今日の臨床サポート

急性散在性脳脊髄炎

著者: 水野昌宣 岩手医科大学 内科学講座 神経内科・老年科

監修: 高橋裕秀 昭和大学藤が丘病院 脳神経内科

著者校正済:2021/12/01
現在監修レビュー中
参考ガイドライン:
  1. 日本神経学会:多発性硬化症・視神経脊髄炎診療ガイドライン2017
  1. 厚生労働省:重篤副作用疾患別対応マニュアル 急性散在性脳脊髄炎(平成23年3月)
患者向け説明資料

概要・推奨   

  1. ADEMの急性期にはメチルプレドニゾロンによるステロイドパルス療法が推奨される(推奨度1)
  1. ADEMの急性期にはステロイドパルス療法を用いるが、反応が不良な症例に対し単純血漿交換や大量静脈ガンマグロブリン血症、シクロホスファミド静注療法が有効な可能性もある(推奨度1)
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
水野昌宣 : 未申告[2021年]
監修:高橋裕秀 : 特に申告事項無し[2021年]

改訂のポイント:
  1. COVID-19感染後のADEMの情報を加筆した。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 急性散在性脳脊髄炎(acute disseminated encephalomyelitis; 以下ADEM)は、急性発症で単相性の経過をたどり、中枢神経に炎症機序による脱髄性病変が散在する疾患である。感染後、予防接種後、数日~8週間後(多くは1~3週間後)に発症する。
  1. すべての年齢で発症し得るが、主に3歳から9歳の小児に多くみられる。わが国の報告では、15歳未満の小児は、罹患率が年間10万人あたり0.64人、男女比は2.3:1、平均年齢5.7歳[1]。また、小児脱髄性疾患の全国疫学調査では、罹患率は0.4人/10万人/年と、平均発症年齢5.5歳とされ、先行感染あり62%、ワクチン後発症が18%とある[2]。サンティアゴの報告では、罹患率が年間10万人あたり0.4人、ドイツで0.07人と地域差がみられた[3]
  1. ワクチン接種後のADEMに関しては、インフルエンザワクチン、B型肝炎ワクチン、日本脳炎ワクチン、天然痘、黄熱病、腸チフス、結核、狂犬病、ポリオなどが知られる。発症率に関しては100万回のワクチン接種に対して1~3.5人とされる[4]
  1. 2021年8月の時点でCOVID-19感染後のADEMの報告が散見されてきている。基本的な治療は通常のADEMと変わらないものと思われるが[5][6]、急性出血性壊死性脳炎、急性出血性白質脳炎、致死性壊死性脳炎など様々な重篤な中枢神経疾患の症例報告もなされており、ADEMの重症型も含まれると思われる。また、COVID-19による肺炎などで全身状態が悪い症例も含まれており、予後に関してはデータの蓄積が必要と思われる。
 
脱髄の説明

髄鞘が主に障害を受けることを脱髄という。
a:中枢神経のしくみ
b:脱髄をおこした中枢神経

出典

問診・診察のポイント  
  1. 一般に用いられる診断基準や特異的なマーカーが存在しないため、病歴聴取が非常に大切な疾患である。特に感染性(特にウイルス性)脳炎、多発性硬化症/視神経脊髄炎との鑑別は困難を要することもある。

