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Brain and Nerve No to Shinkei Volume 41, Issue 6 （June 1989）

Brain and Nerve 脳と神経 41巻6号（1989年6月発行）

Japanese English

原著

両手筋萎縮を伴った家族性痙性対麻痺の兄弟例 Two CASES OF FAMILIAL SPASTIC PARAPARESIS WITH AMYOTROPHY OF THE HANDS 山田隆良 ¹ , 田代邦雄 ¹ , 森若文雄 ¹ , 藤木直人 ¹ , 伊藤和則 ¹ , 本間早苗 ¹ , 松浦亨 ¹ , 土井静樹 ¹ Takayoshi Yamada ¹ , Kunio Tashiro ¹ , Fumio Moriwaka ¹ , Naoto Fujiki ¹ , Kazunori Ito ¹ , Sanae Honma ¹ , Tohru Matsuura ¹ , Shizuki Doi ¹ ¹北海道大学医学部神経内科 ¹Department of Neurology, Hokkaido University school of medicine pp.583-588

発行日 1989年6月1日 Published Date 1989/6/1

DOI https://doi.org/10.11477/mf.1406206333

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Abstract 文献概要
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　抄録　青少年期に軽度の歩行障害にて初発し，ついで両手の筋萎縮をきたした家族性痙性対麻痺の兄弟例を報告した。症例1は18歳，男性。12歳頃より軽度の痙性歩行，17歳頃より両手の筋萎縮をきたしている。症例2は20歳，男性。症例1の兄で，小学生頃より軽度の痙性歩行，19歳時になって両手の筋萎縮が出現した。両症例の検索により，電気生理学的に脊髄側索の他に前角，後索の病変が示唆され，神経放射線学的に脊髄の軽度の萎縮，筋組織学的に，神経原性の変化が認められた。両症例の妹にも軽度の痙性対麻痺を認めたが，筋萎縮はなかった。手の筋萎縮を認めた家族性痙性対麻痺は文献的にはSilverの報告を認めるのみで，極めて稀なものということができる。

Familial spastic paraparesis with amyotrophy of the hands was reported, and its significance in the literature was reviewed.

Case 1: An 18 year-old boy, who had been suffering from spastic gait since 12 years old, no-ticed his hand muscle wasting distributed bilateral first interossei muscle, thenar and hypothenar muscle at age 17.

Case 2 : A 20 year-old man, elder brother of case 1, who also walked in spastic manner from his childhood, developed bilateral hand muscle atro-phy similar to case 1 at age 19.

Clinical features of these two cases could be summerized as familial spastic paraparesis with amyotrophy caracterized by hand muscle atrophy, spasticity of lower extremities with hyperreflexia and bilateral positive pathological reflexes and spastic gait. Their younger sister was also ex-amined, who showed only minimal spastic para-paresis.

The electrophysiological examination including EMG and SEP suggested the pathological process could involve not only lateral column, but also posterior column and anterior horn. Slight but generalized spinal cord atrophy was demonstrated on metrizamide CT myelography. The muscle biopsy performed from left gastrocnemius in case 2, confirmed neurogenic changes.

Although the association of retinal degeneration, cataracta, mental retardation, pes cavus or even generalized amyotrophy has been reported in familial spastic paraparesis, only limited cases are available, dealing with the amyotrophy of limbs. As far the cases with amyotrophy localized to the hands are concerned, it is absolutely rare and only the cases reported by Silver could be re-garded as similar or same clinical entities to our cases.