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Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism
1Clinica Neurologica, Dipartimento di Neuroscienze, Università di Roma Tor Vergata, Via Montpellier 1, 00133 Rome, Italy. pisani@uniroma2.it
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.
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