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Levine-Critchley syndrome; neuroacanthocytosis; choreoacanthyocytosis
Epidemiology: onset 23-59 years
Genetics:
- autosomal recessive mostly
- associated with mutations in chorein gene
Pathology: atrophy of caudate and putamen
Clinical manifestations:
- neurologic/behavioral:
- choreoathetosis, progressive
- orofacial dyskinesia
- hyporeflexia
- dysarthria
- seizure
- dementia
- personality change
- peripheral sensory neuropathy
- oral self-mutilation
- hematologic
- acanthocytosis
Differential diagnosis:
- Huntington's disease
- McLeod syndrome
General
basal ganglia disease
neurodegenerative disease
Properties
PATHOLOGY: gliosis
SITE: caudate nucleus
putamen
Database Correlations
OMIM correlations
References
- OMIM 200150
- National Institute of Neurological Disorders and Stroke (NINDS)
- Levine-Critchley Syndrome Information Page
https://www.ninds.nih.gov/disorders/all-disorders/levine-critchley-syndrome-information-page
- NINDS Neuroacanthocytosis Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Neuroacanthocytosis-Information-Page