Case Presentation: A 58 year old female with history of hypertension, hypothyroidism and noninsulin dependent diabetes mellitus presented with two days of involuntary movements of her right upper extremity. Bloodwork was remarkable for blood glucose 1273MG/DL, anion gap 15, serum creatinine 2.0 MG/DL from normal baseline, no ketosis. Infectious workup with urinalysis, respiratory viral panel and chest xray was negative. CT angiogram of the brain and neck was normal. She was admitted to the ICU for management of Hyperosmolar Hyperglycemic State (HHS). HbA1c later returned at 16%. MRI of the brain was ordered, but not pursued as her choreiform movements had resolved within 24 hours of correction of her blood glucose. Her involuntary movements were attributed to diabetic hemiballism.
Discussion: Diabetic hemiballism is a rare metabolic syndrome characterized by the triad of hemichorea-hemiballismus, hyperglycemic state, and unique abnormalities limited to the striatum on neuroimaging. It goes by many names: 1. Nonketotic Hyperglycemia Chorea-Ballismus, 2. Hyperglycemic Nonketotic Hemichorea/Hemiballism, 3. Diabetic Hemiballism/Hemichorea, 4. Chorea, Hyperglycemia, Basal Ganglia Syndrome, and 5. Diabetic Striatopathy.The estimated prevalence of this disorder is less than 1 in 100,000 people. Age of onset is 50–80 years of age with a female to male ratio of 1.8: to 1. The typical presentation is an acute onset choreiform movement of one side of the body that occurs over a period of hours in the setting of severe, nonketotic hyperglycemia such as Hyperosmolar Hyperglycemic State (HHS). These movements are described as involuntary, irregular, unpredictable, and nonrhythmic. They generally develop while awake, and disappear during sleep. Neuroimaging shows hyperintense lesions in the striatum on MRI brain, hence the term diabetic striatopathy. The most commonly affected area is the putamen, followed by the caudate. Clinical and imaging findings are typically unilateral. Prognosis is often favorable. Aggressive glucose control can result in resolution of chorea within 24-48hrs of normalization of blood glucose, as was the case with our patient. MRI findings typically resolve within 2-12 months.
Conclusions: Diabetic hemiballism is a rare metabolic syndrome characterized by the triad of hemichorea-hemiballismus, hyperglycemic state, and unique abnormalities limited to the striatum on neuroimaging. Prompt recognition of this syndrome, coupled with aggressive glucose management can lead to favorable outcomes including rapid resolution of chorea, and complete resolution of neuroimaging abnormalities.