ミナミ キュウシュウ チホウ ニオケル VON RECKLINGHAUSEN ビョウ ノ ブンプ オヨビ イデン ニツイテノ ケントウ

The diagnostic criteria of VON RECKLINGHAUSEN's disease (neurofibromatosis) are multiple macular pigmentation of the skin, known as café au lait spots and cutaneous and/or subcutaneous neurofibromas, usually multiple. If complicated by intracranial tumors, we diagnose it as central neurofibromatosis. The authors examined, especially for epidemiologic stand point of view, 21 cases of central neurofibromatosis in our department of neurosurgery, The results are as follows:① Central neurofibromatosis is a hereditary disease and its pattern is autosomal dominant.② Half of the patients (48%) that had no occurrence of the disease in their family were sporadic cases caused by natural mutation.③ Many patients occurred in the Satsunan islands. Satsunan islands is one of the condensed occurrene region of central neurofibromatosis.In the serums from patients with VON RECKLINGHAUSEN's disease, an increase of "nerve-growth factor (NGF)" was demonstrated. With a popularization of radioimmunoassay of NGF, the genetic study of VON RECKLINGHAUSEN's disease will become more developed