Apraxia and Motor Dysfunction in Corticobasal Syndrome

Brain and Cognition, 1999

Corticobasal degeneration (CBD) is a degenerative disease that often presents with an asymmetric progressive ideomotor limb apraxia. Some apraxic subjects may fail to perform skilled purposive movements on command because they have lost the memories or representations that specify how these movements should be performed (representational deficit). In contrast, other apraxic subjects may have the movement representations but are unable to utilize the information contained in them to execute skilled purposive movements (production-execution deficit). To learn if the apraxic deficit in CBD is induced by a representational or a production-execution deficit, we tested three nondemented subjects with CBD on tasks requiring production of meaningful or meaningless gestures to command, gesture imitation, gesture discrimination, and novel gesture learning. A fourth subject with incomplete data also is presented. The results suggest that the apraxia associated with CBD is initially induced by a production-execution defect with relative sparing of the movement representations.

Cogent Psychology

We describe a patient (CG) suffering from early onset dementia who presented with corticobasal syndrome (CBS). The aims of the study were as follows: (i) a detailed description of the cognitive phenotype; (ii) a comprehensive, longitudinal evaluation of apraxia; (iii) an appraisal of the impact of apraxia and other cognitive impairments on patient functional status; and (iv) an indirect mapping of degeneration spreading. A three-year longitudinal, observational follow-up study of cognitive and functional status was performed. Four main results emerged. First, an unusual CBS phenotype appeared that was characterized by symmetrical presentation, asymmetrical course, and prominent posterior (bi-parietal) cognitive and motor cortical manifestations. Second, some findings of limb apraxia in CBS were replicated and substantiated; moreover, some novel findings of other cognitive impairments emerged. Third, an early, significant functional decline, probably related to apraxia and to visuospatial attention impairments, became apparent. Fourth, CG's clinical picture was compatible with an underlying dysfunction of the large-scale, dorsal sensory-motor association network, as already suggested in previous CBS cases.

Journal of Neurology, 2015

Background: Corticobasal syndrome is defined clinically on the basis of symptoms and findings related to dysfunction of the cerebral cortex and the basal ganglia. Usually, marked asymmetry of motor findings is observed. Although aphasia has now been recognised as a frequent feature of corticobasal syndrome, it remains unclear whether it is usually associated with right-sided motor symptoms, pointing to the involvement of the left hemisphere. Hence, we set out to examine the relationship between the presence of language symptoms and the side affected by extrapyramidal symptoms. Method: We analysed the electronic care records of patients seen in the years 2003-2013 in the Neurology Department of the University Hospital of Munich. The diagnosis of corticobasal syndrome was discussed in ninety-two individuals. Of those, 38 cases fulfilled diagnostic criteria for corticobasal syndrome. Results: Aphasia correlated highly with a predominant right-sided movement disorder (p = 0.002). In contrast, it was less common in patients with left-sided motor presentation. Dysarthria did not show a preferential correlation (p = 0.25). Conclusions: Our analysis suggests a characteristic presentation of corticobasal syndrome in which motor dysfunction of the right side of the body is associated with aphasia.

International Journal of Language & Communication Disorders, 2020

Background: Despite initial underreporting of language dysfunctions in corticobasal syndrome (CBS), aphasia is now recognized as a frequent feature of this disease. Aphasia in CBS seems clinically overlying to a nonfluent/agrammatic primary progressive aphasia (nfaPPA), which is also a clinical phenotype associated with corticobasal degeneration (CBD) pathology. However, the clinical features of aphasia in CBS still remain poorly delineated, resulting in misjudgements in the differential diagnosis from a PPA presentation of the disease. Aims: To investigate the language disorders of this syndrome, also through a systematic examination of recoding skills (reading, written spelling and repetition) and articulatory disturbances, which have been rarely examined in previous studies. Methods & Procedures: We present a clinical and neuropsychological descriptive study of the language impairments in a case series of 12 aphasic patients with a clinical diagnosis of CBS. Language assessment was conducted by means of the Esame NeuroPsicologico dell'Afasia, a comprehensive Italian battery for language functions, the Token Test, and the Apraxia of Speech Rating Scale. Outcomes & Results: The language profile of the patients showed a severe expressive language disorder, characterized by non-fluent speech, apraxia of speech (AoS) with predominant stuttering-like dysfluencies, spatial/apraxic agraphia, lack of word-finding and defective sentence repetition. Severe limb apraxia, visual-spatial deficit and alien hand syndrome were also present. Neuroimaging showed bilateral left asymmetric atrophies and hypometabolism in the frontal premotor, parietal posterior and temporal areas. Conclusions & Implications: These findings suggest that aphasia in CBS might present as a 'mixed PPA', instead of an nfaPPA as previously stated, showing a combination of features of the nfa and logopenic variants of the PPA, associated with AoS, stuttering and agraphia, which might be additional important cognitive markers for the clinical diagnosis of CBS and discriminating features of an nfaPPA presentation of a CBD. These results might also suggest specific intervention areas in the rehabilitation of patients with CBS.

