Abstract
Neurofibromatosis is a dominant autonomic inherit disease. It englobes seven different types, but only NF1 and NF2 are common. In this study, only neurofibromas will be considered. From the beginning and for a very long time, its peripheral nervous system positioning will progressively occasioned a slowly spinal cord compression We report retrospectively a history of 4 patients in Dakar from 2000 to 2009. All the patients came to hos- pital with huge motor deficit (spastic or flasco-spas- modic stage) and were operated in neurosurgery. The sex ratio was 3 men for 1 woman (3/1). Our patients were aged between 4 and 45 years old, the average was 26, 75 years old. We didn’t found a familial history of NF1 in this study. They had all radiographies, myelography, cervical TDM or myeloscan We found in all cases an NF1 disease’s clinical signs like: nodes on the all body in 3 cases, brown spots on the skin in 1 cases, dorso lombar scoliosis in 1 case,anterior cervical mass in 1 case, in front of C2-C4 and a royal tumor in 1 case. In 100% of cases, pain was the first aspect accompanied with tetrapyramidal syndrom. In all patients, we had paresthesia. Only 1 patient complained for sphincter dysfunction. Cervical X ray exam (face with profile) were characteristical in 1 case and showed an enlargement foramen. In 75% of patients who underwent myelography and/or myeloscan, we found - in 1 of cases a multiples lesions. - in 1, a bilobulated tumor (one part intra canalar and the another one extra foraminal). The surgical resection was incomplete in 1 case and complete in all those. We did laminectomy in 3 cases. Only the children aged 4 years old underwent laminotomy with laminoplasty at the end of surgical procedure. Finally, histo-anatomo-pathology exam confirm that it was a neurofibroma in all cases. All constituents of the nerve was hypertrophy ( schwannoma gaine, fibroblastic cells, collagen, myelin, nervous fiber etc). NF1’s gen is localised on Chromosom 17. This entity was described for the first time by Von Recklinghausen in 1882 and associated skin lesion, osseous lesion and peripheral nervous desease. NF1 is responsable for important aesthetic damages, psycho sociologic disinterest and engage the functional and vital prognostics when
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