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;
Origin ID : 348567584;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- False automatic mappings
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.
