Unilateral Myoclonus as an initial manifestation in Moyamoya disease with asymmetric vasculature.

Movement disorders have been identified as a rare early manifestation of the Moyamoya disease. Herein, we present a case of a 34-year-old man with a genetically confirmed Moyamoya disease who showed unilateral myoclonus as an initial manifestation. Neuroimaging studies showed prominent asymmetrically developed Moyamoya disease on the right hemisphere with near-complete loss of normal vessels while the left hemisphere was adjunctively fed with extension of posterior cerebral artery: uneven progression of vasculature. Tc-hexamethylpropylene amine oxime single-photon emission computed tomography demonstrated impaired vascular reserve. Electroencephalography showed occasional sharp waves on right temporal area. The phenomenon of this patient could be explained in the context of excitable cortex and hypoactive subcortical substrate that might imply putative contradictory neurobiology in Moyamoya disease.