メチルプレドニゾロンパルス療法及び持続的血液濾過透析が有効であった急性出血性白質脳炎（Hurst脳炎）の1例

症例は37歳男性．20歳代からクローン病の治療中であった．腎機能悪化のため入院した．入院後，急に左片麻痺が出現し，全身性痙攣，昏睡状態に至った．頭部MRIで両側大脳白質に多発性異常信号域，病変内部の多発性微小出血を認めた．脳脊髄液検査は，細胞11,136/mm3（多形核球98％），蛋白311 ‍mg/dl，MBP 2,000 pg/ml以上．胸部X線写真で両肺野に浸潤影・多発結節影を認め，静脈血培養で表皮ブドウ球菌が検出された．抗菌薬およびメチルプレドニゾロンパルス療法・持続的血液濾過透析を継続し，徐々に改善した．急性出血性白質脳炎は激症型急性散在性脳脊髄炎で，本例のような著明な改善を認めた例...

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Published in臨床神経学 p. cn-002133
Main Author 梅原 藤雄
Format Journal Article
LanguageJapanese
Published 日本神経学会 2025
Subjects
Hurst脳炎
急性出血性白質脳炎
持続的血液濾過透析
表皮ブドウ球菌性敗血症
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ISSN0009-918X
1882-0654
DOI10.5692/clinicalneurol.cn-002133

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Abstract 症例は37歳男性．20歳代からクローン病の治療中であった．腎機能悪化のため入院した．入院後，急に左片麻痺が出現し，全身性痙攣，昏睡状態に至った．頭部MRIで両側大脳白質に多発性異常信号域，病変内部の多発性微小出血を認めた．脳脊髄液検査は，細胞11,136/mm3（多形核球98％），蛋白311 ‍mg/dl，MBP 2,000 pg/ml以上．胸部X線写真で両肺野に浸潤影・多発結節影を認め，静脈血培養で表皮ブドウ球菌が検出された．抗菌薬およびメチルプレドニゾロンパルス療法・持続的血液濾過透析を継続し，徐々に改善した．急性出血性白質脳炎は激症型急性散在性脳脊髄炎で，本例のような著明な改善を認めた例は稀である．
AbstractList 症例は37歳男性．20歳代からクローン病の治療中であった．腎機能悪化のため入院した．入院後，急に左片麻痺が出現し，全身性痙攣，昏睡状態に至った．頭部MRIで両側大脳白質に多発性異常信号域，病変内部の多発性微小出血を認めた．脳脊髄液検査は，細胞11,136/mm3（多形核球98％），蛋白311 ‍mg/dl，MBP 2,000 pg/ml以上．胸部X線写真で両肺野に浸潤影・多発結節影を認め，静脈血培養で表皮ブドウ球菌が検出された．抗菌薬およびメチルプレドニゾロンパルス療法・持続的血液濾過透析を継続し，徐々に改善した．急性出血性白質脳炎は激症型急性散在性脳脊髄炎で，本例のような著明な改善を認めた例は稀である．
Author 梅原 藤雄
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References 7) Dos Santos DT, Borelli WV, Pinto CB, et al. Acute hemorrhagic leukoencephalitis associated with COVID-19. Arq Neuropsiquiatr 2022;80:448-449.
19) Alemdar M, Selekler HM, Iseri P, et al. The importance of EEG ‍and variability of MRI findings in acute hemorrhagic leukoencephalitis. Eur J Neurol 2006;13:e1-e3.
3) Hofer M, Weber A, Haffner K, et al. Acute hemorrhagic leukoencephalitis (Hurst’s disease) linked to Epstein-Barr virus infection. Acta Neuropathol 2005;109:226-230.
10) Tanser SJ, Walker MB, Hilton DA. Acute haemorrhagic leucoencephalitis complicating sepsis. Anaesth Intensive Care 2001;29:54-57.
17) Skarsta L, Nicoletti T, Frick K, et al. Acute haemorrhagic leucoencephalitis as clinical manifestation of MOG antibody-associated disease. J Neurol Neurosurg Psychiatry 2023;94:583-585.
1) Hurst EW. Studies on Pseudorabies (Infectious Bulbar Paralysis, Mad Itch): I. Histology of the Disease, with a Note on the Symptomatology. J Exp Med 1933;58:415-433.
