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Brain and Nerve No to Shinkei Volume 25, Issue 12 （December 1973）

Brain and Nerve 脳と神経 25巻12号（1973年12月発行）

Japanese English

特集脳神経外科論文集

Ataxia-Telangiectasiaに伴つた原発性脳腫瘍の2剖検例 TWO AUTOPSY CASES OF ATAXIA-TELANGIECTASIA WITH BRAIN TUMOR 川口進 ¹ , 大沼悌一 ² , 下田肇 ² , 松谷善吉 ³ , 塚本考 ³ , 吉村教皞 ⁴ Susumu Kawaguch ¹ , Teiichi Onuma ² , Hazime Shimoda ² , Zenkichi Matsuya ³ , Takashi Tsukamoto ³ , Noriaki Yoshimura ⁴ ¹弘前大学医学部脳神経外科 ²弘前大学医学部神経精神科 ³青森県立さわらび園 ⁴新潟大学脳研究所神経病理 ¹Dept. of Neurosurgery, Hirosaki University, School of Medicine ²Dept. of Neuropsychiatry, Hirosaki University ³Sawarabi Institute for Cripped Children ⁴Dept. of Neuropathology, Brain Research Institute, Niigata University pp.1817-1822

発行日 1973年12月1日 Published Date 1973/12/1

DOI https://doi.org/10.11477/mf.1406203456

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Abstract 文献概要
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Ｉ．はじめに

　Louis-Ber症候祥は1941年Madam D．　Louis^1）によつて初めて記載され，進行性小脳性運動失調，眼球結膜および皮膚および皮膚に毛細血管拡張を有するもので，1957年Boder^2）らは本症の綜説を行ないataxia-telangi—ectasiaと命名した。

　本症の特徴は遺伝性家族性失調症にぞくし，かつpha—komatosesの範疇にも入り，一面，免疫異常が関与し，悪性腫瘍を合併することも多いといわれている^10）14）。

The autopsy findings of two cases of ataxia-telangiectasia who had primary brain tumor were reported. Case I was 10 year old boy with reti-culosarcoma in the Rt frontal lobe and case II was 16 year old girl with medulloblastoma in the cerebellum. The disease of ataxia-telangiectasia were rare condition of childhood, characterized by progressive ataxia, oculocutaneous telangiectasia, low immunoglobulin in serum, frequent infection and occasional death from malignant tumor.

From the literatures, there were about 10 cases of ataxia telangiectasia who had malignant tumor and only 2 of them were of primary intracranial in origin.

Young (1964) reported mixed glioma in the frontal lobe and Schuster (1964) medulloblastoma in cerebellum. No reports were made in Japan so far. The primary intracranial tumors occurred in the course of ataxia telangiectasia like our cases, are therefore considered to be extremely rare.

Further discussion was made as to the possible relation to the hypof unction of thymus to the immunological abnormality, viral infection and malignant tumor.