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JAMA Neurology Clinical Challenge
December 21, 2018

Type 2 Diabetes Presenting With Persistent Chorea

Author Affiliations
  • 1Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania
JAMA Neurol. Published online December 21, 2018. doi:10.1001/jamaneurol.2018.4089

A man in his late 60s with a diagnosis of type 2 diabetes for more than 10 years that was complicated by neuropathy and retinopathy presented with persistent chorea in the left upper extremity for about 1 month despite aggressive correction with insulin. He was alert and oriented with healthy vital signs. No evidence of cognitive impairments was present on bedside testing results. The patient denied any recent infections and risk for human immunodefiency virus (HIV) infection. Neurological examination results were significant for choreiform and hemiballistic movements involving the left upper extremity. His home medications were carefully reviewed to rule out any medications that could cause chorea. A review of his previous records indicated an admission to an outside facility 3 weeks beforehand with severe hyperglycemia and a hemoglobin A1c level of 14.1% (to convert to the proportion of total hemoglobin, multiply by 0.01). Despite an aggressive correction of blood glucose levels and metabolic correction, his choreiform movements had remained unresolved. On admission, a complete electrolyte and toxicology panel, including calcium, magnesium, and phosphorus, yielded normal results. The patient’s serum glucose level was 159 mg/dL (to convert to millimoles per liter, multiply by 0.0555) and his hemoglobin A1c was 10.9% without ketoacidosis. Test results for antinuclear antibodies, lupus anticoagulants, and antiphospholipid antibodies were negative. Serum thyrotropin levels, liver function test results, and parathyroid hormone levels were healthy. Copper studies, a peripheral smear, and a routine electroencephalogram yielded normal results. A general screening for cancers yielded negative results. The patient underwent brain magnetic resonance imaging (MRI) (Figure), which showed a T1 hyperintensity in the right caudate head and a lentiform nucleus with no restricted diffusion. Oral administration of olanzapine, 2.5 mg daily, in divided doses with careful monitoring of blood glucose levels resulted in the complete resolution of chorea.