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Acanthocytosis as a predisposing factor for non-ketotic hyperglycaemia induced chorea-ballism
  1. A Pisani1,
  2. M Diomedi1,
  3. A Rum1,
  4. P Cianciulli2,
  5. R Floris3,
  6. A Orlacchio1,
  7. G Bernardi1,
  8. P Calabresi4
  1. 1Clinica Neurologica, Università di Roma Tor Vergata and Fondazione Santa Lucia, IRCCS, Rome I-00133, Italy
  2. 2Day Hospital Talassemie, Ospedale S. Eugenio, Rome I-00144, Italy
  3. 3Istituto di Radiologia, Università di Roma Tor Vergata, Italy
  4. 4Clinica Neurologica, Università di Perugia, Perugia I-06516, Italy
  1. Correspondence to:
 Dr A Pisani
 Clinica Neurologica, Dipartimento di Neuroscienze, Università di Roma Tor Vergata, Via Montpellier 1, 00133, Rome, Italy; pisaniuniroma2.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.

  • apoB, apolipoprotein B
  • HCHB, hemichorea-hemiballism
  • MRI, magnetic resonance imaging
  • chorea
  • ballism
  • diabetes
  • acanthocytosis

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Footnotes

  • Competing interests: none declared

  • The patients gave their informed consent for this report to be published