4) Mondia MWL, Reyes NGD, Espiritu AI, et al. Acute hemorrhagic leukoencephalitis of Weston Hurst secondary to herpes encephalitis presenting as status epilepticus: a case report and review of literature. J Clin Neurosci 2019;67:265-270.
2) Grzonka P, Scholz MC, De Marchis GM, et al. Acute Hemorrhagic Leukoencephalitis: a case and systematic review of the literature. Front Neurol 2020;11:899.
22) Runzhong H, Yingjin Z, Jianlan W, et al. Favorable outcome with early and repeated continuous renal replacement therapy in acute necrotizing encephalopathy. Indian J Pediatr 2022;89:412.
11) Duggal N, Ahmed I, Duggal N. Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. J Vasc Interv Neurol 2014;7:19-22.
21) Zhao JL, Wang ZY, Li SJ, et al. The efficacy of haemoperfusion combined with continuous venovenous haemodiafiltration in the treatment of severe viral encephalitis in children. Ital J Pediatr 2023;49:21.
20) Garbero E, Livigni S, Ferrari F, et al. High dose coupled plasma filtration and adsorption in septic shock patients. Results of the COMPACT-2: a multicentre, adaptive, randomised clinical trial. Intensive Care Med 2021;47:1303-1311.
6) Alqahtani A, Alaklabi A, Kristjansson S, et al. Acute necrotic hemorrhagic leukoencephalitis related to COVID-19: a report of 2 cases. Radiol Case Rep 2021;16:2393-2398.
12) Gibbs WN, Kreidie MA, Kim RC, et al. Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. J Comput Assist Tomogr 2005;29:689-693.
14) dos Santos MP, Martin J, Woulfe J, et al. Autopsy-proven acute Hemorrhagic Leukoencephalitis in an elderly patient. Can J Neurol Sci 2014;41:99-102.
9) Ryan LJ, Bowman R, Zantek ND, et al. Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. Transfusion 2007;47:981-986.
13) Kao HW, Alexandru D, Kim R, et al. Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis. J Clin Neurosci 2012;19:1740-1741.
16) Tan SY, Pillai P, Wee JS, et al. Anti-myelin oligodendrocyte glycoprotein antibodies and acute Hemorrhagic Encephalo­myelitis. Can J Neurol Sci 2024:1-5.
5) Fugate JE, Lam EM, Rabinstein AA, et al. Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. Arch Neurol 2010;67:756-758.
15) Graham DI, Behan PO, More IA. Brain damage complicating septic shock: acute haemorrhagic leucoencephalitis as a complication of the generalised Shwartzman reaction. J Neurol Neurosurg Psychiatry 1979;42:19-28.
8) Sivasubramanian D, Mohamed Kalifa MRH. Acute Hemorrhagic leukoencephalitis in a patient with hepatitis B. Cureus 2024;16:e67587.
18) Bang SJ, Kim S, Seok HY. Acute hemorrhagic leukoencephalitis as a new phenotype of myelin oligodendrocyte glycoprotein antibody-associated disease. Neurol Sci 2023;44:3741-3743.
References_xml – reference: 20) Garbero E, Livigni S, Ferrari F, et al. High dose coupled plasma filtration and adsorption in septic shock patients. Results of the COMPACT-2: a multicentre, adaptive, randomised clinical trial. Intensive Care Med 2021;47:1303-1311.
– reference: 10) Tanser SJ, Walker MB, Hilton DA. Acute haemorrhagic leucoencephalitis complicating sepsis. Anaesth Intensive Care 2001;29:54-57.
– reference: 12) Gibbs WN, Kreidie MA, Kim RC, et al. Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. J Comput Assist Tomogr 2005;29:689-693.
– reference: 17) Skarsta L, Nicoletti T, Frick K, et al. Acute haemorrhagic leucoencephalitis as clinical manifestation of MOG antibody-associated disease. J Neurol Neurosurg Psychiatry 2023;94:583-585.
– reference: 6) Alqahtani A, Alaklabi A, Kristjansson S, et al. Acute necrotic hemorrhagic leukoencephalitis related to COVID-19: a report of 2 cases. Radiol Case Rep 2021;16:2393-2398.
