MON-649 - Bilateral Acquired Chorea: A Unique Presentation of Diabetic Ketoacidosis

Hemichorea-hemiballismus (HCHB) is a rare hyperkinetic movement disorder caused by dysfunction of the basal ganglia and characterized by involuntary, nonrhythmic choreiform or ballistic movements. Diabetic striatopathy is an acquired and underrecognized cause of HCHB associated with prolonged hyperglycemia, most commonly occurring in patients with poorly controlled type 2 diabetes mellitus and classically presenting with unilateral symptoms in the setting of nonketotic hyperglycemia. Bilateral involvement and presentation with diabetic ketoacidosis (DKA) are uncommon. We report a rare case of bilateral diabetic striatopathy presenting with DKA and acute encephalopathy. An 82-year-old woman with long-standing type 2 diabetes mellitus presented with three days of progressive confusion and polydipsia after a period of excessive carbohydrate intake and was previously fully independent in activities of daily living. On examination, she was oriented to name only and demonstrated acute encephalopathy, dysarthric speech, involuntary facial movements, and prominent bilateral choreiform movements involving both upper and lower extremities. Laboratory evaluation revealed severe hyperglycemia (821 mg/dL), high anion gap metabolic acidosis (pH 7.20, bicarbonate 9 mmol/L, anion gap 28), elevated β-hydroxybutyrate (10.1 mmol/L), and hemoglobin A1c of 15.8%, confirming DKA and chronic poor glycemic control. CT of the head without contrast demonstrated symmetric hyper densities of the bilateral caudate nuclei and putamen, consistent with diabetic striatopathy. The patient was treated with intravenous insulin, aggressive fluid resuscitation, and antibiotics, resulting in rapid closure of the anion gap and significant improvement in mental status; however, involuntary choreiform movements persisted despite metabolic correction. Following initiation of risperidone, there was marked improvement in both movement symptoms and cognition. At two- and three-month follow-up hospitalizations for unrelated medical issues, choreiform movements had completely resolved without recurrence. This case highlights an uncommon presentation of bilateral diabetic striatopathy associated with DKA and acute encephalopathy, emphasizing the heterogeneity of clinical features. While prompt glycemic control remains the cornerstone of management, adjunctive antipsychotic therapy may be necessary for severe or persistent symptoms. Early recognition of this rare but reversible complication is critical to improve neurological outcomes in patients with uncontrolled diabetes.

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