n = 132), 76.52% of patients had their first epileptic seizures within their first year of life. The risk factors for cognitive decline were seizure history≥2 years (adjusted odds ratio [aOR] = 3.829, 95% confidence interval [CI]: 1.810-9.021, p = 0.008), bilateral leptomeningeal angiomas (aOR = 3.173, 95% CI: 1.970-48.194, p = 0.013), age at onset < 1 year (aOR = 2.903, 95% CI: 1.230-6.514, p = 0.013), brain calcification (aOR = 2.375, 95% CI: 1.396-5.201, p = 0.021) and left leptomeningeal angiomas (aOR = 2.228, 95% CI: 1.351-32.571, p = 0.030). Of the patients who underwent epilepsy surgery (n = 90), 44 were subject to focal resection, and 46 underwent hemisphere surgery (19 anatomical hemispherectomies and 27 modified hemispherotomies). A postoperative seizure-free status, favorable cognitive outcomes, and favorable motor outcomes were achieved in 83.33%, 44.44%, and 43.33% of surgical patients, respectively. The modified hemispherotomy group had similar surgical outcomes, less intraoperative blood loss and shorter postoperative hospital stays than the anatomical hemispherectomy group. Regarding seizure outcomes, full resection (aOR = 11.115, 95% CI: 1.260-98.067, p = 0.020) and age at surgery < 2 years (aOR = 6.040, 95% CI: 1.444-73.367, p = 0.031) were positive influential factors for focal resection. Age at surgery < 2 years (aOR = 15.053, 95% CI: 1.050-215.899, p = 0.036) and infrequent seizures (aOR = 8.426, 95% CI: 1.086-87.442, p = 0.042; monthly vs. weekly) were positive influential factors for hemisphere surgery.
In conclusion, epilepsy surgery resulted in a good postoperative seizure-free rate and favorable cognitive and motor functional outcomes and showed acceptable safety for patients with epilepsy and Sturge-Weber syndrome. Modified hemispherotomy is a less invasive and safer type of hemisphere surgery than traditional anatomic hemispherectomy with similar surgical outcomes. Early surgery may be helpful to achieve better seizure outcomes and cognitive protection, while the risk of surgery for young children should also be considered.
... Worsening or new gross motor deficits occurred after surgery in approximately half of patients [6,11,30,47]. In the proximal upper and lower extremity, these deficits recovered to baseline by 12 months but fine motor control of the affected hand did not [6,44]. A 20% total improvement in the Gross Motor Function Measure has been shown at 2 years after surgery [44]. ...
... In the proximal upper and lower extremity, these deficits recovered to baseline by 12 months but fine motor control of the affected hand did not [6,44]. A 20% total improvement in the Gross Motor Function Measure has been shown at 2 years after surgery [44]. New dysphagia developed in 25.6% of patients and prior dysphagia worsened in 54.5%, requiring nasogastric feeding in 62.5% and conversion to gastrostomy in 13.3% [4]. ...
... Preoperative motor deficits worsen in approximately half of patients after surgery; most recover by 1 year and improve from pre-operative status by 2 years [7,29,44] with the exception of fine motor control of the contralateral hand [6]. Ambulation and gross motor function of the legs are spared due to the locomotive pathways from the brainstem, cerebellum, and spinal cord [12,49]. ...
Purpose
Several variations of functional disconnection surgery have been described for the treatment of lateralized, hemispheric, drug-resistant epilepsy in children. The purpose of this study is to investigate the existing literature regarding patient selection, approach, and outcomes after hemispherotomy.
Methods
A systematic review of the English literature through February 2019 was performed in accordance with the PRISMA statement. The articles were classified by level of evidence and summarized in an evidentiary table. Seizure outcomes, functional outcomes, surgical techniques, complications, and patient selection were critically analyzed.
