A Case of Bilateral Upgaze Palsy associated with Unilateral Midbrain Hemorrhage in Moyamoya Disease

ABSTRACT

PURPOSE: To report a case of bilateral upgaze palsy associated with unilateral midbrain hemorrhage in moyamoya disease. METHODS: A 29-year-old woman presented with a sudden decrease of consciousness and was diagnosed with a spontaneous brain hemorrhage in the right side of the midbrain, right basal ganglion, and third ventricle. The mentality was improved after external ventricular drainage of hemorrhage, but bilateral upgaze difficulty remained. We performed neuro-ophthalmic and radiologic evaluation of the cause of bilateral upgaze difficulty. RESULTS: She showed a upgaze limitation of -2 grade in the right eye and -3 grade in the left eye, but normal movements in other versions and ductions. There were normal responses in pupillary light reflex, forced duction and convergence tests, but no ocular elevation in the vertical vestibulo-ocular reflex. The Bell's phenomenon was absent. She was diagnosed with moyamoya disease through brain MRI and cerebral angiography, and we found a hemorrhage in the right area of the midbrain. This hemorrhagic area was consistent with the right rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF). We think that the bilateral upgaze palsy was caused by damage to the riMLF in the affected side and to projections through the posterior commissure.