Objective: Improve recognition of Diabetic Striatopathy (DS) as a stroke mimic causing acute hemichorea-hemiballismus, highlight factors leading to prolonged recovery, and propose tetrabenazine as a non-neuroleptic first-line therapy.

Background: DS is an acute onset movement disorder caused by poorly controlled diabetes mellitus (DM), marked by a triad of non-ketotic hyperglycemia, hemichorea-hemiballismus, and specific neuroimaging changes in the basal ganglia [1]. Although rare, DS is the second most common cause of acute hemichorea-hemiballismus overall, and the most common cause among metabolic derangements [2]. Early recognition and glycemic control are key to potentially reversing the condition.

Method: Case report

Results: A 69-year-old man with long-standing poorly controlled type 2 DM, hypertension, hyperlipidemia, and former tobacco abuse developed abrupt onset left-sided hemichorea-hemiballismus with acute hyperglycemia (>400 mg/dL). CT brain was normal; MRI was unavailable due to a medically underserved location at the time of presentation. The initial working diagnosis was stroke. Symptoms persisted despite glycemic correction. A month-long trial of haloperidol, clonazepam, and valproic acid did not resolve the symptoms, prompting him to seek a higher level of care at our center.  The hyperglycemia was improved (137 mg/dL), and hemoglobin A1c was 7.3%. MRI brain with and without contrast revealed a circumscribed lesion in the right putamen that was hyperintense on T1 and hypointense on T2 but without diffusion restriction. Fingings suggested DS rather than stroke. Further testing ruled out toxic-metabolic, infectious, and autoimmune causes. He was admitted to inpatient rehabilitation for two weeks with tight glycemic control and started on tetrabenazine, leading to rapid clinical improvement. Within two weeks, all other medications were weaned off, and symptoms remained controlled on tetrabenazine monotherapy. At four-month follow-up, the chorea had completely resolved.

Conclusion: This case underscores the importance of recognizing DS as a stroke mimic, the potential for prolonged hemichorea-hemiballismus even after glycemic control, and the successful use of tetrabenazine as a non-neuroleptic treatment. Further research is needed to understand the pathophysiology of DS and refine management strategies.

References: 1. Wang, X, Zhang, Y, Yang,F, Bao,S, Duan, L, Jiang X. Further learning of clinical characteristics and imaging manifestations of nonketotic hyperglycemic hemichorea. Journal of Diabetes. 2024 16:e13543.
2. Arreco, A, Ottaviani, S, Boschetti, M, Renzetti, P, Marinelli, L. Diabetic Striatopathy: an updated overview of current knowledge and future perspectives. Journal of Endocrinological Investigation (2024) 47: 1-15.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/diabetic-striatopathy-treated-with-tetrabenazine-a-case-report-and-discussion-on-a-commonly-forgotten-stroke-mimic/