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Limb apraxia is a neurological disorder of higher cognitive function characterized by an inability to perform purposeful skilled movements and not attributable to an elementary sensorimotor dysfunction or comprehension difficulty. Corticobasal Syndrome (CBS) is an akinetic rigid syndrome with asymmetric onset and progression with at least one basal...
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... information-processing approach has been developed by Roy (1996), whose model consists of three systems: a sensory/perceptual, conceptual and production system (see Fig. 1). The sensory/perceptual system processes informa- tion from the environment, which can be visual, auditory or tactile. The conceptual system stores our knowledge about tools and actions. The production system consists of several subsystems subserving such functions as response selec- tion, image generation, working memory storage of the motor plan and response organization and control of movement. Roy (1996) suggests that disruptions in any of these three systems will result in a specific pattern of praxis deficits. For example, a disruption of the production system should impair the ability to produce a movement irrespec- tive of the modality in which the task is being presented (be it visual, verbal or tactile). Therefore, in order to draw meaningful conclusions from studies examining limb apraxia it is not sufficient to say that a patient is presenting with apraxia, but rather one needs to specify the pattern of deficits more precisely by assessing each ...
Similar publications
There exist few clinical assessments for limb apraxia, a disorder of learned, purposeful action, that enable clinicians to distinguish pathological from normal variance in limb praxis performance. We describe a theoretically motivated, comprehensive assessment battery for limb apraxia and present control comparison scores for 16 older healthy norma...
Citations
... Importantly, the dorso-dorsal pathway is functionally symmetric, while the ventro-dorsal pathway is left-lateralized. In a seminal paper about apraxia in CBS, Stamenova et al. [13] suggested that neurodegeneration along the dorsodorsal pathway might be the primary source of apraxia in CBS and that, since this stream has a symmetric representation, apraxia 'would equally affect left and right hemisphere patients' [13]. Our results are in line with this thesis, as our lhCBS and rhCBS groups were equally impaired on the Ideomotor Apraxia test. ...
... Importantly, the dorso-dorsal pathway is functionally symmetric, while the ventro-dorsal pathway is left-lateralized. In a seminal paper about apraxia in CBS, Stamenova et al. [13] suggested that neurodegeneration along the dorsodorsal pathway might be the primary source of apraxia in CBS and that, since this stream has a symmetric representation, apraxia 'would equally affect left and right hemisphere patients' [13]. Our results are in line with this thesis, as our lhCBS and rhCBS groups were equally impaired on the Ideomotor Apraxia test. ...
Background
Corticobasal syndrome (CBS) is typically asymmetric. Case reports suggest that left-hemisphere CBS (lhCBS) is associated with major language impairment, and right-hemisphere CBS (rhCBS) is associated with major visuospatial deficits, but no group study has ever verified these observations. In our study, we enrolled 49 patients with CBS, classified them as lhCBS or rhCBS based on asymmetry of hypometabolism on brain FDG-PET and compared their cognitive and behavioural profiles.
Methods
We defined asymmetry of hypometabolism upon visual inspection of qualitative PET images and confirmed it through paired comparison of left- and right-hemisphere FDG uptake values. The two groups were also matched for severity of hypometabolism within the more affected and more preserved hemispheres, to unravel differences in the cognitive profiles ascribable specifically to each hemisphere’s functional specializations. All patients were assessed for memory, language, executive and visuospatial deficits, apraxia, neglect, dyscalculia, agraphia and behavioural disturbances.
Results
LhCBS (n. 26) and rhCBS (n. 23) patients did not differ for demographics, disease duration and severity of global cognitive impairment. The two cognitive profiles were largely overlapping, with two exceptions: Digit span forward was poorer in lhCBS, and visual neglect was more frequent in rhCBS.
