Hemichorea Associated With Nonketotic Hyperglycemia: Rare Presentation of Uncontrolled Diabetes Mellitus

Abstract

Abstract Introduction: Hemichorea is usually associated with a contralateral lesion in the central nervous system and can result from infection, genetic mutation, neoplasms, neurodegeneration, stroke, metabolic disease, drug-exposure, and autoimmune disease. Nonketotic hyperglycemic (NKH) hemichorea is mainly seen in elderly patients, especially females from East Asian origin with poor blood glucose control. The general treatment measures include improving control of blood glucose and the use of neuroleptic drugs. We report a unique case of hemichorea secondary to nonketotic hyperglycemia with negative imaging changes. Case Presentation: A 72-year-old woman with history of COPD, CKD stage III, CAD, diabetes type II for more than 30 years with poor glycemic control presented with sudden onset of uncontrolled involuntary dance like movements of left upper and lower extremities and left side of the face. These activities occurred while awake and disappeared during sleep. Neurological examination found that patient has a clear verbal ability, sanity, present involuntary dance-like movements of limbs, facial, jaw, and tongue. She had normal muscle strength and mild hypotonia. Other physical examination found no abnormalities. A CAT scan of the head showed high density in basal ganglia region and brain MRI(T1-weighted) showed and hyperintensity in the right basal ganglia. Laboratory tests showed that random blood sugar was 572 mg/dL, urine sugar (4+), and negative urine ketone, Hba1c was 14%. The patient’s subsequent thyroid function tests, autoantibodies, liver and kidney function tests, anti-neutrophilic cytoplasmic antibodies, ceruloplasmin, vitamins D and B12, and folic acid found to be normal. She was diagnosed with hemichorea associated with NKH. The treatments included improving blood glucose with IV insulin and symptomatic treatment of chorea with risperidone 2mg and clonazepam 1mg every night. On sixth day of admission patient’s involuntary movements improved. Blood sugar level normalized after a week. After 10 days, her dance-like symptoms disappeared completely. She tapered off risperidone and clonazepam within 3 months and continued to control blood sugar with premixed insulin after discharge. Conclusion: In conclusion, although uncommon, chorea/ballism associated with nonketotic hyperglycemia can be the first presenting sign of unknown diabetes mellitus or can occur after weeks or even months of poor glycemic control in diabetic patients. Timely neuro imaging examination and active blood glucose control can avoid misdiagnosis and delay in treatment.