両側中小脳脚に主病変をもち，JC virus granule cell neuronopathyの合併が示唆された進行性多巣性白質脳症の1例．診断確定上再三の髄液検査の重要性

症例は65歳の女性である．再発性の小リンパ球性リンパ腫に対し，半年間rituximab等が投与され，めまいと小脳性運動失調が発症した．頭部MRIで両側中小脳脚病変を認め，進行性多巣性白質脳症（progressive multifocal leukoencephalopathy; PML）を疑い，髄液中JC virus（JCV）-DNA PCR検査を数回施行し，4回目に初めてJCV-DNAが検出され，診断が確定した．小脳萎縮を認め，granule cell neuronopathyの合併が示唆された．Mirtazapineとmefloquineの併用治療により長期生存が得られた．PMLでは両側中...

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Published in臨床神経学 Vol. 56; no. 7; pp. 481 - 485
Main Authors 中道, 一生, 勝野, 雅央, 長谷川, 康博, 伊藤, 大輔, 髙橋, 昭, 安井, 敬三
Format Journal Article
LanguageJapanese
Published 日本神経学会 2016
Subjects
granule cell neuronopathy
JCウイルス
中小脳脚
小脳萎縮
進行性多巣性白質脳症
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ISSN0009-918X
1882-0654
DOI10.5692/clinicalneurol.cn-000873

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Abstract 症例は65歳の女性である．再発性の小リンパ球性リンパ腫に対し，半年間rituximab等が投与され，めまいと小脳性運動失調が発症した．頭部MRIで両側中小脳脚病変を認め，進行性多巣性白質脳症（progressive multifocal leukoencephalopathy; PML）を疑い，髄液中JC virus（JCV）-DNA PCR検査を数回施行し，4回目に初めてJCV-DNAが検出され，診断が確定した．小脳萎縮を認め，granule cell neuronopathyの合併が示唆された．Mirtazapineとmefloquineの併用治療により長期生存が得られた．PMLでは両側中小脳脚の病変で発症する例がある．また，病初期には髄液検査でJCV-DNAが陰性のことがあり，繰り返し検査する必要がある．
AbstractList 症例は65歳の女性である．再発性の小リンパ球性リンパ腫に対し，半年間rituximab等が投与され，めまいと小脳性運動失調が発症した．頭部MRIで両側中小脳脚病変を認め，進行性多巣性白質脳症（progressive multifocal leukoencephalopathy; PML）を疑い，髄液中JC virus（JCV）-DNA PCR検査を数回施行し，4回目に初めてJCV-DNAが検出され，診断が確定した．小脳萎縮を認め，granule cell neuronopathyの合併が示唆された．Mirtazapineとmefloquineの併用治療により長期生存が得られた．PMLでは両側中小脳脚の病変で発症する例がある．また，病初期には髄液検査でJCV-DNAが陰性のことがあり，繰り返し検査する必要がある．
Author 勝野, 雅央
中道, 一生
安井, 敬三
長谷川, 康博
伊藤, 大輔
髙橋, 昭
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18) Paues J, Vrethem M. Fatal progressive multifocal leukoencephalopathy in a patient with non-Hodgkin lymphoma treated with rituximab. J Clin Virol 2010;48:291-293.
24) Wijburg MT, van Oosten BW, Murk JL, et al. Heterogeneous imaging characteristics of JC virus granule cell neuronopathy (GCN): a case series and review of the literature. J Neurol 2015;262:65-73.
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19) Vaklavas C, Sotelo-Rafiq EP, Lovy J, et al. Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression.Virol J 2010;7:256.
3) Keene DL, Legare C, Taylor E, et al. Monoclonal antibodies and progressive multifocal leukoencephalopathy. Can J Neurol Sci 2011;38:565-571.
8) 髙橋　昭，飯田光男，飯島宗一ら．Progressive multifocal leuko-encephalopathyの本邦第1例（会）．臨床神経 1962;2:215-216.
