Oculomotor nerve palsy in neurofibromatosis type 2

Abstract

Neurofibromatosis (NF) type 2 is a rare neurological, autosomal dominant and genetic disorder. It is caused by a mutation in the tumor suppressor gene, called NF2 gene. The disorder results in several benign tumors of the nervous system. These typically include vestibular schwannomas, meningiomas, and ependymomas. Multiple cranial nerve abnormalities affect the brain, spinal cord, nerves, and skin and cause significant morbidity in patients. We describe a 20-year-old patient, with a family history of brain tumors, with symptoms of left sided third nerve palsy. Magnetic Resonance Imaging (MRI) of the brain and orbits revealed a small sized cavernous sinus meningioma and bilateral vestibular schwannomas. As per the differential diagnosis and optimal resolution brain imaging, NF2 was diagnosed. The patient was referred for specific treatment to the neuro-oncology unit. The case is distinct as the patient presented with a parasellar meningioma leading to third nerve palsy besides bilateral vestibular schwannomas. Manchester criteria and high contrast MR imaging proved more beneficial in our patient for the diagnosis of a wider clinical spectrum of NF2.

Keywords

• NF2
• Radiology
• Manchester criteria
• Schwannoma
• Cranial nerves
• Neurology