SAT-594 - Deciphering the Dance-A Case of Nonketotic Hyperglycemic Hemichorea-Hemiballismus Syndrome

Non-ketotic hyperglycemia hemichorea-hemiballismus syndrome is a rare condition characterized by glucose levels greater than 200 mg/dL and continuous, uncontrollable, and irregular jerky movements localized on one side of the body1. It is more commonly seen in type 2 diabetics compared to type 1 diabetics.
68 YO female presented with sudden onset involuntary movements of left upper and lower limbs for 3 days. She had a background of hypertension but denied history of diabetes mellitus. On general physical examination, she had a blood pressure of 150/90mmHg and raised blood Glucose levels of 475 mg/dL. She had involuntary flailing and dance like movements of left upper and lower limbs with variable amplitude and undefined direction. She had no focal neurological deficit. Further workup showed HbA1c of 12.7% and blood sugar fasting of 244 mg/dL. Her venous blood gases showed no acidemia and her urine was found negative for ketone bodies. Her CT head showed subtle hyperdensities in right lentiform nucleus; features suggestive of non ketotic hyperglycemic hemichorea. The radiological findings were confirmed by MRI Brain which revealed hyperintense signals in same location on T1 weighted sequences. She was managed with variable rate IV insulin infusion. Her involuntary movements resolved over the next 4 days as her glycemic control improved. She was discharged home on a multiple daily injection insulin regimen with no recurrence of symptoms reported at follow up. For patients presenting with the clinical picture of sudden onset of uncontrolled movements, the initial workup should include neurological evaluation, as well as evaluation for non-ketotic hyperglycemia syndrome. MRI and serum glucose levels are both helpful in the prompt diagnosis of non-ketotic hyperglycemia hemichorea-hemiballismus syndrome.

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