Abstract

Moyamoya disease (MMD) is a progressive cerebrovascular disorder caused by narrowing of the arteries located at the base of the brain in the basal ganglia. Dystonia is a relatively rare symptom of MMD, and little is known about the treatment of this symptom in MMD patients. We report a case of dystonia in an MMD patient that was alleviated after valproate treatment. A 57-year-old female patient with MMD presented with new-onset dystonia of the left upper extremity and left hemifacial spasm. Although she already suffered from mild cognitive dysfunction, there was no exacerbation when dystonia presented. She was treated with valproate 300 mg b.i.d, and the symptoms were alleviated. We discuss the efficacy of valproate in terms of anatomy and neurotransmitters. In addition, the patient’s symptoms are similar to those associated with LGI-1 encephalitis but are distinguished by the progression of the disease.

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