Mosaic Neurofibromatosis Type 2 with Subdural Hematoma and Hydrocephalus: What Therapeutic Strategy?
ATROUNE L., SELLAMI A., DJOUADI Y., SAADEDDINE C., HACHID A., BENIDER M. ,DJAAFER M.
Neurosurgery Department Mustapha Pacha – Algiers-Algeria
*Corresponding Author: : ATROUNE L.,Neurosurgery Department , Mustapha Pacha University Hospital, Algiers, Algeria
Abstract:
Neurofibromatosis type 2 (NF2) is a rare autosomal dominant genetic disease with a birth incidence of 1 in 25,000. Nearly ¾ of the NF2 gene mutations are sporadic, affecting the Merlin protein on chromosome 22q. Hence, the disease is a tumor suppressor gene on chromosome 22q, encoding Merlin protein.
We report the case of a 63-year-old man who was diagnosed with spastic tetraparesis over six months. The patient had anterior cervical meningioma, calcified right anterolateral meningioma of the foramen magnum, posterior meningioma, schwannoma of the posterior root of C7, and multiple schwannomas of the cauda equina. The diagnosis is based on the Manchester criteria (1992), which include bilateral vestibular schwannomas, first-degree relatives with NF2 and unilateral vs., first-degree relatives with NF2 OR unilateral VS AND two of the following: meningioma, cataract, glioma (intramedullary ependymoma), schwannoma, or multiple meningioma (2 or more). The patient underwent total surgical excision of the cervical meningioma, and regular MRI monitoring allowed for assessment of the lesions' evolution.
Keywords: neurofibromatosis type 2, vestibular schwannomas, meningioma