Subacute brainstem ischemic syndrome in juvenile neurofibromatosis type 2: An underrecognized condition

We report the case of a teenager with a neurofibromatosis Type 2 (NF2) presenting a locked-in syndrome due to a brainstem ischemic syndrome. The presence of sudden or rapidly worsening onset of neurological deficits in NF2 patients, should evoke this underknown entity and not only tumors as predisposed by NF2.