Trochlear nerve palsy associated with Moyamoya disease

Abstract

A 48-year-old woman with intracranial hemorrhage at the right hemisphere and the right midbrain was diagnosed as Moyamoya disease (MMD). While restoring consciousness, she complained of diplopia. The left hypertropia with a compensatory right head tilt was noted. The follow up brain image showed the evidence of past hemorrhage at the level of the right inferior colliculus. In this case, the hemorrhage directly damaged the right trochlear nucleus or intra-axial trochlear nerve before decussation and caused left (contralateral) hypertropia. To our knowledge, no report has been described of the trochlear nerve palsy in hemorrhagic Moyamoya disease. Here, we present a case of the patient with trochlear nerve palsy after hemorrhagic MMD and summarize the characteristics of trochlear nerve palsy according to its lesion sites.

Moyamoya Disease Associated With Morning Glory Disc Anomaly and Other Ophthalmic Findings: A Mini-Review

“…Diverse ocular manifestations are sometimes consequences of ischemia or hemorrhage in MMD and may differ from the regions involved. A total of 10 cases from eight articles referring to ischemia-or hemorrhage-related ocular manifestations are summarized and reviewed, five of which are from case series ( Table 2) (31)(32)(33)(34)(35)(36)(37)(38). Most patients had a significant ischemia lesion in the cerebral imaging, and only one patient was diagnosed as having a hemorrhage.…”

“…Even after receiving a superficial temporal artery to MCA bypass, he still suffered from diplopia and hypertropia after the operation. His hypertropia and diplopia were considered as consequences of trochlear nerve palsy, for the past hemorrhage had damaged the right trochlear nucleus and intra-axial trochlear nerve (32).…”