Generalized dystonia : unusual mode of revelation of Moyamoya disease

Abstract

The Moyamoya disease is defined by bilateral hypoplasia of the internal carotid arteries, forming an abnormal arterial network of substitution. Movement disorders including dystonia are exceptionally reported in this context. We report the case of a 46 year old woman with a history of hypertension, dyslipidemia and diabetes, which has since the age of 43 years of gait disorders and abnormal attitude of the trunk. Neurological examination found a generalized dystonia involving the tongue, neck, trunk, upper limbs and lower fees. Brain MRI revealed multiple ischemic lesions in the white matter subcortical and deep. MR angiography showed a consistent appearance with Moyamoya disease. The indication of an anastomosis of the superficial temporal artery and the middle cerebral artery was challenged. The patient improved with medical treatment. Thus, dystonia can be a fashion unusual revelation of Moyamoya disease. Angio-MR seems necessary in the workup of a dystonia.