Cervical Spinal Cord Compression and Demyelinating Neuropathy Complicating Neurofibromatosis Type 1: About A Case

Neurofibromatosis (NF) is a term that has been applied to a variety of related syndromes, characterized by neuroectodermal tumors arising within multiple organs and autosomal-dominant inheritance. At least 8 different clinical phenotypes of neurofibromatosis have been identified and are linked to at least two genetic disorders. Neurofibromatosis type I (NF-1) is the most common type of the disease accounting 90% of the cases. Spinal neurofibromas may cause neurologic symptoms by compressing the spinal cord or spinal roots within the foraminal spaces. We report the case of 64 year old male, Senegalese; admitted in June 2016 at the Neurological Clinic of the National Teaching Hospital-FANN, Dakar-Senegal for a syndrome of slow cervical spinal cord compression and a demyelinating neuropathy of both upper and lower limbs. MRI confirmed compression of the sixth and seventh cervical spine segment, which is in favor of a neurofibroma and electroneuromyography showed sensory and motor impairment with slowing of conduction velocities and latencies. The progression was fatal with death after 34 days.