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Brain and Nerve No to Shinkei Volume 55, Issue 3 （March 2003）

Brain and Nerve 脳と神経 55巻3号（2003年3月発行）

Japanese English

症例報告

全身性AVMを伴ったLhermitte-Duclos diseaseの1例 A Case Report of Lhermitte-Duclos Disease with Systematic AVMs 奥永知宏 ^1,3 , 高畠英昭 ¹ , 中村稔 ¹ , 岩崎啓介 ² Tomohiro Okunaga ^1,3 , Hideaki Takahata ¹ , Minoru Nakamura ¹ , Keisuke Iwasaki ² ¹佐世保市立総合病院脳神経外科 ²佐世保市立総合病院病理 ³長崎大学医学部附属病院脳神経外科 ¹Department of Neurosurgery, Sasebo General Hospital ²Department of Pathology, Sasebo General Hospital ³Department of Neurosurgery, Nagasaki University School of Medicine キーワード： Lhermitte-Duclos disease , Cowden disease , arteriovenous malfomation（AVM） , parallel linear striation Keyword: Lhermitte-Duclos disease , Cowden disease , arteriovenous malfomation（AVM） , parallel linear striation pp.251-255

発行日 2003年3月1日 Published Date 2003/3/1

DOI https://doi.org/10.11477/mf.1406100458

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Abstract 文献概要
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要旨　左上下肢に巨大なarteriovenous malformation（AVM）を伴ったLhermitte-Duclos disease（LDD）の1例を経験した。症例は13歳時にAVMを指摘され，左右シャントのために心不全をきたし加療された既往があった。46歳時意識消失発作を起こし，当院入院となった。頭部MRIで小脳虫部にparallel linear striationを呈する腫瘍を認め，後頭蓋下開頭腫瘍部分摘出術を施行した。病理診断はdysplastic gangliocytomaであり，LDDと診断した。LDDとCowden diseaseの関係，Cowden diseaseとAVMの関係は示唆されているが，本例のようにLDDとAVMの合併を報告した例はない。本例は，LDD，Cowden disease，AVMの三者の関係を示唆する興味深い症例と考えられた。

We report a case of Lhermitte-Duclos disease（LDD） with huge AVMs of left extremities. The patient is a 46-year-old woman, who was identified heart failure due to AVMs at 13 years old and underwent amputation of left arm and several embolizations for AVM of left leg.

　Following a loss of consciousness, she was admitted to our hospital at 46 years old. CT scan showed a low-density area in the vermis of cerebellum. MR imaging showed a tumor with characteristic parallel linear striation. She was treated with partial resection of tumor. Pathological findings were dysplastic gangliocytoma. She was diagnosed LDD by MR imaging and pathological findings.

　Some reports describe an association between LDD and Cowden disease. On the other hand, there are reports of an association between Cowden disease and AVM. However, to our knowledge, there is no report of an association between LDD and AVM.

　Although this case didn't be revealed an association with Cowden disease, we believe that this case is a very interesting one henceforth suggesting the association between LDD, Cowden disease, and AVM.