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Neurological examination showed a mild unconsciousness, asterixis, symmetrical akinesia, rigospasticity, hyperreflexia, foot clonus, and extensor toes. Brain MRI disclosed symmetrical hyperintense lesion on T2-weighted images in the centrum semiovale, posterior limb of internal capsule, and cerebral peduncle. The serum ammonia level was increased to 155 μg/dL, whereas the levels of serum copper and caeruloplasmin were normal. The electroencephalogram did not show triphasic wave. Abdominal contrast CT examination revealed a large splenorenal shunt between splenic vein and left renal vein. The patient was diagnosed as acquired hepatocerebral degeneration (AHD) caused by splenorenal shunt. A protein-restricted diet and lactulose preparations ameliorated neurological symptoms and normalized serum ammonia level to 34 μg/dL. However, the abnormalities in the brain MRI were unchanged after the treatment. We report the case an AHD patient who had an unique hyperintense pyramidal tract lesion on T2-weighted magnetic resonance images. Future studies are required to clarify underlying pathophysiological mechanisms causing pyramidal tract lesion."}]}, "item_10001_textarea_6": {"attribute_name": "抄録(日)", "attribute_value_mlt": [{"subitem_textarea_language": "ja", "subitem_textarea_value": "患者は68歳女性.5か月前より徐々に動作緩慢や歩行障害が出現し当科を受診した.神経学的所見では,軽度の意識障害とasterixis を認め,左右差のない無動,痙固縮,下肢腱反射亢進,足クローヌスおよびバビンスキー徴候があった.頭部MRI 検査では,半卵円中心から内包後脚,および大脳脚にかけて拡散強調画像,T2強調画像,FLAIR 画像で高信号,T1強調画像で低信号の病変を認めた.採血ではアンモニア値が155 μg/dL と高値で,血清銅やセルロプラスミンは正常範囲内であった.脳波検査で基礎律動はθ波であったが,三相波は認められなかった.腹部造影CT 検査では,巨大な脾静脈-左腎静脈シャントを認め,Acquired hepatocerebral degenerationと診断した.蛋白制限食とラクツロース製剤内服を開始し,意識障害やパーキンソニズムなどの神経症状は改善し,アンモニア値は34 μg/dL と正常化したが,頭部MRI 病変の改善は認められなかった.本例は巨大脾腎シャントによるAcquired hepatocerebral degeneration(AHD)と考えられた.頭部MRIT2強調画像で両側錐体路に高信号病変を認めるAHD が散見され,文献的考察を含めて報告する. 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頭部MRI上両側錐体路にT2高信号域を認めた脾静脈-腎静脈シャントによるAcquired hepatocerebral degeneration
https://kwmed.repo.nii.ac.jp/records/1888
https://kwmed.repo.nii.ac.jp/records/188880a6e565-e4a0-4349-8195-e3646d8e0ce2
名前 / ファイル | ライセンス | アクション |
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PDF (1.0 MB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2018-03-01 | |||||
タイトル | ||||||
言語 | ja | |||||
タイトル | 頭部MRI上両側錐体路にT2高信号域を認めた脾静脈-腎静脈シャントによるAcquired hepatocerebral degeneration | |||||
タイトル | ||||||
言語 | en | |||||
タイトル | Acquired hepatocerebral degeneration caused by a large splenorenal shunt with hyperintense signal changes in the bilateral pyramidal tract on T2-weighted magnetic resonance imaging | |||||
言語 | ||||||
言語 | jpn | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
深井, 雄太
× 深井, 雄太× 大澤, 裕× 久徳, 弓子× 砂田, 芳秀× FUKAI, Yuta× OHSAWA, Yutaka× KUTOKU, Yumiko× SUNADA, Yoshihide |
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著者所属(日) | ||||||
ja | ||||||
川崎医科大学神経内科学 | ||||||
著者所属(日) | ||||||
ja | ||||||
川崎医科大学神経内科学 | ||||||
著者所属(日) | ||||||
ja | ||||||
川崎医科大学神経内科学 | ||||||
著者所属(日) | ||||||
ja | ||||||
川崎医科大学神経内科学 | ||||||
著者所属(英) | ||||||
en | ||||||
Department of Neurology, Kawasaki Medical School | ||||||
著者所属(英) | ||||||
en | ||||||
Department of Neurology, Kawasaki Medical School | ||||||
著者所属(英) | ||||||
en | ||||||
Department of Neurology, Kawasaki Medical School | ||||||
著者所属(英) | ||||||
en | ||||||
Department of Neurology, Kawasaki Medical School | ||||||
キーワード | ||||||
言語 | ja | |||||
