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Non-Ketotic Hyperglycaemia Chorea — SCE Neurology MCQ

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HardMovement DisordersNon-Ketotic Hyperglycaemia ChoreaSCE Neurology

A 55-year-old man presents with sudden onset of hemichorea-hemiballismus affecting his left arm and leg. CT brain shows a small right subthalamic nucleus lacunar infarct. His blood glucose is 28 mmol/L and HbA1c is 140 mmol/mol. CT also shows increased density in the right striatum (basal ganglia). What additional cause of his chorea should be considered?

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Correct answer: B — Non-ketotic hyperglycaemia (diabetic striatopathy) — hyperglycaemia can cause unilateral chorea with characteristic CT/MRI findings of striatal hyperdensity/T1 hyperintensity, which may coexist with or mimic a structural lesion

Non-ketotic hyperglycaemia (NKH) is a well-recognised cause of unilateral chorea/hemiballismus, particularly in elderly patients with poorly controlled type 2 diabetes. The characteristic imaging finding is T1 hyperintensity (MRI) or hyperdensity (CT) in the contralateral striatum. The mechanism involves metabolic derangement of basal ganglia neurons. Treatment: correction of hyperglycaemia (which usually resolves the chorea). In this case, both the STN infarct and NKH may be contributing. A: Essential tremor is different. C: HD is bilateral and progressive. D: Wilson has different features. E: No drug exposure mentioned.

Reference: EAN Movement Disorders; Stroke/Diabetes Overlap Guidelines