これより先の閲覧には個人契約のトライアルまたはお申込みが必要です。

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文献 

著者: Hiroyuki Torisu, Ryutaro Kira, Yoshito Ishizaki, Masafumi Sanefuji, Yui Yamaguchi, Sawa Yasumoto, Yoshihiko Murakami, Masayuki Shimono, Shinichiro Nagamitsu, Mayumi Masuzaki, Masano Amamoto, Rikako Kondo, Tomohiko Uozumi, Miyuki Aibe, Kenjiro Gondo, Toshio Hanai, Sinichi Hirose, Toyojiro Matsuishi, Akira Shirahata, Akihisa Mitsudome, Toshiro Hara
雑誌名: Brain Dev. 2010 Jun;32(6):454-62. doi: 10.1016/j.braindev.2009.10.006. Epub 2009 Nov 25.
Abstract/Text Acute disseminated encephalomyelitis (ADEM) has recently been studied in several countries owing to the development and wide spread use of imaging technology, but few epidemiological studies of childhood ADEM have been undertaken in Asian countries. To perform a comprehensive survey of ADEM and related diseases in Japanese children, we conducted a multicenter, population-based study on childhood ADEM, multiple sclerosis, and acute isolated transverse myelitis in Fukuoka Prefecture, Japan. We identified 26 children with ADEM, 8 with multiple sclerosis, and 4 with acute transverse myelitis during 5 years between September 1998 and August 2003. The incidence of childhood ADEM under the age of 15 years was 0.64 per 100,000 person-years, mean age at onset was 5.7 years, and male-female ratio was 2.3:1. The prevalence of childhood multiple sclerosis was 1.3 per 100,000 persons. The mean age at onset of multiple sclerosis, 9.3 years, was significantly higher than that of ADEM. Nineteen (73%) and four (15%) patients with ADEM experienced antecedent infectious illnesses and vaccinations, respectively, within 1 month before the onset. Clinical and radiological findings of ADEM revealed that the frequency of seizures, mean white blood cell counts in cerebrospinal fluid, and the frequency of subcortical lesions in Fukuoka study, seemed to be higher than those in previous non-Asian studies. These findings suggest that there are ethnic or geographical differences in the incidence and clinical features of ADEM, and that there might be potent genetic or environmental risk factors for ADEM distinct from those for multiple sclerosis.

Copyright (c) 2009 Elsevier B.V. All rights reserved.
PMID 19942388  Brain Dev. 2010 Jun;32(6):454-62. doi: 10.1016/j.braind・・・
著者: Y Yamaguchi, H Torisu, R Kira, Y Ishizaki, Y Sakai, M Sanefuji, T Ichiyama, A Oka, T Kishi, S Kimura, M Kubota, J Takanashi, Y Takahashi, H Tamai, J Natsume, S Hamano, S Hirabayashi, Y Maegaki, M Mizuguchi, K Minagawa, H Yoshikawa, J Kira, S Kusunoki, T Hara
雑誌名: Neurology. 2016 Nov 8;87(19):2006-2015. doi: 10.1212/WNL.0000000000003318. Epub 2016 Oct 14.
Abstract/Text OBJECTIVE: To investigate the clinical and epidemiologic features of pediatric acquired demyelinating syndromes (ADS) of the CNS in Japan.
METHODS: We conducted a nationwide survey and collected clinical data on children with ADS aged 15 years or younger, who visited hospitals between 2005 and 2007.
RESULTS: Among 977 hospitals enrolled, 723 (74.0%) responded to our inquiries and reported a total of 439 patients as follows: 244 with acute disseminated encephalomyelitis (ADEM), 117 with multiple sclerosis (MS), 14 with neuromyelitis optica (NMO), and 64 with other ADS. We collected and analyzed detailed data from 204 cases, including those with ADEM (66), MS (58), and NMO (10). We observed the following: (1) the estimated annual incidence rate of pediatric ADEM in Japan was 0.40 per 100,000 children (95% confidence interval [CI], 0.34-0.46), with the lowest prevalence in the north; (2) the estimated prevalence rate of MS was 0.69 per 100,000 children (95% CI, 0.58-0.80), with the lowest prevalence in the south; (3) NMO in Japan was rare, with an estimated prevalence of 0.06 per 100,000 children (95% CI, 0.04-0.08); and (4) the sex ratio and mean age at onset varied by ADS type, and (5) male/female ratios correlated with ages at onset in each ADS group.
CONCLUSIONS: Our results clarify the characteristic clinical features of pediatric ADS in the Japanese population.

© 2016 American Academy of Neurology.
PMID 27742816  Neurology. 2016 Nov 8;87(19):2006-2015. doi: 10.1212/WN・・・
著者: Til Menge, Bernhard Hemmer, Stefan Nessler, Heinz Wiendl, Oliver Neuhaus, Hans-Peter Hartung, Bernd C Kieseier, Olaf Stüve
雑誌名: Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/archneur.62.11.1673.
Abstract/Text Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system that typically follows a febrile infection or a vaccination. Children are predominantly affected. A plethora of viral and bacterial pathogens and a number of vaccinations have been associated with ADEM. Experimental animal studies indicate that both primary and secondary autoimmune responses contribute to central nervous system inflammation and subsequent demyelination. The clinical diagnosis of ADEM is strongly suggested by a close temporal relationship between an infectious incident or an immunization and the onset of leukoencephalopathic neurological symptoms. Paraclinical tests may support the diagnosis. Particularly helpful are acute signs of newly developed extensive, multifocal, subcortical white matter abnormalities on magnetic resonance images of the brain. The cerebrospinal fluid may disclose a mild lymphocytic pleocytosis and elevated albumin levels. Oligoclonal bands are not always present in ADEM and, if so, may be transient. The major differential diagnosis of ADEM is multiple sclerosis. Treatment options for ADEM consist of anti-inflammatory and immunosuppressive agents. In general, the disease is self-limiting and the prognostic outcome favorable. In the absence of widely accepted clinical or paraclinical diagnostic guidelines, a number of recently conducted observational case series have substantially broadened our understanding about the clinical phenotype, diagnosis, and prognosis of ADEM.

