Don’t Forget That Chorea!

Abstract

Background: Hemichorea-hemiballismus is a spectrum of involuntary, continuous non-patterned movement involving one side of the body. Possible causes of hemichorea-hemiballismus include haemorrhagic or ischemic stroke, neoplasm, systemic lupus erythematosus, NHH (non-ketotic hyperglycaemic hemichorea), Wilson’s disease, and thyrotoxicosis. Amongst the metabolic causes, chorea associated with NHH is noteworthy and is mainly reported in elderly Asian women. The pathophysiology of this syndrome remains controversial. It is likely that a combination of hyperglycaemia induced basal ganglia metabolic derangement and failure of cerebral blood flow autoregulation contribute to the syndrome.Case presentation: A 45-year-old Malay gentleman presented to our Emergency Department with right upper and lower limb weakness associated with hemichorea for 3-4 days. His initial blood glucose level was 22 mg/dl with normal serum ketone and bicarbonate levels. CT brain showed a hyperdensity in the left caudate nucleus and globus pallidus region. Subsequent brain MRI revealed an asymmetric T1 hyperintensity of the left putamen. This specific finding was compatible with hyperglycaemia-induced hemichorea-hemiballismus syndrome. The hemiballismus/hemichorea improved rapidly within the next day. Conclusions: This unusual clinical presentation is often accompanied by severe hyperglycaemia. Appropriate blood glycaemic control is important because it is reversible with correction of hyperglycaemia. Thus, prompt recognition and treatment is essential to avoid adverse outcomes.