Nonketotic hyperglycemic hemichorea – A rare presentation of elevated blood sugar

Abstract

Nonketotic hyperglycemic hemichorea, also known as diabetic striatopathy or hyperglycemia basal ganglia syndrome, is a rare presentation of hyperglycemia that can be the initial manifestation of diabetes or present in patients with long-standing poorly controlled diabetes. Patients will demonstrate ballismus or choreiform movements which are usually unilateral. On evaluation, patients' labs will show hyperglycemia without evidence of diabetic ketoacidosis. If imaging is obtained, CT or MRI images may mimic ischemic stroke, hypertensive hemorrhages or deposition diseases in the basal ganglia. The cause of these imaging findings is currently unknown. Treatment of the patients' hyperglycemia generally results in symptom resolution but symptoms can persist for weeks. Antipsychotics, antiepileptics and benzodiazepines have been used for symptom management though very little data exists on their efficacy. The current report documents this rare presentation of hyperglycemia in a poorly controlled diabetic woman. Her symptoms improved after a single dose of benzodiazepines and this case supports their use for symptom management in the emergency department until euglycemia is achieved. Additionally, the CT images obtained during this case mimicked a possible ischemic or infectious lesion and potentially led to a delay in diagnosis. Nonketotic hyperglycemic hemichorea is likely to become more commonplace as incidence of diabetes increases and the symptom profile, objective findings and treatment are something emergency physicians should be aware of.