Benign nonprogressive familial chorea
Incidence
It is an autosomal dominant condition but some autosomal recessive cases has been observed. More than 25 families have been reported.
Clinical Characteristics
This disorder is characterized by isolated non progressive chorea starting in infancy or childhood. Onset is usually in late infancy or early childhood from one to four years or in adolescence. There are typical, rather rapid jerky, random, purposeless choreic movements involving the limbs, trunk, neck and facial musculature. If severe it may cause difficulty in walking and affects speech. There may be a postural tremor as well as axial dystonia. The dyskinesia is persistent except during sleep. It shows no tendency to worsen as time passes except occasionally during adolescence. There are no other neurological signs. Mental deterioration is absent. Intelligence is generally normal. The severity varies from case to case. Some improvements may be obtained using haloperidol, chlorpromazine or prednisone.
Precipitants
no
Provocation Tests
no
Diagnostic Procedures
It is a clinical diagnosis.