Moya Moya Disease, Morning Glory Disc Anomaly, and Neurosensorial Hearing Loss
Journal Title: Journal of Neurological Sciences-Turkish - Year 2005, Vol 22, Issue 4
Abstract
Moya moya disease is a rare disorder characterized by small anastomosing vessel network with segmental stenotic and occlusion areas in terminal branches of both internal carotid arteries. This disease may present with paresis, headache, seizure, anopia, nystagmus, aphasia/dysphasia, sensorial symptoms, or some. 26-year-old female patient admitted to our neurology department with a complaint of sudden onset left hemiparesis and left central facial paralysis. Her neurologic examination established left hemiparesis, increased deep tendon reflexes on the left side, left hemihypoesthesia, and extansor plantar response on left. Ophtalmic examination revealed microophtalmus, microcornea, iridodonosis, hypoplastic iris, atrophy of the lense, retinal epithelial pigment dystrophy, and optic disc hypoplasia (morning glory disc anomaly) on her left eye. Ear-nose-throat examination revealed approximately 90dB neurosensorial hearing loss on her left ear with normal external anatomy. Cranial magnetic resonance imaging showed thalamic infarction next to the right internal capsule. Cranial angiography showed supraclinoid occlusion of the right internal carotid artery and prethrombotic stenotic areas on the supraclinoid portion of the left internal carotid artery, with co-existing diffuse collateral vascular changes. Brain single-photon emission computed tomography showed hypoperfused areas on right peri-thalamic area, and diminished response to acetozolamide infusion. The patient was diagnosed as Moya Moya disease, Morning glory disc anomaly and neurosensorial hearing loss. She underwent right dura-myo-angiosis operation followed by left dura-myo-angiosis operation in our neurosurgery department. She is now being followed in our out-patient clinics with no complaints.
Authors and Affiliations
Gulcin BENBIR, Melda BOZLUOLCAY, Birsen INCE
Keywords
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