Archives of Clinical Neuropsychology, 1991

, is charucterised by impaired production of ajfbive prosody and facial gestures, with intact imitation and comprehension of afj?ect. It has been proposed that cortical TMA arises from lesions in the superior or anterior lateral surface of the right frontal lobe, but for various reasons it has not been possible to fully test this proposal. There have been few cases of TMA reported, and lesions have been too &ruse to permh accurate localization. A case is presented that meets both the Fermi and anato~'~~ criteria ~ro~sedfor cortical TMA, thus providing support for this system of chzssifying disorders of afective communication. Results suggest that the prosodic and gesturai deficits in TMA may be dissociated, and that the gestural component extends to nonaf$ectivefacial movements.

Archives of neurology, 2008

Background: Data on white matter changes in corticobasal degeneration syndrome (CBDS) are not yet available, whereas cortical gray matter loss is a feature of this condition. The structural abnormalities related to a key feature of CBDS (limb apraxia) are unknown. Objectives: To measure selective structural changes in early CBDS using diffusion tensor imaging and voxelbased morphometry and to evaluate the structural correlates of limb apraxia. Design: Patient and control group comparison. Setting: Referral center for dementia and movement disorders. Participants: Twenty patients with CBDS and 21 matched control subjects. Interventions: Clinical and standardized neuropsychological evaluations, including assessment of limb apraxia. Main Outcome Measures: Gray and white matter changes in early CBDS. Results: Diffusion tensor imaging revealed decreases in fractional anisotropy in the long frontoparietal connecting tracts, the intraparietal associative fibers, and the corpus callosum. Fractional anisotropy was also reduced in the sensorimotor projections of the cortical hand areas. Voxel-based morphometry showed a prevalent gray matter reduction in the left hemisphere (in the inferior frontal and premotor cortices, parietal operculum, superotemporal gyrus, and hippocampus). The pulvinar, bilaterally, and the right cerebellar cortex also showed atrophy. Limb apraxia correlated with parietal atrophy and with fractional anisotropy reductions in the parietofrontal associative fibers (PϽ.01). The limb-kinetic component of apraxia correlated with reduction of hand sensorimotor connecting fibers.

Brain and Cognition, 2004

Limb apraxia is a common symptom of corticobasal degeneration (CBD). While previous research has shown that individuals with CBD have difficulty imitating transitive (tool-use actions) and intransitive non-representational gestures (nonsense actions), intransitive representational gestures (actions without a tool) have not been examined. In the current study, eight individuals with CBD and eight age-matched healthy adults performed transitive, intransitive representational and intransitive non-representational gestures to imitation. The results indicated that compared to controls, individuals with CBD were significantly less accurate in the imitation of transitive and intransitive non-representational gestures but showed no deficits for the imitation of intransitive representational gestures. This advantage for intransitive representational gestures was thought to be due to fewer demands being placed on the analysis of visual-gestural information or the translation of this information into movement when imitating these gestures. These findings speak to the importance of context and the representation of gestures in memory in gesture performance.

Journal of Neurology, Neurosurgery & Psychiatry, 1994

Although apraxia is one of the most frequent signs in corticobasal degeneration, the phenomenology of this disorder has not been formally examined. Hence 10 patients with corticobasal degeneration were studied with a standardised evaluation for different types of apraxia. To minimise the confounding effects of the

2014

Posterior cortical atrophy (PCA) is a rare neurodegenerative syndrome characterized by insidious onset and selective, gradual decline in visuospatial and visuoperceptual skills. We report a typical case of a patient with posterior cortical atrophy who presented initially with visuospatial deficit and apraxia. The aim of this report is to present this rare dementia subtype as a relevant differential diagnosis in respect to other neurodegenerative syndromes

Neuroscience Letters, 2008

We sought to determine whether coherent networks which circumvent lesioned cortex are seen in patients with ideomotor apraxia (IMA) while performing tool use pantomimes. Five normal subjects and five patients with IMA (three patients with corticobasal degeneration and two with left hemisphere stroke) underwent 64-channel EEG recording while performing three tool-use pantomimes with their left hand in a self-paced manner. Beta band (20-22 Hz) coherence indicates that normal subjects have a dominant left hemisphere network responsible for praxis preparation, which was absent in patients. Corticobasal degeneration patients showed significant coherence increase between left parietal-right premotor areas. Left hemisphere stroke patients showed significant coherence increases in a right parietofrontal network. The right hemisphere appears to store useable praxis representations in IMA patients with left hemisphere damage. Performance of pantomiming tool use and communicative gestures with spatial and temporal errors is called ideomotor apraxia (IMA). There are two common etiologies of IMA, corticobasal degeneration (CBD) and stroke. CBD patients typically have neurodegeneration in frontal cortex and subcortical structures including basal ganglia [24]. Movement disorders commonly encountered in CBD such as tremor and dystonia may interfere with the performance of these tasks. Yet, disorganized motor plans such as making large circular arcs in the air when asked to pantomime using a toothbrush are evident, are not indicative of elementary motor disorders. Stroke patients with left hemisphere lesions suffering from IMA will have the same errors types as those patients with CBD [14]. Using beta band coherence analysis of EEG, we previously showed evidence of left hemisphere networks responsible for right hand praxis in normal subjects [22]. Damage to left hemisphere parietal or premotor cortex or the white matter connecting them may cause IMA [11]. While patients may regain praxis function [1,21], the neural mechanisms are unclear. The right hemisphere may play a role in assisting in praxis rehabilitation, as it has been shown to be involved with other types of cognitive task rehabilitation (for example [2,5]). It remains unclear to what extent the right hemisphere actually stores praxis movement representations, as left hemisphere pathology results in the most severe disturbances of praxis [12], even though it is