高度の脳幹浮腫を呈したchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids（CLIPPERS）の1例
症例は66歳の女性である．約1ヵ月の経過で小脳性運動失調と意識障害が増悪し，頭部MRI FLAIR強調画像で脳幹の腫大，および中脳，橋，小脳脚，右小脳半球，右後頭葉に高信号をみとめた．造影MRIでは橋に造影効果を有する多発性の点状病変をみとめ，右後頭葉にも造影病変をみとめた．ステロイド薬により臨床症状と画像所見は急速に改善したが減量後に再燃した．右後頭葉の生検で血管周囲にT細胞を主とする炎症性細胞浸潤をみとめchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids（...
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| Published in | 臨床神経学 Vol. 54; no. 7; pp. 581 - 584 |
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| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経学会
2014
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0009-918X 1882-0654 |
| DOI | 10.5692/clinicalneurol.54.581 |
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| Abstract | 症例は66歳の女性である．約1ヵ月の経過で小脳性運動失調と意識障害が増悪し，頭部MRI FLAIR強調画像で脳幹の腫大，および中脳，橋，小脳脚，右小脳半球，右後頭葉に高信号をみとめた．造影MRIでは橋に造影効果を有する多発性の点状病変をみとめ，右後頭葉にも造影病変をみとめた．ステロイド薬により臨床症状と画像所見は急速に改善したが減量後に再燃した．右後頭葉の生検で血管周囲にT細胞を主とする炎症性細胞浸潤をみとめchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids（CLIPPERS）と診断した．ステロイド薬増量と維持によりこれらの病変は消失し寛解した．MRIで高度脳幹浮腫を呈する疾患としてCLIPPERSを考慮する必要がある． |
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| AbstractList | 症例は66歳の女性である．約1ヵ月の経過で小脳性運動失調と意識障害が増悪し，頭部MRI FLAIR強調画像で脳幹の腫大，および中脳，橋，小脳脚，右小脳半球，右後頭葉に高信号をみとめた．造影MRIでは橋に造影効果を有する多発性の点状病変をみとめ，右後頭葉にも造影病変をみとめた．ステロイド薬により臨床症状と画像所見は急速に改善したが減量後に再燃した．右後頭葉の生検で血管周囲にT細胞を主とする炎症性細胞浸潤をみとめchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids（CLIPPERS）と診断した．ステロイド薬増量と維持によりこれらの病変は消失し寛解した．MRIで高度脳幹浮腫を呈する疾患としてCLIPPERSを考慮する必要がある． |
| Author | 山根, 一志 中嶋, 秀人 木村, 文治 大西, 宏之 花房, 俊昭 谷, 裕基 |
| Author_xml | – sequence: 1 fullname: 山根, 一志 organization: 大阪医科大学内科I・神経内科 – sequence: 1 fullname: 花房, 俊昭 organization: 大阪医科大学内科I・神経内科 – sequence: 1 fullname: 木村, 文治 organization: 大阪医科大学内科I・神経内科 – sequence: 1 fullname: 中嶋, 秀人 organization: 大阪医科大学内科I・神経内科 – sequence: 1 fullname: 谷, 裕基 organization: 大阪医科大学内科I・神経内科 – sequence: 1 fullname: 大西, 宏之 organization: 大阪医科大学脳神経外科 |
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| References | 6) Taieb G, Duflos C, Renard D, et al. Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 2012;69:847-855. 1) Pittock SJ, Debruyne J, Krecke KN, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 2010;133:2626-2634. 3) Tohge R, Nagao M, Yagishita A, et al. A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in East Asia. Intern Med 2012;51:1115-1119. 8) Ortega MR, Usmani N, Parra-Herran C, et al. CLIPPERS complicating multiple sclerosis causing concerns of CNS lymphoma. Neurology 2012;79:715-716. 4) Sempere AP, Mola S, Martin-Medina P, et al. Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up. J Neuroimaging 2013;23:254-255. 7) Buttmann M, Metz I, Brecht I, et al. Atypical chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS), primary angiitis of the CNS mimicking CLIPPERS or overlap syndrome? A case report. J Neurol Sci 2013;324:183-186. 2) Kastrup O, van de Nes J, Gasser T, et al. Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study. J Neurol 2011;258:2140-2146. 5) Simon NG, Parratt JD, Barnett MH, et al. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurol Neurosurg Psychiatry 2012;83:15-22. 10) De Graaff HJ, Wattjes MP, Rozemuller-Kwakkel AJ, et al. Fatal B-cell lymphoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. JAMA Neurol 2013;70:915-918. 9) Limousin N, Praline J, Motica O, et al. Brain biopsy is required in steroid-resistant patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurooncol 2012;107:223-224. |
| References_xml | – reference: 5) Simon NG, Parratt JD, Barnett MH, et al. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurol Neurosurg Psychiatry 2012;83:15-22. – reference: 4) Sempere AP, Mola S, Martin-Medina P, et al. Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up. J Neuroimaging 2013;23:254-255. – reference: 2) Kastrup O, van de Nes J, Gasser T, et al. Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study. J Neurol 2011;258:2140-2146. – reference: 3) Tohge R, Nagao M, Yagishita A, et al. A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in East Asia. Intern Med 2012;51:1115-1119. – reference: 9) Limousin N, Praline J, Motica O, et al. Brain biopsy is required in steroid-resistant patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurooncol 2012;107:223-224. – reference: 8) Ortega MR, Usmani N, Parra-Herran C, et al. CLIPPERS complicating multiple sclerosis causing concerns of CNS lymphoma. Neurology 2012;79:715-716. – reference: 7) Buttmann M, Metz I, Brecht I, et al. Atypical chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS), primary angiitis of the CNS mimicking CLIPPERS or overlap syndrome? A case report. J Neurol Sci 2013;324:183-186. – reference: 6) Taieb G, Duflos C, Renard D, et al. Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 2012;69:847-855. – reference: 10) De Graaff HJ, Wattjes MP, Rozemuller-Kwakkel AJ, et al. Fatal B-cell lymphoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. JAMA Neurol 2013;70:915-918. – reference: 1) Pittock SJ, Debruyne J, Krecke KN, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 2010;133:2626-2634. |
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| SubjectTerms | CLIPPERS（chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids） MRI 橋浮腫 脳幹 血管性浮腫 |
| Title | 高度の脳幹浮腫を呈したchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids（CLIPPERS）の1例 |
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