A disease characterized by chorea or ballism with associated hyperglycemia, and/or reversible basal ganglia abnormalities on imaging, diabetic striatopathy is a rare complication of diabetes mellitus. Patients usually present with acute onset chorea and is more common among elderly women.

An 88-year-old male was brought to the emergency room due to 2-day history of involuntary jerking and flailing movements of the left arm, which progressed to involve twitching of lips and tongue. Physical examination revealed normal cranial nerve, motor, and sensory examinations, but with choreiform movements of left upper extremities and twitching of perioral and jaw muscles.

Laboratory tests revealed HbA1c of 10.4%, random blood sugar of 665mg/dL, and negative ketones. Cranial MRI and electroencephalogram were unremarkable. He was started on insulin therapy with improvement of blood sugar to 120-160mg/dL. The choreiform movements resolved with improvement of blood sugar control.

Diabetic striatopathy, also known as non-ketotic hyperglycemia chorea-ballismus (NKH-CB) is the most common metabolic cause of chorea-ballismus. It should be considered when patients present with acute or subacute choreiform movements, particularly in the context of poorly controlled diabetes. The pathophysiology is unclear but proposed mechanisms include hyperglycemia induced basal ganglia dysfunction due to hypoperfusion and depletion of gamma-aminobutyric acid. Prognosis is generally favorable with appropriate glycemic management.