主題Scheme | Other | |||||
主題 | 頭部MRI | |||||
キーワード | ||||||
言語 | ja | |||||
主題Scheme | Other | |||||
主題 | 錐体路 | |||||
キーワード | ||||||
言語 | ja | |||||
主題Scheme | Other | |||||
主題 | 脾腎シャント | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Acquired hepatocerebral degeneration | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Brain magnetic resonance image (MRI) | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Pyramidal tract | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Splenorenal shunt | |||||
キーワード | ||||||
言語 | en | |||||
主題Scheme | Other | |||||
主題 | Acquired hepatocerebral degeneration | |||||
抄録(日) | ||||||
ja | ||||||
患者は68歳女性.5か月前より徐々に動作緩慢や歩行障害が出現し当科を受診した.神経学的所見では,軽度の意識障害とasterixis を認め,左右差のない無動,痙固縮,下肢腱反射亢進,足クローヌスおよびバビンスキー徴候があった.頭部MRI 検査では,半卵円中心から内包後脚,および大脳脚にかけて拡散強調画像,T2強調画像,FLAIR 画像で高信号,T1強調画像で低信号の病変を認めた.採血ではアンモニア値が155 μg/dL と高値で,血清銅やセルロプラスミンは正常範囲内であった.脳波検査で基礎律動はθ波であったが,三相波は認められなかった.腹部造影CT 検査では,巨大な脾静脈-左腎静脈シャントを認め,Acquired hepatocerebral degenerationと診断した.蛋白制限食とラクツロース製剤内服を開始し,意識障害やパーキンソニズムなどの神経症状は改善し,アンモニア値は34 μg/dL と正常化したが,頭部MRI 病変の改善は認められなかった.本例は巨大脾腎シャントによるAcquired hepatocerebral degeneration(AHD)と考えられた.頭部MRIT2強調画像で両側錐体路に高信号病変を認めるAHD が散見され,文献的考察を含めて報告する. | ||||||
抄録(英) | ||||||
en | ||||||
68-year-old woman was admitted to our hospital for five months’ episode of progressive gait disturbance and bradykinesia. Neurological examination showed a mild unconsciousness, asterixis, symmetrical akinesia, rigospasticity, hyperreflexia, foot clonus, and extensor toes. Brain MRI disclosed symmetrical hyperintense lesion on T2-weighted images in the centrum semiovale, posterior limb of internal capsule, and cerebral peduncle. The serum ammonia level was increased to 155 μg/dL, whereas the levels of serum copper and caeruloplasmin were normal. The electroencephalogram did not show triphasic wave. Abdominal contrast CT examination revealed a large splenorenal shunt between splenic vein and left renal vein. The patient was diagnosed as acquired hepatocerebral degeneration (AHD) caused by splenorenal shunt. A protein-restricted diet and lactulose preparations ameliorated neurological symptoms and normalized serum ammonia level to 34 μg/dL. However, the abnormalities in the brain MRI were unchanged after the treatment. We report the case an AHD patient who had an unique hyperintense pyramidal tract lesion on T2-weighted magnetic resonance images. Future studies are required to clarify underlying pathophysiological mechanisms causing pyramidal tract lesion. | ||||||
書誌情報 |
ja : 川崎医学会誌 en : Kawasaki medical journal 巻 43, 号 2, p. 95-100, 発行日 2017 |
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出版者 | ||||||
言語 | ja | |||||
出版者 | 川崎医学会 | |||||
ISSN | ||||||
収録物識別子タイプ | PISSN | |||||
収録物識別子 | 0386-5924 | |||||
ISSN | ||||||
収録物識別子タイプ | EISSN | |||||
収録物識別子 | 2758-089X | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN00045593 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN12940574 | |||||
DOI | ||||||
関連タイプ | isIdenticalTo | |||||
識別子タイプ | DOI | |||||
関連識別子 | https://doi.org/10.11482/KMJ-J43(2)95 | |||||
記事種別(日) | ||||||
内容記述タイプ | Other | |||||
内容記述 | 症例報告 | |||||
言語 | ja | |||||
記事種別(英) | ||||||
内容記述タイプ | Other | |||||
内容記述 | Case Report | |||||
言語 | en | |||||
関連サイト | ||||||
識別子タイプ | URI | |||||
関連識別子 | http://igakkai.kms-igakkai.com/wp/wp-content/uploads/2017/KMJ-J43(2)95.pdf | |||||
著者版フラグ | ||||||
出版タイプ | VoR | |||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 |