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16) Antinori A, Cingolani A, Lorenzini P, et al. Clinical epidemiology and survival of progressive multifocal leukoencephalopathy in the era of highly active antiretroviral therapy: Data from the Italian Registry Investigative Neuro AIDS (IRINA). J Neurovirol 2003;9:47-53.
6) Brickelmaier M, Lugovskoy A, Kartikeyan R, et al. Identification and characterization of mefloquine efficacy against JC virus in vitro. Antimicrob Agents Chemother 2009;53:1840-1849.
11) Uchino A, Sawada A, Takase Y, et al. Symmetrical lesions of the middle cerebellar peduncle: MR imaging and differential diagnosis. Magn Reson Med Sci 2004;3:133-140.
9) Nakamichi K, Mizusawa H, Yamada M, et al. Characteristics of progressive multifocal leukoencephalopathy clarified through internet-assisted laboratory surveillance in Japan. BMC Neurol 2012;12:121.
20) Di Pauli F, Berger T, Walder A, et al. Progressive multifocal leukoencephalopathy complicating untreated chronic lymphatic leukemia: case report and review of the literature. J Clin Virol 2014;60:424-427.
4) Irie T, Kasai M, Abe N, et al. Cerebellar form of progressive multifocal leukoencephalopathy in a patient with chronic renal failure. Intern Med 1992;31:218-223.
21) Kuhle J, Gosert R, Bühler R, et al. Management and outcome of CSF-JC virus PCR-negative PML in a natalizumab-treated patient with MS. Neurology 2011;77:2010-2016.
22) Carson KR, Evens AM, Richey EA, et al. Progressive multifocal leukoencephalopathy after rituximab therapy in HIV-negative patients: a report of 57 cases from the research on adverse drug events and reports project. Blood 2009;113:4834-4840.
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1) Nakamichi K, Kurane I, Saijo M. Evaluation of a quantitative real-time PCR assay for the detection of JC polyomavirus DNA in cerebrospinal fluid without nucleic acid extraction. Jpn J Infect Dis 2011;64:211-216.
7) Åström KE, Mancall EL, Richardson EP. Progressive multifocal leuko-encephalopathy. A hitherto unrecognized complication of chronic lymphatic leukaemia and Hodgkin’s disease. Brain 1958;81:93-111.
References_xml – reference: 5) Verma S, Cikurel K, Koralnik IJ, et al. Mirtazapine in progressive multifocal leukoencephalopathy associated with polycythemia vera. J Infect Dis 2007;196:709-711.
– reference: 23) Du Pasquier RA, Corey S, Margolin DH, et al. Productive infection of cerebellar granule cell neurons by JC virus in an HIV+ individual. Neurology 2003;61:775-782.
– reference: 15) Svensson PA, Larsson EM. Infratentorial progressive multifocal leucoencephalopathy (PML) in a patient with SLE (2009:4b). Eur Radiol 2008;18:1526-1528.
– reference: 22) Carson KR, Evens AM, Richey EA, et al. Progressive multifocal leukoencephalopathy after rituximab therapy in HIV-negative patients: a report of 57 cases from the research on adverse drug events and reports project. Blood 2009;113:4834-4840.
– reference: 2) 厚生労働科学研究費補助金 難治性疾患等克服研究事業（難治性疾患克服研究事業）プリオン病及び遅発性ウイルス感染症に関する調査研究班（編）．進行性多巣性白質脳症（Progressive Multifocal Leukoencephalopathy: PML）診療ガイドライン 2013.
– reference: 6) Brickelmaier M, Lugovskoy A, Kartikeyan R, et al. Identification and characterization of mefloquine efficacy against JC virus in vitro. Antimicrob Agents Chemother 2009;53:1840-1849.
– reference: 14) Völker HU, Kraft K, Arnold E, et al. Progressive multifocal leukoencephalopathy developing in advanced pulmonal sarcoidosis. Clin Neurol Neurosurg 2007;109:624-630.
– reference: 3) Keene DL, Legare C, Taylor E, et al. Monoclonal antibodies and progressive multifocal leukoencephalopathy. Can J Neurol Sci 2011;38:565-571.