Results
A total of 173 papers were reviewed, of which 37 met criteria of inclusion and exclusion. Thirteen studies were classified as level III evidence, the remaining reached level IV. Vertical and lateral hemispherotomy achieve similar rates of seizure freedom and functional outcomes, though parasagittal and interhemispheric approaches may have shorter operative times and less blood loss. Etiology, bilateral MRI abnormalities, and nonlateralizing EEG did not predict worse seizure or functional outcomes.
Conclusions
Both vertical and lateral hemispherotomy approaches result in durable, reproducible benefits to epilepsy severity and functional status in appropriately selected pediatric patients.
... Regarding the paretic leg movement, our patients maintained lower limb function and locomotor control after surgery, except for Case 1 who slightly worsened. This is in line with previous studies showing that hand movements, especially finger movements, are subject to a stronger modulation of cortical control, while locomotion is more under the control of subcortical regions such as brainstem, cerebellum and spinal neuronal circuits (12,(35)(36)(37). In this study it is difficult to establish whether the re-organized motor circuit involves the ipsilateral corticospinal pathway or extra-pyramidal pathways. ...
Objective: Mechanisms of motor plasticity are critical to maintain motor functions after cerebral damage. This study explores the mechanisms of motor reorganization occurring before and after surgery in four patients with drug-refractory epilepsy candidate to disconnective surgery.
Methods: We studied four patients with early damage, who underwent tailored hemispheric surgery in adulthood, removing the cortical motor areas and disconnecting the corticospinal tract (CST) from the affected hemisphere. Motor functions were assessed clinically, with functional MRI (fMRI) tasks of arm and leg movement and Diffusion Tensor Imaging (DTI) before and after surgery with assessments of up to 3 years. Quantifications of fMRI motor activations and DTI fractional anisotropy (FA) color maps were performed to assess the lateralization of motor network. We hypothesized that lateralization of motor circuits assessed preoperatively with fMRI and DTI was useful to evaluate the motor outcome in these patients.
Results: In two cases preoperative DTI-tractography did not reconstruct the CST, and FA-maps were strongly asymmetric. In the other two cases, the affected CST appeared reduced compared to the contralateral one, with modest asymmetry in the FA-maps. fMRI showed different degrees of lateralization of the motor network and the SMA of the intact hemisphere was mostly engaged in all cases. After surgery, patients with a strongly lateralized motor network showed a stable performance. By contrast, a patient with a more bilateral pattern showed worsening of the upper limb function. For all cases, fMRI activations shifted to the intact hemisphere. Structural alterations of motor circuits, observed with FA values, continued beyond 1 year after surgery.
Conclusion: In our case series fMRI and DTI could track the longitudinal reorganization of motor functions. In these four patients the more the paretic limbs recruited the intact hemisphere in primary motor and associative areas, the greater the chances were of maintaining elementary motor functions after adult surgery. In particular, DTI-tractography and quantification of FA-maps were useful to assess the lateralization of motor network. In these cases reorganization of motor connectivity continued for long time periods after surgery.
... Following HS, remarkable residual behavioral functions have been reported as unchanged or even improved, not only in infants but also in adults, with the recovery being greater for early-lesioned subjects (Bell and Karnosh, 1949;Krynauw, 1950;Cairns and Davidson, 1951;Johnson, 1955a, 1955b;Gardner et al., 1955;Obrador, 1964;Ueki 1966;Van Buren and Fedio, 1976;Wilson, 1970;Zülch, 1974;Damásio et al., 1975;Zülch and Micheler, 1978;Ameli, 1980;Glees, 1980;Verity et al., 1982;Beardsworth and Adams, 1988;Müller et al., 1991;Beckung et al., 1994;Peacock et al., 1996;Vargha-Khadem et al., 1997;Devlin et al., 2003;Jonas et al., 2004;van Empelen et al., 2004;Basheer et al., 2007;Terra-Bustamante et al., 2007;Limbrick et al., 2009;Barras et al., 2010). The experimental animal model studies reported here have been performed principally in the Rat and Cat. ...