Conclusions
After balancing out patients for hemispheric hypometabolism, we did not confirm worse language or visuospatial deficits in, respectively, lhCBS and rhCBS. However, verbal short-term memory was more impaired in lhCBS, and spatial attention was more impaired in rhCBS. Both of these functions reflect the functional specialization of the left and right fronto-parietal pathways, i.e. of the main loci of neurodegeneration in CBS.
... For example, the following apraxias have been identified: limb apraxia (performing meaningless or meaningful skillful movements with the limbs), callosal apraxia (performing skilled movements with the left upper limb in patients with corpus callosum injury), buccofacial apraxia or orofacial apraxia (performing or imitation of facial or lip gestures such as whistling, winking, or licking the lips), apraxia of speech or verbal apraxia (planning and coordination of mouth and speech movements), and constructional apraxia (constructing or copying of simple drawings including simple diagrams, figures, or objects). The current chapter focuses on limb apraxia as a motor cognitive disorder that can occur with varying reported incidence rates and severity in developmental disorders as well as acquired neurological or psychiatric diseases, including stroke, traumatic brain injury, dementia, Parkinson' s disease, corticobasal syndrome, Huntington' s disease, multiple sclerosis, and schizophrenia (Dewey, 1995;Dutschke et al., 2018;Harscher et al., 2017;Holl et al., 2011;Kübel et al., 2017;Stamenova et al., 2009;Stegmayer et al., 2016;. Limb apraxia can affect activities of daily living, ranging from communicative gesturing to the actual use of novel and familiar tools and objects. ...
The chapter describes models, neuroanatomical correlates, assessments and therapy approaches for limb apraxia. It considers both, developmental and acquired disorders of motor cognition.
Furthermore, it includes supplementary material with a list of a test selection.
For further questions, please feel free to send me a message.
... La apraxia es la dificultad o imposibilidad de realizar correctamente movimientos proposicionales aprendidos como consecuencia de una lesión cerebral, en ausencia de trastornos elementales sensoriomotores, alteraciones perceptivas o de comprensión. 1 Es una alteración que aparece frecuentemente en las personas que han sufrido un ictus y se calcula que al menos el 50% de ictus cursan con apraxia. 2 También puede aparecer en personas que han sufrido otras lesiones cerebrales, tales como un traumatismo craneoencefálico y estadios avanzados en enfermedades neurodegenerativas (por ejemplo, demencia tipo Alzheimer). [3][4] La apraxia cursa con mayor frecuencia en aquellos pacientes diestros en los que la lesión está localizada en el hemisferio izquierdo del cerebro. Su prevalencia se estima entre el 28% y el 57% en casos con lesiones de hemisferio izquierdo y hasta del 80% si además cursa con afasia, 5 mientras que en lesiones del hemisferio derecho oscila entre el 0% y el 34%. ...
La apraxia es una alteración frecuente en las personas que han sufrido un ictus en el hemisferio izquierdo, con importantes repercusiones sobre su calidad de vida. Aunque se han propuesto varios modelos para su interpretación y abordaje, algunos aspectos están aún en estudio. El objetivo de este trabajo es revisar los modelos actuales sobre la apraxia de extremidades, analizar las pruebas de evaluación disponibles y realizar recomendaciones para la práctica clínica. En la revisión se pone de manifiesto que en la literatura existe un mayor interés en el sistema conceptual de la ruta léxico-semántica, sin que haya sido desarrollado en detalle el sistema conceptual de la ruta visuomotora. En este artículo se propone un modelo de las apraxias que supera estas limitaciones y se describe un método de valoración centrado en el conocimiento del cuerpo en la ruta visuomotora. Finalmente se concluye que son necesarios más estudios que validen experimentalmente el modelo propuesto y los métodos de evaluación asociados.
