von Recklinghausen病のCT所見

CT-FINDINGS OF VON RECKLINGHAUSEN DISEASE

I．はじめに

　von Recklinghausen病（以下von R．病）は，臨床的にはcafé au lait spots （以下CALSと略す） fibro—ma mollsucum （以下FMと略す）によつて特徴づけられる家族性，遺伝性疾患である。1882年報告されて以来，Adrian （1901）が初めて，骨変化を記載し，Brools（1924）がそのレントゲン所見を報告している。近年Holt,Huntらが相次いで多数例を報告した。本疾患のcomputed tomography （以下CTと略す）所見に関する報告は少ない^5,8）。今回著者らは本疾患17例のCTを検討し，その特徴的変化について報告する。

Bone lesions of von Recklinghausen's disease have long been the subject of controversy in the fields of neurology and radiology. Recent application of computed tomograpy to this diesease gave us fol-lowing findings. We studied 17 cases, of which 13 (76%) had abnolmalities. The most frequently encountered abnormality was ventricular dilatation (47%). Bone abnormalities were seen in 5 cases, of which the axial distortion of the skull was especially noteworthy. Of 4 cases of exophthalmos, two had orbital defect, one of which had tumor in vicinity.

Intracranial tumors were demonstrated in tow cases. Though the intracranial tumors were posi-tively contrasted with dye study, the extracranial neurofibromas were scarcely enhanced. These cranial bony lesions were thought to be primarily mesodermal disorder, although in one case tumor and bony defect existed in close correlation.