PMID 16286539  Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/arch・・・
著者: Timothy Parsons, Sarah Banks, Chay Bae, Joel Gelber, Hussein Alahmadi, Matthew Tichauer
雑誌名: J Neurol. 2020 Oct;267(10):2799-2802. doi: 10.1007/s00415-020-09951-9. Epub 2020 May 30.
Abstract/Text A 51-year-old woman with COVID-19 infection developed coma and an impaired oculocephalic response to one side. MRI of the brain demonstrated acute multifocal demyelinating lesions, and CSF testing did not identify a direct cerebral infection. High-dose steroids followed by a course of IVIG was administered, and the patient regained consciousness over the course of several weeks. As more patients reach the weeks after initial infection with COVID-19, acute disseminated encephalomyelitis should be considered a potentially treatable cause of profound encephalopathy or multifocal neurological deficits.

PMID 32474657  J Neurol. 2020 Oct;267(10):2799-2802. doi: 10.1007/s004・・・
著者: Adélia Maria de Miranda Henriques-Souza, Ana Cláudia Marques Gouveia de Melo, Bianca de Aguiar Coelho Silva Madeiro, Leonardo Furtado Freitas, Pedro Augusto Sampaio Rocha-Filho, Fabrício Guimarães Gonçalves
雑誌名: Neuroradiology. 2021 Jan;63(1):141-145. doi: 10.1007/s00234-020-02571-0. Epub 2020 Oct 1.
Abstract/Text The authors present a case of acute disseminated encephalomyelitis in a COVID-19 pediatric patient with positive SARS-CoV2 markers from a nasopharyngeal swab. A previously healthy 12-year-old-girl presented with a skin rash, headache, and fever. Five days after that, she had an acute, progressive, bilateral, and symmetrical motor weakness. She evolved to respiratory failure. Magnetic resonance imaging (MRI) of the brain and cervical spine showed extensive bilateral and symmetric restricted diffusion involving the subcortical and deep white matter, a focal hyperintense T2/FLAIR lesion in the splenium of the corpus callosum with restricted diffusion, and extensive cervical myelopathy involving both white and gray matter. Follow-up examinations of the brain and spine were performed 30 days after the first MRI examination. The images of the brain demonstrated mild dilatation of the lateral ventricles and widespread widening of the cerebral sulci, complete resolution of the extensive white matter restricted diffusion, and complete resolution of the restricted diffusion in the lesion of the splenium of the corpus callosum, leaving behind a small gliotic focus. The follow-up examination of the spine demonstrated nearly complete resolution of the extensive signal changes in the spinal cord, leaving behind scattered signal changes in keeping with gliosis. She evolved with partial clinical and neurological improvement and was subsequently discharged.

PMID 33001220  Neuroradiology. 2021 Jan;63(1):141-145. doi: 10.1007/s0・・・
著者: Farshid Noorbakhsh, Richard T Johnson, Derek Emery, Christopher Power
雑誌名: Neurol Clin. 2008 Aug;26(3):759-80, ix. doi: 10.1016/j.ncl.2008.03.009.
Abstract/Text Acute disseminated encephalomyelitis (ADEM) is an immune-mediated disorder of the central nervous system (CNS). Disease typically starts with an abrupt onset of neurologic symptoms and signs within days to weeks after a viral infection or immunization. Neuropathological examination of the CNS in ADEM reveals involvement of white matter, with infiltration of monocytoid cells and perivenous demyelination.

