Diabetic Striatopathy (Hyperglycemic Hemichorea-Hemiballismus Syndrome) in a Young Patient with Type 1 Diabetes Mellitus in Dar es Salaam, Tanzania: A Case Report

Abstract

Introduction: Diabetic striatopathy, or nonketotic hyperglycemic hemichorea-hemiballismus syndrome, is a rare movement disorder linked to poorly controlled diabetes mellitus. It predominantly affects older women with type 2 diabetes mellitus and presents with characteristic basal ganglia abnormalities on computed tomography (CT) and magnetic resonance imaging (MRI). Even rarer is the presentation in a young patient, which may pose diagnostic and management challenges.

Case presentation: We report a 17-year-old male with poorly controlled type 1 diabetes mellitus presenting with left-sided hemichorea-hemiballismus of acute onset associated with hyperglycemia without ketoacidosis. Brain imaging revealed increased attenuation in the right caudate and putamen on CT and hyperintensity on T1-weighted MRI, consistent with diabetic striatopathy. The abnormal movements abated after 1 month through dietary counseling, increased insulin dosage, and anti-chorea therapy.

Conclusion: Diabetic striatopathy may occur in young patients with type 1 diabetes mellitus. In resource-limited settings, its management can be challenging. There is a need for increased awareness among physicians of this potentially reversible condition, especially when seeing atypical patient populations. Strict glycemic control is an essential part of treatment.

Keywords: Caudate and putamen hyperdensity; Diabetic striatopathy; Hyperglycemic hemichorea-hemiballismus; Nonketotic hyperglycemia; Type 1 diabetes mellitus.

Fig. 1.

a Axial CT scan of the brain showing hyperdense lesions in the right basal ganglia involving the caudate and lentiform nuclei with calcifications. b Axial T1-weighted MRI of the brain showing hyperintense lesions on the corresponding basal ganglia area.