Case report: Bilateral spinal neurofibromatosis

Spinal neurofibromatosis (SNF) is a rare form of Neurofibromatosis in whichneurofibromas exist bilaterally throughout all spinal roots. Despite previousattempts made to characterize and classify the disease as a separate clinicalform of the disease, the low incidence rate of the disease and scarcity ofprevious reports calls for further studies and reports to elaborate this clinicalentity. The patient in this report was a ۳۶-year-old man presenting with lowerlimb weakness, unsteady gait, and paresthesia. The patient also presentedwith multiple cutaneous café-au-lait spots, cutaneous neurofibromas, and alarge neurocutaneous neurofibroma of right facial nerve. Magnetic resonanceimaging (MRI) of spine revealed bilateral spinal neurofibromas across all spinalcord roots. MRI study of head revealed no abnormalities in the brain and optictract. The patient fulfilled both NIH criteria as well as revised criteria for NF۱.Despite total spinal cord involvement, surgical intervention was withheld fromthe patient due to high propensity of recurrence as seen with previous attemptsin removing peripheral neurofibromas, slowprogression of symptoms, and lackof significant pain and impairment. SNF is often described as a form of diseasewith infrequent presentation of classical NF۱ symptoms other than spinaltumors. The case presented here however, presented with several cutaneousneurofibromas and café-au-lait spots. Considering the positive outcome ofsurgical intervention in a few other reports, the decision to surgically interveneshould be left to the clinical judgement of the participating surgeon, patientpreference and socioeconomic background in a case-by-case manner.