... The symptoms cannot be attributed to elementary sensory-motor deficits or language disorders (Zadikoff and Lang, 2005;Goldenberg, 2013). Apraxia usually occurs after left hemisphere lesions (LBD), although cases have also been reported after right hemisphere damage (RBD, Stamenova et al., 2009;Petreska et al., 2007;Barbieri and De Renzi, 1988) and in degenerative syndromes (Rohrer et al., 2010). Various forms of apraxia have been described, with symptoms involving the upper limbs, face, eyes, legs or trunk (Petreska et al., 2007) during transitive or intransitive actions (i.e. with or without an object; Rapcsak et al, 1993;Dumont et al., 1999;Goldenberg, 2013;Canzano et al., 2016). ...
Objective:
Erroneous gesture execution is at the core of motor cognition difficulties in apraxia. While a taxonomy of errors may provide important information about the nature of the disorder, classifications are currently often inconsistent. This study aims to identify the error categories which distinguish apraxic from non-apraxic patients.
Method:
Two groups of mixed (bucco-facial and limb) and bucco-facial apraxic patients suffering from stroke were compared to non-apraxic, left and right hemisphere damaged patients in tasks tapping the ability to perform limb and bucco-facial actions. The errors were analysed and classified into 6 categories relating to content, configuration or movement, spatial or temporal parameters and unrecognisable actions. Furthermore, an anatomical investigation (VLMS) was conducted in the whole group of left hemisphere damaged patients to investigate potential correlates of the various error categories.
Results:
Although all the above error typologies may be observed, the most indicative of mixed apraxia is the content-related one relate to content in all the typologies of actions (transitive and intransitive), and configuration errors in transitive ones. Configuration and content errors in mouth actions seem to be typical of bucco-facial apraxia. Spatial errors are similar in both apraxic and right brain damaged, non-apraxic patients. A lesion mapping analysis of left-brain damaged patients demonstrates that all but the spatial error category are associated with the fronto-parietal network. Moreover, content errors are also associated with fronto-insular lesions and movement errors with damage to the paracentral territory (precentral and postcentral gyri). Spatial errors are often associated to ventral frontal lesions.
Conclusions:
Bucco-facial and mixed apraxic patients make different types of errors in different types of actions. Not all errors are equally indicative of apraxia. In addition, the various error categories are associated with at least partially different neural correlates.
... Limb apraxia is among the most commonly identified signs that suggests cortical dysfunction in the corticobasal syndrome, occurring in 70%-80%. [39][40][41] Apraxia is defined as a disorder of higher level motor control, manifesting as impaired skilled and learnt motor acts, despite intact primary sensory and motor pathways 39 Apraxia generally affects both sides of the body. Because corticobasal syndrome is usually asymmetrical, finding apraxia on the less affected side (as is common) adds weight to the conclusion that abnormality of movements are not simply due to extrapyramidal features such as rigidity and bradykinesia. ...
Corticobasal syndrome is a disorder of movement, cognition and behaviour with several possible underlying pathologies, including corticobasal degeneration. It presents insidiously and is slowly progressive. Clinicians should consider the diagnosis in people presenting with any combination of extrapyramidal features (with poor response to levodopa), apraxia or other parietal signs, aphasia and alien-limb phenomena. Neuroimaging showing asymmetrical perirolandic cortical changes supports the diagnosis, while advanced neuroimaging may give insight into the underlying pathology. Identifying corticobasal syndrome carries some management implications (especially if protein-based treatments arise in the future) and prognostic significance. Its treatment is largely symptomatic and is best undertaken within a multidisciplinary setting, including a neurologist, physiotherapist, occupational therapist, speech language therapist, psychiatrist and, ultimately, a palliative care clinician. Corticobasal syndrome can be a confusing entity for neurologists, not least because it has over time evolved from being considered predominantly as a movement disorder to a condition spanning a wide range of cognitive and motor manifestations. In this practical review, we attempt to disentangle this syndrome and provide clarity around diagnosis, its underlying pathological substrates, key clinical features and potential treatments.