... In particular, a functional network connecting the IPC, PMC and SMA, as well as the existence of ipsilateral projections originating from these regions may explain why these areas are predominantly involved in reorganization confined to a single hemisphere. Notably, infant-lesioned monkeys (Burke et al., 2010) and humans (Devlin et al., 2003;van Empelen et al., 2004;Ptito and Leh, 2007) may show different degrees of functional recovery between upper and lower limbs. This might be due to the involvement of different networks of interneurons within the spinal cord in primates. ...
... This might be due to the involvement of different networks of interneurons within the spinal cord in primates. In fact, while the purposeful movements of upper limbs are under the control of corticospinal pathways, repetitive movements of gait are thought to be under the control of neuronal circuits of the central pattern generator, within the spinal cord (Devlin et al., 2003;van Empelen et al., 2004). The mechanisms underlying visual recovery from massive cortical lesions that include all visual cortical areas of one cerebral hemisphere (as in HS) also remain unclear. ...
Hemispherectomy (HS) is an effective surgical procedure aimed at managing otherwise intractable epilepsy in cases of diffuse unihemispheric pathologies. Neurological recovery in subjects treated with HS is not limited to seizure reduction, rather, sensory-motor and behavioral improvement is often observed. This outcome highlights the considerable capability of the brain to react to such an extensive lesion, by functionally reorganizing and rewiring the cerebral cortex, especially early in life. In this narrative review, we summarize the animal studies as well as the human neurophysiological and neuroimaging studies dealing with the reorganizational processes that occur after HS. These topics are of particular interest in understanding mechanisms of functional recovery after brain injury. HS offers the chance to investigate contralesional hemisphere activity in controlling ipsilateral limb movements, and the role of transcallosal interactions, before and after the surgical procedure. These post-injury neuroplastic phenomena actually differ from those observed after less extensive brain damage. Therefore, they illustrate how different lesions could lead the contralesional hemisphere to play the "good" or "bad" role in functional recovery. These issues may have clinical implications and could inform rehabilitation strategies aiming to improve functional recovery following unilateral hemispheric lesions. Future studies, involving large cohorts of hemispherectomized patients, will be necessary in order to obtain a greater understanding of how cerebral reorganization can contribute to residual sensorimotor, visual and auditory functions.
... Functional hemispherectomy is a surgical method with a high rate of seizure reduction in patients with intractable epilepsy related to hemi-malformations of cortical development or infantile brain damage with large hemispheric defects [1][2][3][4][5][6]. However, the prediction of the functional outcome after surgery is challenging. ...
... Currently, clinical features such as time interval of the insult and the type of injury are taken into consideration to estimate postoperative motor function [4,8,43]. Clinical experience in most cases suggests that either the affected pyramidal tract contains no functional axons or compensatory mechanisms of the contralateral side succeed [8,9,11]. ...
Functional hemispherectomy (FH) is an infrequent method to reduce seizure frequency in patients with intractable epilepsy. The risk that hemispherotomy injures brain structures involved in residual motor function is challenging to predict. Our purpose was to evaluate MR diffusion tensor imaging (DTI) to preoperatively assess residual ipsilateral motor function prior to FH.
We applied DTI in 34 patients scheduled for FH to perform fiber tracking in healthy and damaged hemispheres of the corticospinal tracts (CSTs) and of the corpus callosum. We assessed the CSTs and the commissural fibers for streamline count, for fractional anisotropy (FA), and for respective ratios (affected/unaffected side). We correlated these DTI values to post-to-prior changes of muscle strength and evaluated their diagnostic accuracy.
FA of the affected CSTs and of commissural fibers was significantly higher in patients with postoperative loss of muscle strength compared to patients without (p = 0.014 and p = 0.008). In contrast, CST FA from healthy hemispheres was not different between both groups. Ratios of streamline counts and FA from CSTs were higher in patients with postoperative reduced muscle strength compared to those without (1.14 ± 0.22 vs. 0.58 ± 0.14, p = 0.040; 0.93 ± 0.05 vs. 0.74 ± 0.03, p = 0.003). CSTs' normalized FA ratio greater than -0.085 predicted loss of muscle strength with 80 % sensitivity and 69.6 % specificity.
