Hyperglycemic chorea: clinical insights and a narrative review of case reports

Hyperglycemic chorea (HC), also known as diabetic striatopathy, is a rare, potentially reversible complication of poorly controlled diabetes mellitus. It manifests as involuntary, often unilateral, choreiform or ballistic movements. Despite its clinical significance, HC remains underrecognized, and its pathophysiology is not fully understood. We present a case of HC and a narrative review of the published case reports to characterize its epidemiology, clinical features, imaging findings, treatment, and outcomes. We conducted a narrative review of case reports and case series on HC using PubMed/MEDLINE from inception to 1 September 2024, following PRISMA guidelines. Inclusion criteria were English-language reports of adult patients (≥18 years) with chorea attributed to hyperglycemia. A total of 80 publications encompassing 100 patients were included. The mean age was 66.6 years, with a female predominance (67%). Hemichorea was more common (71%), with left-sided preponderance. Most patients (75%) had a prior diagnosis of diabetes, and the mean presenting blood glucose and HbA1c were 468 mg/dL and 13.7%, respectively. Imaging revealed basal ganglia hyperintensity on MRI in 80% of cases. Treatment primarily involved insulin therapy, with 69% receiving adjunctive anti-chorea medications. Symptom resolution occurred in 73.9% of patients, with a mean recovery time of 36.7 days. HC is an important differential diagnosis for acute-onset chorea in elderly patients with uncontrolled diabetes. Prompt recognition and glycemic control are key to symptom resolution. Neuroimaging, particularly MRI, plays a crucial role in diagnosis, and adjunctive pharmacotherapy may aid in symptom management.