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

¹ Clinica Neurologica, Università di Roma Tor Vergata and Fondazione Santa Lucia, IRCCS, Rome I-00133, Italy
² Day Hospital Talassemie, Ospedale S. Eugenio, Rome I-00144, Italy
³ Istituto di Radiologia, Università di Roma Tor Vergata, Italy
⁴ Clinica Neurologica, Università di Perugia, Perugia I-06516, Italy

Correspondence to:
Correspondence to:
Dr A Pisani
Clinica Neurologica, Dipartimento di Neuroscienze, Università di Roma Tor Vergata, Via Montpellier 1, 00133, Rome, Italy; pisani{at}uniroma2.it

ABSTRACT
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.

Abbreviations: apoB, apolipoprotein B; HCHB, hemichorea-hemiballism; MRI, magnetic resonance imaging

Keywords: chorea; ballism; diabetes; acanthocytosis