An endemic neurological disorder in tribal Australian aborigines.
Thirteen, and possibly sixteen cases of neurological disorder have been identified in a population of approximately 1100 tribal aborigines living in Groote Eylandt and the adjacent mainland. There were two relatively distinct clinical pictures: one coming on in childhood involved the motor system, the patients often having remarkably lax ligaments; and the other, generally of later onset, comprising cerebellar, upper motor neurone and sometimes supranuclear ophthalmoplegic features. There was some evidence that the two syndromes are varieties of a single condition. No causal factors were identified but there were indications that the disorder might be genetically determined. Attention is drawn to the similarities between this disorder and other ethnic-geographic isolates, particularly the ALS-Parkinsonism-dementia complex of Guam.
D000293:Adolescent; D000328:Adult; D001315:Australia; D002524:Cerebellar Ataxia / Q000235:genetics; D002648:Child; D005006:Ethnicity; D005260:Female; D006801:Humans; D008297:Male; D009046:Motor Neurons; D009123:Muscle Hypotonia / Q000235:genetics; D009133:Muscular Atrophy / Q000235:genetics; D009468:Neuromuscular Diseases / Q000235:genetics*; D009886:Ophthalmoplegia / Q000235:genetics; D012021:Reflex, Abnormal / Q000235:genetics; D012934:Social Isolation