Familial Involvement of the Nervous System by Multiple Tumors of the Sheaths and Enveloping Membranes: Hereditary, Clinical, and Pathological Study of Central and Peripheral Neurofibromatosis

摘要: Von Recklinghausen (1) in 1882 recognized the fact that presence of “multiple neuromas” could not be considered as a purely acquired disease, but Thomson (2) 1900 was first to point out clearly condition hereditary. Since time hereditary and familial character disease has been fully established is generally recognized. Cases multiple neurofibromatosis with diffuse involvement central nervous system are unusual. The association this meningeal tumors rare more so when there distinct background. present communication clinico-pathological study von Recklinghausen9s peripheral associated glial tumors. Review Literature Multiple or may looked upon congenital anomaly dysontogenetic sense, according Hosoi (3). Penfield (4) describes “familial which fibro-blastomas, gliomas sarcomas occasionally encountered.” Preiser Davenport (5) reviewed literature collected 30 cases type. They were able show appears both sexes follows mendelian law dominant trait. Hoekstra (6) 1922 also pointed tendency condition. While affecting mainly nerves (7), system, though unusual, by no means rare. German (8), reporting changes ear bilateral acoustic nerve tumors, states over have recorded. reported recently van Bogaert (9), Lewin (10), de Klejn Gray (11), others. Of interest respect occurrence Although these rarely occur without evidence elsewhere at it conceded they represent local manifestation general process. Gardner Frazier (12), review literature, collect 44 nerve, 37 neurofibromatosis. In addition, report family 38 members affected deafness autopsied shown due transmitted dominant. Roger, Alliez Sarradon (13) described verified autopsy one member clinical cerebellar tumor four These writers believe sufficient warrant full acceptance Frazier9s view part neurofibromatosis, transferred reviewing Roger his associates find only 7 convincing histories intracranial involvement.