... La presentazione iniziale è generalmente caratterizzata da una goffaggine della mano legata ai disturbi prassici, una leggera ipoestesia e una sensazione di mano estranea, che traducono una lesione delle regioni frontale e parietale e dei gangli della base. L'aprassia è presente nel 70% dei casi di DCB [45] , il più delle volte di tipo ideomotorio [46,47] , spesso bilaterale, ma asimmetrica e precoce [48] . Non c'è dissociazione automaticovolontaria. L'aprassia ideomotoria riscontrata in questi pazienti avrebbe alla base un'atrofia delle regioni parietali e una lesione della sostanza bianca associativa parietofrontale [49] . ...
Riassunto
L’aprassia è un deficit acquisito dei movimenti appresi. Tre forme principali di aprassia gestuale sono state descritte all’inizio del XIX secolo da Liepmann: aprassia ideomotoria, ideatoria e melocinetica. Ciascuna di queste aprassie corrisponde a un disturbo selettivo di un aspetto del gesto e a una lesione di una regione cerebrale specifica. Questo articolo descrive i tre tipi di aprassia gestuale, da un punto di vista semeiologico e anatomico. Viene, inoltre, proposta un’interpretazione innovativa dell’aprassia ideomotoria nell’ambito della teoria dei “neuroni specchio”. Sono, quindi, esaminati altri disturbi neurologici che sono stati chiamati aprassia, come l’aprassia costruttiva, quella dell’abbigliamento o, anche, quella della deambulazione, che sono molto frequentemente osservate nella pratica clinica. L’aprassia costruttiva, corrispondente a un deficit delle capacità di pianificazione e/o di analisi visuospaziale, è molto frequente dopo l’insorgenza di una lesione cerebrale (vascolare, degenerativa, ecc.). Di comparsa più rara, la disprassia diagonistica e il fenomeno della mano estranea sono, qui, considerati come forme particolari di aprassia. Sono discusse le correlazioni lesionali di ciascun tipo di aprassia, così come alcune malattie neurologiche caratterizzate dalla centralità dei disturbi prassici, come la degenerazione corticobasale. Si sottolinea l’importanza della valutazione clinica dei disturbi prassici. Per aiutare il neurologo nella sua pratica clinica, sono proposti esempi di gesti imitativi utili per la diagnosi di aprassia ideomotoria, la forma più frequente di aprassia.
... Corticobasal syndrome can also arise from other neuropathologies, such as Alzheimertype pathology or progressive supranuclear palsy [13][14][15], Creutzfeldt-Jakob disease [14] or the cumulative effects of cerebrovascular disease [16]. The corticobasal syndrome can present with subcortical motor features (including akinetic rigidity, dystonia and myoclonus) or cortical features (including visuospatial and deficits, non-fluency and cortical sensory loss) [17,18]. The current international consensus clinical diagnostic criteria for corticobasal syndrome include alien limb [19], as have earlier diagnostic criteria [20,21]. ...
... Apraxia may be defined as a disorder of "the execution of learned movement which cannot be accounted for either by weakness, incoordination or sensory loss, or by incomprehension of or inattention to commands" [23]. It is assessed through the pantomiming of actions (performing an action on request without example) and imitation of gestures [18]. It can result from focal lesions, classically in the corpus callosum and dominant hemisphere [23,24], as well as neurodegenerative disorders such as corticobasal syndrome [18,19]. ...
... It is assessed through the pantomiming of actions (performing an action on request without example) and imitation of gestures [18]. It can result from focal lesions, classically in the corpus callosum and dominant hemisphere [23,24], as well as neurodegenerative disorders such as corticobasal syndrome [18,19]. Limb apraxia is usually considered in three forms, often called 'ideational', 'ideomotor', and 'limb-kinetic apraxia' [18,25]. ...
Alien limb refers to movements that seem purposeful but are independent of patients’ reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n = 30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 23/30 (77%) patients. Moreover, 25/30 (83%) patients reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and either the occurrence of an alien limb or the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severity apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.
