Hemichorea associated with nonketotic hyperglycemia: clinical and neuroimaging features in 12 patients

Nonketotic hyperglycemia is a rare cause of hemichorea. Patients with hemichorea associated with nonketotic hyperglycemia (HCNH) always have a favorable prognosis when given prompt treatment.

We reviewed the medical records of 12 patients with HCNH in our hospital between January 2005 and January 2013. The clinical data, laboratory findings, and imaging features of the patients were collected.

All 12 patients were admitted to the hospital with a complaint of involuntary movements. Ten patients had a history of diabetes, while the other 2 patients had not been diagnosed. The mean level of blood glucose on admission was 330.7 ± 107.8 mg/dl, and the ketones were negative. A cranial computed tomography scan showed hyperdensity in the striatum, which quickly resolved. Magnetic resonance imaging showed hyperintensity on T1-weighted images without change over several months. Nearly all of the patients experienced relief from the hemichorea symptoms after correcting hyperglycemia with a combination of dopamine receptor inhibitors and the sedative lorazepam, if necessary.

HCNH is a benign disorder, the pathogenesis of which remains unclear. Radiologic changes can provide guidance for early treatment and generally give an estimation of the degree of injury.