PMID 18657725  Neurol Clin. 2008 Aug;26(3):759-80, ix. doi: 10.1016/j.・・・
著者: M Studahl, T Bergström, L Hagberg
雑誌名: Scand J Infect Dis. 1998;30(3):215-20.
Abstract/Text We have prospectively studied 27 adult patients attending the Department of Infectious Diseases, Göteborg, Sweden, between October 1992 and October 1996 with a diagnosis of acute viral encephalitis. In addition to cerebrospinal fluid (CSF) virus isolations and antibody analyses against herpes simplex virus, cytomegalovirus, varicella zoster virus, Epstein-Barr virus (EBV), enterovirus, adenovirus, tick-borne encephalitis virus, and mycoplasma, polymerase chain reaction test (PCR) to 5 viruses from the family of human herpes viridae, and to adenovirus as well as to enterovirus were analysed in CSF. 10 patients had herpes simplex virus type-1 (HSV-1), 1 had varicella zoster virus, 1 had tick-borne encephalitis, and 2 had Influenza A infections. In 13 patients the aetiology remained unclear. Eight patients with HSV-1 encephalitis and clinical symptoms for 2-11 d before admission were PCR-positive, while 2 patients with a < or = 2 d history of disease were negative for HSV-1 DNA on admission. These 2 patients became positive for HSV-1 DNA in CSF samples taken 4 d later in 1 case and 7 d later in the other. In 4 patients with HSV-1 encephalitis, in 1 patient with Influenza A complicated by encephalitis, and in 1 patient with encephalitis of unknown origin EBV DNA was found in CSF samples during the study. The clinical significance of these findings is unclear. The study shows that HSV-1 was the most common etiological agent in patients with viral encephalitis in the Göteborg area. In spite of improved diagnostic procedures, a large proportion of patients with symptoms and laboratory findings compatible with viral encephalitis still have an unclear aetiology.

PMID 9790126  Scand J Infect Dis. 1998;30(3):215-20.
著者: B Sivertsen, P B Christensen
雑誌名: Acta Neurol Scand. 1996 Feb-Mar;93(2-3):156-9.
Abstract/Text Acute encephalitis: etiology, clinical findings and prognosis. We studied 44 patients with acute encephalitis diagnosed in a neurological university clinic during an 11-year period. An etiology was found in 11 cases (25%). In 3 the cause was herpes simplex virus; in 2 morbilli. There were single patients in which the cause was mycoplasma pneumoniae, epidemic parotitis, ornithosis, infectious mononucleosis, influenza B-virus and recent tetanus immunization. No specific etiology was found in 33 (75%). Besides fever the most frequent sign was impairment of consciousness in 39% of cases. Four patients (9%) died. Among the survivors mental and/or focal neurological deficits persisted in 22 (55%). Most frequent was dementia in 6 cases (15% of survivors). Impaired consciousness in the acute phase indicated a worse prognosis (p < 0.005).

PMID 8741136  Acta Neurol Scand. 1996 Feb-Mar;93(2-3):156-9.
著者: Lauren B Krupp, Marc Tardieu, Maria Pia Amato, Brenda Banwell, Tanuja Chitnis, Russell C Dale, Angelo Ghezzi, Rogier Hintzen, Andrew Kornberg, Daniela Pohl, Kevin Rostasy, Silvia Tenembaum, Evangeline Wassmer, International Pediatric Multiple Sclerosis Study Group
雑誌名: Mult Scler. 2013 Sep;19(10):1261-7. doi: 10.1177/1352458513484547. Epub 2013 Apr 9.
Abstract/Text BACKGROUND: There has been tremendous growth in research in pediatric multiple sclerosis (MS) and immune mediated central nervous system demyelinating disorders since operational definitions for these conditions were first proposed in 2007. Further, the International Pediatric Multiple Sclerosis Study Group (IPMSSG), which proposed the criteria, has expanded substantially in membership and in its international scope.
OBJECTIVE: The purpose of this review is to revise the 2007 definitions in order to incorporate advances in delineating the clinical and neuroradiologic features of these disorders.
METHODS: Through a consensus process, in which input was sought from the 150 members of the Study Group, criteria were drafted, revised and finalized. Final approval was sought through a web survey.
RESULTS: Revised criteria are proposed for pediatric acute disseminated encephalomyelitis, pediatric clinically isolated syndrome, pediatric neuromyelitis optica and pediatric MS. These criteria were approved by 93% or more of the 56 Study Group members who responded to the final survey.
CONCLUSIONS: These definitions are proposed for clinical and research purposes. Their utility will depend on the outcomes of their application in prospective research.

