Mass Signを呈したNeuro-Behçet’s Syndromeの1例

A CASE OF NEURO-BEHÇET'S SYNDROME SHOWlNG THE MASS SIGN

I．はじめに

　neuro-Behçet's syndromeのなかには，ときに脳占拠性病変を疑わせるような臨床像をとり，実際うつ血乳頭や髄液圧上昇を示す場合も少なくないが，従来報告された症例に於いては，気脳撮影や脳血管撮影でmass signの認められたものはない。今回我々は，脳血管撮影，脳シンチおよび脳室撮影で左視床部にmass signを呈し，診断的開頭術を行なつた症例を経験したので報告し，併せて若干の文献的考察を加える。

The patient is a 35-year-old man, who was dia-gnosed as Behçet's syndrome several years ago. In March 1977, he was admitted to our clinic with chief complaints of headache and right motor weak-ness. His consciousness was almost clear. He had oral aphthae. Neurological examination disclosed right hemiparesis and right hemihypesthesia and he-mihypalgesia, but fundi were impossible to examine because of both artificial eyes. Three lumbar punctures showed initial pressure of 95~240mmH₂O, cell counts of 19/3~398/3 and total protein level of 48~111mg/di. Cultures of the cerebrospinal fluid for bacteria and fungi were negative. Isotope brain and bone scan showed the hot area which was not well demarcated in the left thalamic region. Cerebral angiograms showed the mass sign in the same place as the hot area of scintigrams. Air and conray ventriculogram revealed dilatation of the both lateral ventricles, shift of third ventricle from left to right, moreover the elevation of the floor of the body of the left lateral ventricle. These findings made us decision to do diagnostic crani-otomy. Abnormal tissue taking the size of 2X2X2 cm was found in the left thalamic region. Patho-logically it was the necrotic brain tissue accom-panied by focal hemorrhage and perivascular in-filtration of lymphocytes.

In reviewing the literatures, we could not find any case showing the mass sign by the neuroradio-logic examination, but as our case some ones of Neuro-Behçet's syndrome have the possibility of showing the mass sign by neuroradiologic procedures as well as the elevation of cerebrospinal fluid pres-sere and findings resembling chocked disc.