Chorea Hyperglycemia Basal Ganglia (CHBG) Syndrome: A Case Report

Abstract

Chorea, characterized by sudden, involuntary movements of the face and limbs, arises from various causes, including neurodegenerative diseases, metabolic disorders, and structural brain changes, notably in the basal ganglia. Acute lesions in the basal ganglia due to ischemia or vascular pathology can also precipitate chorea. Hyperglycemia-induced basal ganglia changes, termed chorea hyperglycemia basal ganglia, predominantly affect elderly females with type 2 diabetes. We report a 62-year-old female with poorly managed diabetes presenting with involuntary jerking movements, initially in the right leg, progressing to the right arm, face, and lips over three days. Her history included hyperlipidemia and hypertension, and lab results showed significant hyperglycemia (601 mg/dL) [fasting <140 mg/dl], hyponatremia, renal impairment, and a high Hemoglobin A1C (HbA1c) (10.4) [<6 %]. Imaging revealed left putamen hypodensity on Computed Tomography (CT) and confirmed microhemorrhage on magnetic resonance imaging (MRI). Diagnosed with Hyperosmolar Hyperglycemic State (HHS) and hemichorea, she was treated with intravenous (IV) insulin and fluids, leading to symptom resolution within two days. This case highlights the link between non-ketotic hyperglycemia and chorea, involving hyperviscosity-induced GABAergic neuron dysfunction in the putamen. Diagnosis relies on choreiform movements, elevated blood glucose, and striatal hyperintensity on T1 MRI. Effective management includes treating underlying HHS with hydration and glycemic control, occasionally supplemented with anti-chorea medications. Recognizing diabetic striatopathy is crucial for prompt treatment and symptom resolution, emphasizing the need for early diagnosis and intervention in patients with uncontrolled diabetes presenting with new-onset chorea.

Keywords: Basal ganglia; Chorea; Diabetic striatopathy; Hemichorea-hemiballismus; Hyperosmolar hyperglycemic state (HHS); Movement disorders; Non-ketotichyperglycemia; Putamen microhemorrhage; Type 2 diabetes mellitus.