" /> Corticobasal degeneration - CISMeF





Preferred Label : Corticobasal degeneration;

ICD-11 definition : Corticobasal degeneration is a rare neurodegenerative disorder of the elderly which affects subcortical, then cortical brain areas and presents with a slowly progressive, unilateral jerky, tremulous, akinetic, rigid and apraxic limb held in a fixed dystonic posture and displaying the alien limb syndrome. The behavioural manifestations may also include language disturbances and frontal-lobe-type behaviour. The pathology is pleomorphic and the progression is hard to predict clinically.;

ICD-11 synonym : other specified degenerative diseases of basal ganglia; CBD - [corticobasal degeneration];

ICD-11 acronym : CBD;

ICD-11 inclusion : olivopontocerebellar atrophy; hereditary olivopontocerebellar degeneration; olivopontocerebellar degeneration; familial olivopontocerebellar degeneration; olivocerebellar atrophy; Déjerine-Thomas syndrome; Thomas syndrome; Calcification of basal ganglia; OPCA - [olivopontocerebellar atrophy]; Déjerine-Thomas atrophy;

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Corticobasal degeneration is a rare neurodegenerative disorder of the elderly which affects subcortical, then cortical brain areas and presents with a slowly progressive, unilateral jerky, tremulous, akinetic, rigid and apraxic limb held in a fixed dystonic posture and displaying the alien limb syndrome. The behavioural manifestations may also include language disturbances and frontal-lobe-type behaviour. The pathology is pleomorphic and the progression is hard to predict clinically.

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30/07/2025


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