... These movements have been described as often bimanual, and liable to occur within two 8 scenarios: (a) the offending hand is involuntarily recruited to tasks which the patient intends to perform Alien limb is a common clinical feature of the corticobasal syndrome. Corticobasal syndrome classically arises 25 from the specific pathology termed 'corticobasal degeneration' [11], which is associated with aggregation of 26 hyperphosphorylated 4-repeat tau protein [12]. Corticobasal syndrome can also arise from other 27 neuropathologies, such as Alzheimer type pathology [13][14][15], Creutzfeldt-Jakob disease [14] or the cumulative 28 effects of cerebrovascular disease [16]. ...
Alien limb refers to movements that seem purposeful but are independent of patients' reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n=30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease-control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 77% of patients. Moreover, 83% reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and both the occurrence of an alien limb and the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severe apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.
... It is also reported in several dementia syndromes such as AD and frontotemporal lobar degeneration (FTLD) and is often observed in head trauma involving the left parietal lobe, which makes it likely to be encountered in general neurological practice. Limb apraxia is highly prevalent in CBS (70-80%) and is a key defining clinical feature of CBS (Armstrong et al., 2013;Stamenova et al., 2009). CBD, its most common neuropathological correlate, is rare, with an estimated prevalence of 2-7 per 100 000 individuals (Togasaki and Tanner, 2000). ...
... sucking on a straw, blowing out a candle) and intransitive (e.g. whistling) gestures (Stamenova et al., 2009). Orofacial apraxia has been associated with inferior frontal, deep frontal white matter, insula and basal ganglia lesions (Ozsancak et al., 2004). ...
Apraxia is defined as the difficulty or inability to perform learned skilled actions. Identifying apraxia in patients has prognostic implications. The praxis network is predominantly in the left hemisphere, and comprises parietal lobe regions that connect to several circuits involving the frontal, temporal and occipital cortices and the basal ganglia. This review discusses the types of apraxia and the disorders associated with apraxia, highlighting studies based on corticobasal syndrome and stroke as disease models. The evolution of historical concepts of praxis leading up to the current ‘pathway’ models is discussed in the context of neuroanatomical and imaging studies. Bedside testing and interpretation of apraxia are elucidated with examples.
Key Concepts
• Apraxia is the difficulty in performing acquired actions and can be seen independent of other symbol‐mediated disorders such as aphasia.
• Apraxia is associated with several neurodegenerative disorders such as corticobasal syndrome, as well as with strokes and head trauma.
• There are several different forms of apraxia such as ideomotor, ideational and orofacial apraxia, and each is characterised by the specific manner in which action performance or interpretation is compromised.
• The praxis network involves circuits from frontal, temporal and parietal cortices and the basal ganglia.
• Left parietal lobe is pivotal for the praxis network.
• Newer models of praxis propose bilaterally represented ventral and dorsal processing streams, with the dorsal stream further subdivided into dorso‐dorsal and ventrodorsal substreams. The dorso‐dorsal or the ‘grasp’ system processes characteristics of a tool such as size, shape and orientation, while the ventrodorsal or the ‘use’ system stores object‐specific actions.
• In patients with left hemisphere damage, apraxia often coexists with aphasia; in patients with right hemisphere lesions, apraxia is often associated with a visuospatial disorder.
• Testing of apraxia at bedside is important for diagnostic and prognostic reasons.
... In previously reported cases, CBS patients rarely exhibit gait disturbance caused by leg apraxia.In addition, there had been little reported video showing symptoms of leg apraxia. In the video, our patient revealed limb-kinetic apraxia (clumsiness and awkwardness in his left leg), and ideational apraxia (do not imitate the gestures)4 . Based on this case report, we are aware of the symptoms of lower limb apraxia and hope to help to consider the diagnosis of CBS when a patient presents with gait disturbance.Conflicts of Interest: The authors have no financial conflicts of interest.Financial disclosure: The authors have nothing to declare. ...