PMID 23572237  Mult Scler. 2013 Sep;19(10):1261-7. doi: 10.1177/135245・・・
著者: S Schwarz, A Mohr, M Knauth, B Wildemann, B Storch-Hagenlocher
雑誌名: Neurology. 2001 May 22;56(10):1313-8.
Abstract/Text OBJECTIVES: To describe the clinical, CSF, and radiologic findings and long-term follow-up in a cohort of patients with acute disseminated encephalomyelitis (ADEM), and to determine possible prognostic factors for progression to MS.
METHODS: Forty adults (28 women, mean age 33.5 years) diagnosed with ADEM were analyzed. Clinical symptoms, cranial MRI and CSF findings, and the response to a standardized treatment during the acute phase of the disease were analyzed by chart review. The final diagnosis of ADEM or clinically definite MS was established upon follow-up examination after 8 to 137 months. The patients with ADEM and MS were compared to detect differences between the two groups.
RESULTS: Fifteen patients had a preceding infection (n = 14) or immunization (n = 1). The most frequent clinical signs were motor deficit (80%), followed by sensory deficits, brainstem signs, and ataxia. CSF findings were highly variable; normal results were present in 20% of patients. Oligoclonal bands were positive in 65% of patients. Ninety-five percent of all patients improved during the acute phase of the disease. Upon follow-up, 14 patients had developed clinically definite MS. Of the 26 patients with the final diagnosis of ADEM, two patients had died, nine had minor deficits, three had moderate deficits, and 12 patients had no remaining symptoms. Patients with the final diagnosis of ADEM were older, and more often had a preceding infection, clinical signs of brainstem involvement, a higher CSF albumin fraction, and infratentorial lesions.
CONCLUSIONS: Many patients initially diagnosed with ADEM develop clinically definite MS upon long-term follow-up. The authors found no useful diagnostic criteria for the differentiation of a first episode of MS from monophasic ADEM. The term ADEM may still be employed as a description of a clinical syndrome, but should not be used as a distinct entity until reliable diagnostic criteria have been developed.

PMID 11376180  Neurology. 2001 May 22;56(10):1313-8.
著者: Tomas Olsson
雑誌名: Nat Rev Neurol. 2011 May;7(5):248-9. doi: 10.1038/nrneurol.2011.45. Epub 2011 Apr 12.
Abstract/Text
PMID 21537356  Nat Rev Neurol. 2011 May;7(5):248-9. doi: 10.1038/nrneu・・・
著者: Silvia Tenembaum, Tanuja Chitnis, Jayne Ness, Jin S Hahn, International Pediatric MS Study Group
雑誌名: Neurology. 2007 Apr 17;68(16 Suppl 2):S23-36. doi: 10.1212/01.wnl.0000259404.51352.7f.
Abstract/Text Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory disorder of the CNS characterized by a widespread demyelination that predominantly involves the white matter of the brain and spinal cord. The condition is usually precipitated by a viral infection or vaccination. The presenting features include an acute encephalopathy with multifocal neurologic signs and deficits. Children are preferentially affected. In the absence of specific biologic markers, the diagnosis of ADEM is still based on the clinical and radiologic features. Although ADEM usually has a monophasic course, recurrent or multiphasic forms have been reported, raising diagnostic difficulties in distinguishing these cases from multiple sclerosis (MS). The International Pediatric MS Study Group proposes uniform definitions for ADEM and its variants. We discuss some of the difficulties in the interpretation of available literature due to the different terms and definitions used. In addition, this review summarizes current knowledge of the main aspects of ADEM, including its clinical and radiologic diagnostic features, epidemiology, pathogenesis, and outcome. An overview of ADEM treatment in children is provided. Finally, the controversies surrounding pediatric MS and ADEM are addressed.

