脳幹部グリオームを合併し，Stereoanesthesiaを認めたvon Recklinghausen氏病の1剖検例

AN AUTOPSY CASE OF VON RECKLINGHAUSEN'S DISEASE WITH BRAIN STEM GLIOMA SHOWING STEREOANESTHESIA

I．はじめに

　von Recklinghausen氏病（Neurofibrornatosis gene—ralisata）はvon Recklinghausenが1882年に55歳の女性の皮膚，神経系について記載して以来数多くの報告があり，脳腫瘍などの中枢神経系，骨，内分泌系の異常などを伴いやすい皮膚神経系の先天性異常疾患である。本病（以下R病）に脳腫瘍が合併することは稀ならずあり，Canale^1）ら，Crowe^2）らなどの報告がある。その病理組織学的内訳ではNeurinoma, Meningioma, Gliomaが多い。われわれはこのたび35歳のR病の男性で脳幹部のグリオームを合併した1例の経験したので報告するとともに，R病と脳腫瘍，本例の臨床像について若干の考察を試みたい。

A case of von Recklinghausen's disease with brain stem glioblastoma was reported clinico-pathologically. Neurologic examination on ad-mission disclosed decrease of superficial and deep sensation of a left half of the body as well as stereoanesthesia, muscle weakness of the left upper limb and the clumsiness of fine movements of the hand. Athetosis-like movements were also noted on stretching fingers of the left hand. Thesesymptoms gradually progressed, sensory disturbance of a left half of the body extending to the four limbs. Left hemiparesis became tetraparesis, and signs of increased intracranial pressure set in. In the terminal stage, difficulties in swallowing and respiration appeared. The illness lasted about 14 months. Pathological examination confirmed glio-blastoma multiforme with tendency to necroses and infiltrations.

The authors discussed correlation between von Recklinghausen's disease and brain tumor, especi-ally brain stem tumor reported in the literature. Another point of interest lies in the presence of stereoanesthesia. Stereoanesthesia was noted in the absence of lesions of the cerebral cortex, and the possible relationship was suggested between stereoanesthesia and lesions of the pons and me-dulla oblongata.