– reference: 18) Bang SJ, Kim S, Seok HY. Acute hemorrhagic leukoencephalitis as a new phenotype of myelin oligodendrocyte glycoprotein antibody-associated disease. Neurol Sci 2023;44:3741-3743.
– reference: 4) Mondia MWL, Reyes NGD, Espiritu AI, et al. Acute hemorrhagic leukoencephalitis of Weston Hurst secondary to herpes encephalitis presenting as status epilepticus: a case report and review of literature. J Clin Neurosci 2019;67:265-270.
– reference: 3) Hofer M, Weber A, Haffner K, et al. Acute hemorrhagic leukoencephalitis (Hurst’s disease) linked to Epstein-Barr virus infection. Acta Neuropathol 2005;109:226-230.
– reference: 13) Kao HW, Alexandru D, Kim R, et al. Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis. J Clin Neurosci 2012;19:1740-1741.
– reference: 14) dos Santos MP, Martin J, Woulfe J, et al. Autopsy-proven acute Hemorrhagic Leukoencephalitis in an elderly patient. Can J Neurol Sci 2014;41:99-102.
– reference: 19) Alemdar M, Selekler HM, Iseri P, et al. The importance of EEG ‍and variability of MRI findings in acute hemorrhagic leukoencephalitis. Eur J Neurol 2006;13:e1-e3.
– reference: 16) Tan SY, Pillai P, Wee JS, et al. Anti-myelin oligodendrocyte glycoprotein antibodies and acute Hemorrhagic Encephalo­myelitis. Can J Neurol Sci 2024:1-5.
– reference: 7) Dos Santos DT, Borelli WV, Pinto CB, et al. Acute hemorrhagic leukoencephalitis associated with COVID-19. Arq Neuropsiquiatr 2022;80:448-449.
– reference: 5) Fugate JE, Lam EM, Rabinstein AA, et al. Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. Arch Neurol 2010;67:756-758.
– reference: 21) Zhao JL, Wang ZY, Li SJ, et al. The efficacy of haemoperfusion combined with continuous venovenous haemodiafiltration in the treatment of severe viral encephalitis in children. Ital J Pediatr 2023;49:21.
– reference: 1) Hurst EW. Studies on Pseudorabies (Infectious Bulbar Paralysis, Mad Itch): I. Histology of the Disease, with a Note on the Symptomatology. J Exp Med 1933;58:415-433.
– reference: 8) Sivasubramanian D, Mohamed Kalifa MRH. Acute Hemorrhagic leukoencephalitis in a patient with hepatitis B. Cureus 2024;16:e67587.
– reference: 11) Duggal N, Ahmed I, Duggal N. Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. J Vasc Interv Neurol 2014;7:19-22.
– reference: 15) Graham DI, Behan PO, More IA. Brain damage complicating septic shock: acute haemorrhagic leucoencephalitis as a complication of the generalised Shwartzman reaction. J Neurol Neurosurg Psychiatry 1979;42:19-28.
– reference: 2) Grzonka P, Scholz MC, De Marchis GM, et al. Acute Hemorrhagic Leukoencephalitis: a case and systematic review of the literature. Front Neurol 2020;11:899.
– reference: 22) Runzhong H, Yingjin Z, Jianlan W, et al. Favorable outcome with early and repeated continuous renal replacement therapy in acute necrotizing encephalopathy. Indian J Pediatr 2022;89:412.
– reference: 9) Ryan LJ, Bowman R, Zantek ND, et al. Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. Transfusion 2007;47:981-986.
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Snippet 症例は37歳男性．20歳代からクローン病の治療中であった．腎機能悪化のため入院した．入院後，急に左片麻痺が出現し，全身性痙攣，昏睡状態に至った．頭部MRIで両側大脳白質に多発性異常信号域，病変内部の多発性微小出血を認めた．脳脊髄液検査は，細胞11,136/mm3（多形核球98％），蛋白311 ‍mg/dl，MBP...
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SourceType Publisher
StartPage cn-002133
SubjectTerms Hurst脳炎
急性出血性白質脳炎
持続的血液濾過透析
表皮ブドウ球菌性敗血症
Title メチルプレドニゾロンパルス療法及び持続的血液濾過透析が有効であった急性出血性白質脳炎（Hurst脳炎）の1例
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