PMID 17438235  Neurology. 2007 Apr 17;68(16 Suppl 2):S23-36. doi: 10.1・・・
著者: Dorothee Chabas, Jonathan Strober, Emmanuelle Waubant
雑誌名: Curr Neurol Neurosci Rep. 2008 Sep;8(5):434-41.
Abstract/Text Diagnosing multiple sclerosis (MS) in a child is challenging because of the limited diagnostic criteria and their overlap with acute disseminated encephalomyelitis. Pediatric-onset MS patients are more likely to be male, have seizures, and have brainstem and cerebellar symptoms than adults, and are less likely to have spinal cord symptoms than adults. They mostly experience a relapsing-remitting course. Their initial brain MRI shows more frequent involvement of the posterior fossa, less well-defined ovoid lesions, and more confluent lesions that decrease over time in patients with prepubertal onset, making early diagnosis even more difficult. Although disability progression is slower than in adults, pediatric onset MS leads to significant disability at a younger age and may be worse in non-white patients (up to 50% in North America). The rareness of pediatric-onset MS has precluded enrollment in clinical trials. Thus, children are receiving off-label adult therapies without clear evidence of their effectiveness and limited knowledge of their tolerability.

PMID 18713581  Curr Neurol Neurosci Rep. 2008 Sep;8(5):434-41.
著者: Dean M Wingerchuk
雑誌名: Curr Neurol Neurosci Rep. 2003 May;3(3):256-64.
Abstract/Text Postinfectious forms of encephalomyelitis, also termed acute disseminated encephalomyelitis (ADEM), form one of several categories of inflammatory demyelinating disorders of the central nervous system (CNS). Recent large, retrospective case series have refined our understanding of the clinical, laboratory, and neuroimaging characteristics of ADEM. The differences between childhood and adult ADEM, risks of development of multiple sclerosis, and the contributions of recent studies to refining the nosology of CNS demyelinating syndromes are discussed.

PMID 12691631  Curr Neurol Neurosci Rep. 2003 May;3(3):256-64.
著者: Daniela Pohl, Silvia Tenembaum
雑誌名: Curr Treat Options Neurol. 2012 Jun;14(3):264-75. doi: 10.1007/s11940-012-0170-0.
Abstract/Text OPINION STATEMENT: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease, characterized by an acute onset of polyfocal central nervous system (CNS) deficits, including encephalopathy, demonstrating multifocal lesions on MRI. ADEM is typically a monophasic disorder, but recurrent and multiphasic courses have been described. Furthermore, an ADEM presentation has been reported in neuromyelitis optica (NMO) and multiple sclerosis (MS), particularly in younger children. CNS infections, other autoimmune diseases, and neurometabolic disorders may mimic ADEM at manifestation. There is no single test confirming the diagnosis of ADEM, and diagnosis is based upon a combination of clinical and radiologic features and exclusion of diseases that resemble ADEM. Therefore, a broad workup including infectious, immunologic, and metabolic tests, as well as a systematic follow-up including MRI, is indicated to establish an accurate diagnosis as a prerequisite for an optimized treatment approach. There is a lack of evidence-based, prospective clinical trial data for the management of ADEM. Empiric antibacterial and antiviral treatment is standard of care until an infectious disease process is ruled out. Based on the presumed autoimmune etiology of ADEM, the common treatment approach consists of intravenous methylprednisolone at a dosage of 20 to 30 mg/kg per day (maximum 1 g/day) for 3 to 5 days, followed by an oral corticosteroid taper of 4 to 6 weeks. In case of insufficient response or contraindications to corticosteroids, intravenous immunoglobulin G (IVIG) at a dosage of 2 g/kg divided over 2 to 5 days is a therapeutic option. For severe or life-threatening cases of ADEM, plasmapheresis should be considered early in the disease course. Decompressive craniectomy has been reported as a life-saving measure for ADEM patients with intracranial hypertension. There is a lack of specific recommendations for the long-term management of recurrent and multiphasic ADEM. In children with relapsing demyelinating events, the diagnosis of a chronic autoimmune CNS disease like MS or NMO should be considered.