– reference: 16) Antinori A, Cingolani A, Lorenzini P, et al. Clinical epidemiology and survival of progressive multifocal leukoencephalopathy in the era of highly active antiretroviral therapy: Data from the Italian Registry Investigative Neuro AIDS (IRINA). J Neurovirol 2003;9:47-53.
– reference: 17) Hirayama M, Nosaki Y, Matsui K, et al. Efficacy of mefloquine to progressive multifocal leukoencephalopathy initially presented with parkinsonism. Clin Neurol Neurosurg 2012;114:728-731.
– reference: 24) Wijburg MT, van Oosten BW, Murk JL, et al. Heterogeneous imaging characteristics of JC virus granule cell neuronopathy (GCN): a case series and review of the literature. J Neurol 2015;262:65-73.
– reference: 13) Bag AK, Curé JK, Chapman PR, et al. JC virus infection of the brain. AJNR Am J Neuroradiol 2010;31:1564-1576.
– reference: 20) Di Pauli F, Berger T, Walder A, et al. Progressive multifocal leukoencephalopathy complicating untreated chronic lymphatic leukemia: case report and review of the literature. J Clin Virol 2014;60:424-427.
– reference: 9) Nakamichi K, Mizusawa H, Yamada M, et al. Characteristics of progressive multifocal leukoencephalopathy clarified through internet-assisted laboratory surveillance in Japan. BMC Neurol 2012;12:121.
– reference: 8) 髙橋　昭，飯田光男，飯島宗一ら．Progressive multifocal leuko-encephalopathyの本邦第1例（会）．臨床神経 1962;2:215-216.
– reference: 18) Paues J, Vrethem M. Fatal progressive multifocal leukoencephalopathy in a patient with non-Hodgkin lymphoma treated with rituximab. J Clin Virol 2010;48:291-293.
– reference: 7) Åström KE, Mancall EL, Richardson EP. Progressive multifocal leuko-encephalopathy. A hitherto unrecognized complication of chronic lymphatic leukaemia and Hodgkin’s disease. Brain 1958;81:93-111.
– reference: 4) Irie T, Kasai M, Abe N, et al. Cerebellar form of progressive multifocal leukoencephalopathy in a patient with chronic renal failure. Intern Med 1992;31:218-223.
– reference: 11) Uchino A, Sawada A, Takase Y, et al. Symmetrical lesions of the middle cerebellar peduncle: MR imaging and differential diagnosis. Magn Reson Med Sci 2004;3:133-140.
– reference: 10) Okamoto K, Tokiguchi S, Furusawa T, et al. MR features of diseases involving bilateral middle cerebellar peduncles. AJNR Am J Neuroradiol 2003;24:1946-1954.
– reference: 19) Vaklavas C, Sotelo-Rafiq EP, Lovy J, et al. Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression.Virol J 2010;7:256.
– reference: 21) Kuhle J, Gosert R, Bühler R, et al. Management and outcome of CSF-JC virus PCR-negative PML in a natalizumab-treated patient with MS. Neurology 2011;77:2010-2016.
– reference: 1) Nakamichi K, Kurane I, Saijo M. Evaluation of a quantitative real-time PCR assay for the detection of JC polyomavirus DNA in cerebrospinal fluid without nucleic acid extraction. Jpn J Infect Dis 2011;64:211-216.
– reference: 12) Apartis E, Blancher A, Meissner WG, et al. FXTAS: new insights and the need for revised diagnostic criteria. Neurology 2012;79:1898-1907.
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Snippet 症例は65歳の女性である．再発性の小リンパ球性リンパ腫に対し，半年間rituximab等が投与され，めまいと小脳性運動失調が発症した．頭部MRIで両側中小脳脚病変を認め，進...
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StartPage 481
SubjectTerms granule cell neuronopathy
JCウイルス
中小脳脚
小脳萎縮
進行性多巣性白質脳症
Title 両側中小脳脚に主病変をもち，JC virus granule cell neuronopathyの合併が示唆された進行性多巣性白質脳症の1例．診断確定上再三の髄液検査の重要性
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