Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism.

BACKGROUND Episodic non-ketotic hyperglycaemia in patients with diabetes may be responsible for a syndrome characterised by hemichorea-hemiballism associated with unique radiological features. OBJECTIVE To investigate whether factors other than hyperglycaemia may be responsible for the neurological involvement. METHODS Three patients who developed a persistent chorea-ballism syndrome triggered by a hyperglycaemic crisis were investigated. In these patients, the persistence of the involuntary movements required neuroleptic medication. RESULTS T1 weighted magnetic resonance imaging revealed bilateral hyperintense lesions involving the striatum. Surprisingly, in these patients, the laboratory investigations revealed peripheral red blood cell acanthocytosis in a significant proportion of cells. CONCLUSION Compared with the large population of patients with diabetes who do not show abnormal involuntary movements, unrecognised acanthocytosis in diabetes might render patients prone to develop hemichorea-hemiballism.