SAT-599 - A Rare Case of Choreiform Movement Disorder in the Setting of Nonketotic Hyperglycemia

Non-ketotic hyperglycemic hemichorea (NKH), also referred to as diabetic striatopathy, is a rare movement disorder characterized by involuntary, typically unilateral movements in patients with poorly controlled diabetes without ketoacidosis. Proposed mechanisms include hyperglycemia-induced depletion of gamma-aminobutyric acid (GABA), increased dopamine receptor sensitivity, and blood–brain barrier disruption related to hyperviscosity. Neuroimaging typically reveals unilateral basal ganglia abnormalities, most often a hyperdensity on CT or a hyperintensity on T1-weighted MRI. Early recognition and prompt glycemic control frequently result in clinical improvement.

A 59-year-old female with poorly controlled type 2 diabetes mellitus presented with progressive gait instability, multiple falls, and involuntary movements predominantly affecting the right upper extremity. The movements were choreiform and intermittently flailing and began several days prior to presentation. Neurologic examination revealed right upper extremity chorea, distal sensory loss in a stocking distribution, absent vibratory sensation in the toes, and diminished deep tendon reflexes. Laboratory evaluation showed a blood glucose of 456 mg/dL and HbA1c of 14%, without evidence of ketoacidosis. Non-contrast CT of the head demonstrated a unilateral hyperdensity of the left basal ganglia, and MRI with contrast showed an abnormal signal intensity within the left lentiform nucleus without ischemic changes. Based on the characteristic clinical and imaging findings, a diagnosis of NKH was established. The patient received insulin therapy for hyperglycemia and nicardipine for concomitant hypertensive emergency. Improved glycemic control resulted in a significant reduction of the involuntary movements. She was discharged on insulin therapy with an endocrinology follow-up emphasizing strict glycemic control.

NKH is an uncommon but reversible complication of uncontrolled diabetes, most frequently affecting older adults and often presenting with unilateral chorea or hemiballismus. Postmenopausal women may be at increased risk due to enhanced dopamine receptor sensitivity associated with reduced estrogen levels. Management focuses on the rapid correction of hyperglycemia, which is typically sufficient for symptom resolution. This case highlights the importance of considering NKH in patients presenting with acute movement disorders and severe hyperglycemia, as early diagnosis and treatment are imperative for favorable outcomes.

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