Preoperative tracking of the CST and of commissural fibers contributes to the prediction of postoperative motor outcome after functional hemispherectomy.
... More importantly, hemispherotomy does not seem to induce any obvious cognitive or neurodevelopmental damage [20][21][22]. There may even be gains in social functioning after hemispherotomy [4,23]. Thus, there is a discrepancy between cognition, which does not seem to improve significantly, and social functioning, which seems to improve. ...
Explore the long-term life situation for Swedish hemispherotomy patients reporting not only seizure outcome but also patients' perspectives on function, quality of life (QoL) and satisfaction with the surgery.
This population based study uses prospectively collected data from the Swedish National Epilepsy Surgery Register. An independent researcher interviewed patients or parents, using two patient oriented questionnaires.
Twenty-nine patients underwent hemispherotomy in Sweden after 1995 and had a five- or ten-year follow-up. At the 2-year follow-up 55% (16/29) were seizure-free since surgery, and 11/29 (38%) were seizure-free at the long term follow up. Twenty-six (90%) participated in this study. Median time to interview was 13.5 years; 9/26 (35%) were seizure-free then; 23% were off antiepileptic medication. In those not seizure-free, seizures were considered mild or moderate; 11% attended mainstream school and 3/12 adults lived independently. Most parents both of seizure-free and non seizure-free patients reported QoL and general health to be very good/good; 73% were satisfied/very satisfied with the hemispherotomy.
In this series there were more long-term recurrences than previously reported. This might be related to the lower level of function of this cohort and higher percentage of developmental aetiologies compared to other series. However, most hemispherotomy patients have a good QoL in the long run and feel that the operation was worthwhile, even when it did not stop seizures. The majority had persisting impairments. Proxies were not very concerned about seizures, indicating that reduction in seizure frequency and/or severity may be an important gain with hemispherotomy.
Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.
... The procedure arrested seizure activity in 6/7 participants, and, within 1-3 years, antiepileptic drugs were discontinued in all but 2 participants. The clinical history describing the level of hemiparesis as unchanged after surgery is similar to that of existing studies [34]. The first detailed evaluation of sensorimotor function of these participants was performed concurrently with this study and is described elsewhere [35]. ...
Cerebral hemispherectomy to control intractable seizures is becoming a standard procedure with more cases identified and treated early in life [1]. While the effect of the dominant hemisphere resection on spoken language has been extensively researched little is known about written language and reading abilities in individuals after left-sided resection.
Methods
We investigated the potential of the isolated right hemisphere to support reading in 7 carefully selected individuals who all have undergone left cerebral hemispherectomy to control intractable seizures associated with perinatal infarct. Specifically, we examined skills that in English predict word reading and comprehension levels: phonological awareness, memory, rapid naming and vocabulary size [2]. In healthy individuals, these skills with the exception of vocabulary are strongly left- lateralized functions [3].
Results
We found that, as a group, our participants with the isolated right hemisphere developed reading and its underlying skills in the low average range with some correlations between the reading level and its components similar to the contingencies reported in neurologically-intact readers. Later seizure onset was associated with better reading levels.
Conclusions
When cerebral hemispherectomy is performed to control seizures associated with very early, in utero insult the remaining right hemisphere can develop nearly normal written language processing utilizing the same resources as the left hemisphere does in healthy readers.
... The procedure arrested seizure activity in 6/7 participants, and, within 1-3 years, antiepileptic drugs were discontinued in all but 2 participants. The clinical history describing the level of hemiparesis as unchanged after surgery is similar to that of existing studies [34]. The first detailed evaluation of sensorimotor function of these participants was performed concurrently with this study and is described elsewhere [35]. ...