PMID 22476745  Curr Treat Options Neurol. 2012 Jun;14(3):264-75. doi: ・・・
著者: E Marchioni, S Ravaglia, G Piccolo, M Furione, E Zardini, D Franciotta, E Alfonsi, L Minoli, A Romani, A Todeschini, C Uggetti, E Tavazzi, M Ceroni
雑誌名: Neurology. 2005 Oct 11;65(7):1057-65. doi: 10.1212/01.wnl.0000179302.93960.ad.
Abstract/Text BACKGROUND: Acute disseminated encephalomyelitis (ADEM) refers to a monophasic acute multifocal inflammatory CNS disease. However, both relapsing and site-restricted variants, possibly associated with peripheral nervous system (PNS) involvement, are also observed, and a systematic classification is lacking.
OBJECTIVE: To describe a cohort of postinfectious ADEM patients, to propose a classification based on clinical and instrumental features, and to identify subgroups of patients with different prognostic factors.
METHODS: Inpatients of a Neurologic and Infectious Disease Clinic affected by postinfectious CNS syndrome consecutively admitted over 5 years were studied.
RESULTS: Of 75 patients enrolled, 60 fulfilled criteria for ADEM after follow-up lasting from 24 months to 7 years. Based on lesion distribution, patients were classified as encephalitis (20%), myelitis (23.3%), encephalomyelitis (13.3%), encephalomyeloradiculoneuritis (26.7%), and myeloradiculoneuritis (16.7%). Thirty patients (50%) had a favorable outcome. Fifteen patients (25%) showed a relapsing course. Poor outcome was related with older age at onset, female gender, elevated CSF proteins, and spinal cord and PNS involvement. All but two patients received high-dose steroids as first-line treatment, with a positive response in 39 (67%). Ten of 19 nonresponders (53%) benefited from high-dose IV immunoglobulin; 9 of 10 had PNS involvement. The data were not controlled.
CONCLUSIONS: A high prevalence of "atypical variants" was found in this series, with site-restricted damage or additional peripheral nervous system (PNS) involvement. Prognosis and response to steroids were generally good, except for some patient subgroups. In patients with PNS involvement and steroid failure, a favorable effect of IV immunoglobulin was observed.

PMID 16217059  Neurology. 2005 Oct 11;65(7):1057-65. doi: 10.1212/01.w・・・
著者: J Straub, M Chofflon, J Delavelle
雑誌名: Neurology. 1997 Oct;49(4):1145-7.
Abstract/Text We describe a patient with acute disseminated encephalomyelitis (ADEM) who was treated with high-dose intravenous methylprednisolone 2 days after onset of neurologic symptoms. Despite poor prognostic factors and extensive white matter lesions, the patient recovered dramatically with no need for maintenance steroid therapy. This case report of fulminant ADEM treated successfully with early high-dose intravenous methylprednisolone, although uncontrolled, suggests that this agent should be studied in other cases.

PMID 9339706  Neurology. 1997 Oct;49(4):1145-7.
著者: D S Kanter, D Horensky, R A Sperling, J D Kaplan, M E Malachowski, W H Churchill
雑誌名: Neurology. 1995 Apr;45(4):824-7.
Abstract/Text We describe two patients with fulminant acute disseminated encephalomyelitis (ADEM) treated with plasmapheresis after they failed to improve on steroids. Both patients improved concomitant with the plasma exchange. These are the first reported cases of fulminant ADEM with extensive white matter abnormalities on imaging studies treated with a regimen of plasmapheresis and steroids. Plasmapheresis may be beneficial in this disorder.

PMID 7723979  Neurology. 1995 Apr;45(4):824-7.
著者: E Marchioni, K Marinou-Aktipi, C Uggetti, M Bottanelli, A Pichiecchio, D Soragna, PiccoloG, F Imbesi, A Romani, M Ceroni
雑誌名: J Neurol. 2002 Jan;249(1):100-4.
Abstract/Text Randomized Controlled Trials have not let established the best pharmacological management of Acute Disseminated Encephalomyelitis (ADEM). High dose steroids are usually employed with good results, but in a few cases the clinical outcome is poor. In other patients, particularly those affected by the site restricted ADEM variants (myelitis), the disease shows a recurrent course resembling that of Multiple Sclerosis. We present here five patients, 3 of them affected by classic disseminated encephalomyelitis and 2 by a post infectious myelitis, which showed a good response to intravenous immunoglobulin (IVIg) after steroid treatment failure. In our report high dose steroids administration was substantially uneffective in all but one case, who showed a good response only during the first episode. On the contrary IVIg injection (0,4 gr/kg/day) produced a marked functional improvement in all patients starting within the first five days of drug administration and reaching a maximum within three weeks. One patient experienced a good effect nothwithstanding a steady dysability. In all cases, clinical evidence was supported by MRI controls showing improving posttreatment changes.

PMID 11954856  J Neurol. 2002 Jan;249(1):100-4.

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