Torisu H, Kira R, Ishizaki Y, Sanefuji M, Yamaguchi Y, Yasumoto S, Murakami Y, Shimono M, Nagamitsu S, Masuzaki M, Amamoto M, Kondo R, Uozumi T, Aibe M, Gondo K, Hanai T, Hirose S, Matsuishi T, Shirahata A, Mitsudome A, Hara T.
Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis, and acute transverse myelitis in Fukuoka Prefecture, Japan.
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.
Yamaguchi Y, Torisu H, Kira R, Ishizaki Y, Sakai Y, Sanefuji M, Ichiyama T, Oka A, Kishi T, Kimura S, Kubota M, Takanashi J, Takahashi Y, Tamai H, Natsume J, Hamano S, Hirabayashi S, Maegaki Y, Mizuguchi M, Minagawa K, Yoshikawa H, Kira J, Kusunoki S, Hara T.
A nationwide survey of pediatric acquired demyelinating syndromes in Japan.
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.
Menge T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, Kieseier BC, Stüve O.
Acute disseminated encephalomyelitis: an update.
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.
[www.info.pmda.go.jp/juutoku/file/jfm1104009.pdf 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 厚生労働省].
Parsons T, Banks S, Bae C, Gelber J, Alahmadi H, Tichauer M.
COVID-19-associated acute disseminated encephalomyelitis (ADEM).
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.
de Miranda Henriques-Souza AM, de Melo ACMG, de Aguiar Coelho Silva Madeiro B, Freitas LF, Sampaio Rocha-Filho PA, Gonçalves FG.
Acute disseminated encephalomyelitis in a COVID-19 pediatric patient.
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.
Noorbakhsh F, Johnson RT, Emery D, Power C.
Acute disseminated encephalomyelitis: clinical and pathogenesis features.
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.
Studahl M, Bergström T, Hagberg L.
Acute viral encephalitis in adults--a prospective study.
Scand J Infect Dis. 1998;30(3):215-20. doi: 10.1080/00365549850160828.
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.
Sivertsen B, Christensen PB.
Acute encephalitis.
Acta Neurol Scand. 1996 Feb-Mar;93(2-3):156-9. doi: 10.1111/j.1600-0404.1996.tb00192.x.
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).
Krupp LB, Tardieu M, Amato MP, Banwell B, Chitnis T, Dale RC, Ghezzi A, Hintzen R, Kornberg A, Pohl D, Rostasy K, Tenembaum S, Wassmer E; International Pediatric Multiple Sclerosis Study Group.
International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions.
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.
Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B.
Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients.
Neurology. 2001 May 22;56(10):1313-8. doi: 10.1212/wnl.56.10.1313.
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.
Olsson T.
White matter disease: Roles of anti-MOG antibodies in demyelinating diseases.
Nat Rev Neurol. 2011 May;7(5):248-9. doi: 10.1038/nrneurol.2011.45. Epub 2011 Apr 12.
Abstract/Text
Tenembaum S, Chitnis T, Ness J, Hahn JS; International Pediatric MS Study Group.
Acute disseminated encephalomyelitis.
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.
Chabas D, Strober J, Waubant E.
Pediatric multiple sclerosis.
Curr Neurol Neurosci Rep. 2008 Sep;8(5):434-41. doi: 10.1007/s11910-008-0067-1.
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.
Wingerchuk DM.
Postinfectious encephalomyelitis.
Curr Neurol Neurosci Rep. 2003 May;3(3):256-64. doi: 10.1007/s11910-003-0086-x.
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.
Pohl D, Tenembaum S.
Treatment of acute disseminated encephalomyelitis.
Curr Treat Options Neurol. 2012 Jun;14(3):264-75. doi: 10.1007/s11940-012-0170-0.
Abstract/Text
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.
Marchioni E, Ravaglia S, Piccolo G, Furione M, Zardini E, Franciotta D, Alfonsi E, Minoli L, Romani A, Todeschini A, Uggetti C, Tavazzi E, Ceroni M.
Postinfectious inflammatory disorders: subgroups based on prospective follow-up.
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.
Straub J, Chofflon M, Delavelle J.
Early high-dose intravenous methylprednisolone in acute disseminated encephalomyelitis: a successful recovery.
Neurology. 1997 Oct;49(4):1145-7. doi: 10.1212/wnl.49.4.1145.
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.
道具 伸浩, 高嶋 修太郎, 田口 芳治, 笹原 悦子, 井上 博, 田中 耕太郎.神経内科地域基幹教育病院における，成人発症急性脳炎と臨床的に診断した症例の，臨床・画像・予後に関する検討臨床神経，46：533－539，2006.
Kanter DS, Horensky D, Sperling RA, Kaplan JD, Malachowski ME, Churchill WH Jr.
Plasmapheresis in fulminant acute disseminated encephalomyelitis.
Neurology. 1995 Apr;45(4):824-7. doi: 10.1212/wnl.45.4.824.
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.
Marchioni E, Marinou-Aktipi K, Uggetti C, Bottanelli M, Pichiecchio A, Soragna D, PiccoloG, Imbesi F, Romani A, Ceroni M.
Effectiveness of intravenous immunoglobulin treatment in adult patients with steroid-resistant monophasic or recurrent acute disseminated encephalomyelitis.
J Neurol. 2002 Jan;249(1):100-4. doi: 10.1007/pl00007836.
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.
