Objective: To describe a video-case report of hemichorea-hemiballism improved by valbenazine therapy

Background: Hemichorea-hemiballism (HCHB) is an uncommon movement disorder with numerous etiologies including structural lesions, i.e.stroke, and metabolic disorders, i.e. nonketotic hyperglycemia. HCHB can be disabling and treatment challenging. Dopamine receptor blocking agents have variable efficacy in HCHB and have potential adverse effects including tardive dyskinesia and metabolic syndrome. Dopamine depleting agents have been approved for treatment of chorea in Huntington’s disease and tardive dyskinesia and have shown promise in treatment of other hyperkinetic disorders.

Method: A video-case report of a subject with HCHB of unclear etiology, possibly due to nonketotic hyperglycemia. Subject was examined and videotaped prior to valbenazine treatment. He was started on valbenazine 40mg qd and examined after 4 weeks. Valbenazine was then increased to 80mg qd and he was examined and videotaped 8 weeks later. Clinical evaluation included AIMS (Abnormal Involuntary Movement Scale).

Results: Case report: 90-year-old man with coronary artery disease, hypertension, and diabetes mellitus subacutely developed involuntary movements of the left arm and leg. He had been experiencing uncontrolled hyperglycemia (blood glucose reported as high as 500) for 3 weeks prior to onset. Movements worsened to the point of self-injury and he was admitted to hospital. On admission he displayed left hemiballism. Head CT showed chronic left putaminal lacune (present on previous head CT prior to onset of HCHB) and no acute pathology. He was unable to have brain MRI due to pacemaker. During his hospital course glucose levels normalized, movements were less ballistic though he continued to have disabling hemichorea. He did not respond to olanzapine, risperidone or valproic acid. AIMS score was 16. Valbenazine 40mg qd was started and at 4 weeks hemichorea improved (AIMS score=10). Valbenazine dose was increased to 80mg qd and 8 weeks later he displayed minimal hemichorea with AIMS score of 3. Valbenazine was well-tolerated.

Conclusion: In this video-case report a subject with HCHB demonstrated clear improvement on valbenazine. The available data suggest possible nonketotic hyperglycemia as the etiology for HCHB though an ischemic infarct could not be definitely ruled out. Regardless of etiology, this case of HCHB suggests that valbenazine is an effective and well-tolerated treatment.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/treatment-of-hemichorea-hemiballism-with-valbenazine/