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Neuropediatrics 2010; 41(6): 246-255
DOI: 10.1055/s-0031-1271656
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Acute Disseminated Encephalomyelitis: A Long-Term Prospective Study and Meta-Analysis

P. Pavone1 , M. Pettoello-Mantovano2 , A. Le Pira3 , I. Giardino2 , A. Pulvirenti4 , R. Giugno4 , E. Parano5 , A. Polizzi6 , 7 , A. Distefano5 , A. Ferro4 , 8 , L. Pavone3 , M. Ruggieri9
  • 1Department of Pediatrics, University Hospital “Vittorio Emanuele”, Catania, Italy
  • 2Department of Pediatrics, University of Foggia, Foggia, Italy
  • 3Unit of Clinical Pediatrics, Department of Pediatrics, University of Catania, Catania, Italy
  • 4Department of Mathematics and Computer Science, University of Catania, Catania, Italy
  • 5Institute of Neurological Science, Italian National Research Council, Catania, Italy
  • 6Superior Institute of Health, National Centre for Rare Diseases, Rome, Italy
  • 7Unit of Community Pediatrics “Etna Sud”, ASP3, Catania, Italy
  • 8Department of Biomedical Sciences, University of Catania, Catania, Italy
  • 9Chair of Pediatrics, Department of Formative Processes, University of Catania, Catania, Italy
Further Information

Publication History

received 09.06.2009

accepted 04.01.2011

Publication Date:
28 March 2011 (online)

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Abstract

Background: There are only a few series in the literature on acute disseminated encephalomyelitis (ADEM) in children.

Objectives and Methods: the aims of this study were to perform (i) a prospective clinical/imaging study (1992–2009) on ADEM in children consecutively referred to our institution in Catania, Italy, and (ii) to undertake a systematic review and meta-analysis of published ADEM pediatric cohorts (>10 cases).

Results: We identified 17 patients with ADEM (incidence <10 years of age=1.1 per 100 000 person-years). 15 previously published cohorts were compared with our cohort: (i) systematically reviewed (750 cases); and (ii) meta-analyzed (492/750 cases). The 17 patients had the following characteristics: (a) male-to-female ratio, 1.4 (vs. 1.2–1.3 in previous cohorts); (b) mean age at presentation, 3.6 years (vs. 7.1 years in previous cohorts); (c) specific preceding triggering factor, 88% (vs. 69–79% in previous cohorts); (d) the most common initial signs were ataxia, seizures, headache, and thalamic syndrome; (e) brain imaging revealed >3 lesions in 100% (vs. 92% in previous cohorts); (f) the outcome was good in 94% (vs. 70–75% in previous cohorts); and (g) 12% relapsed once (vs. 18% in previous cohorts).

Conclusions: ADEM is generally a benign condition that mosly affects boys more than girls and rarely recurs.

Key words

acute disseminated encephalomyelitis - ADEM - prospective cohort study - sytematic review - meta-analysis

References

  • 1 Anlar B, Basaran C, Kose G. et al . Acute disseminated encephalomyelitis in children: outcome and prognosis.  Neuropediatrics. 2003;  34 194-199
    Article in Thieme ConnectPubMedGoogle Scholar
  • 2 Banwell B, Ghezzi A, Bar-Or A. et al . Multiple sclerosis in children: clinical diagnosis, therapeutic strategies, and future directions.  Lancet Neurol. 2007;  6 887-902
    CrossrefPubMedGoogle Scholar
  • 3 Banwell B, Kennedy J, Sadovnick D. et al . Incidence of acquired demyelination of the CNS in Canadian children.  Neurology. 2009;  72 232-239
    CrossrefPubMedGoogle Scholar
  • 4 Caldemyer KS, Smith RR, Harris TM. et al . MRI in acute disseminated encephalomyelitis.  Neuroradiology. 1994;  36 216-220
    CrossrefPubMedGoogle Scholar
  • 5 Callen DJ, Shroff MM, Branson HM. et al . Role of MRI in the differentiation of ADEM from MS in children.  Neurology. 2009;  72 968-973
    CrossrefPubMedGoogle Scholar
  • 6 Dale RC, de Sousa C, Chong WK. et al . Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children.  Brain. 2000;  123 2407-2422
    CrossrefPubMedGoogle Scholar
  • 7 Efron B, Tibshirani RJ. An introduction to the bootstrap.. New York: Chapman & Hall; 1993
    Google Scholar
  • 8 Gupte G, Stonehouse M, Wassmer E. et al . Acute disseminated encephalomyelitis: A review of 18 cases in childhood.  J Paediatr Child Health. 2003;  336-342
    PubMedGoogle Scholar
  • 9 Hardison H, Yum S, Faerber EN. et al . Neuroimaging abnormalities and response to plasmapheresis acute disseminated encephalomyelitis in children: discordant neurologic and response to plasmapheresis.  Pediatrics. 2005;  116 431-436
    CrossrefPubMedGoogle Scholar
  • 10 Hung K-L, Liao H-T, Tsai M-L. The spectrum of postinfectious encephalomyelitis.  Brain Dev. 2001;  23 42-45
    CrossrefPubMedGoogle Scholar
  • 11 Hynson JL, Kornberg AJ, Coleman LT. et al . Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children.  Neurology. 2001;  56 1308-1312
    CrossrefPubMedGoogle Scholar
  • 12 Idrissova ZR, Boldyreva MN, Dekonenko EP. et al . Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage.  Eur J Neurol. 2003;  10 537-546
    CrossrefPubMedGoogle Scholar
  • 13 Jones CT. Childhood autoimmune neurologic diseases of the central nervous system.  Neurol Clin. 2003;  21 745-764
    CrossrefPubMedGoogle Scholar
  • 14 Krupp LB, Banwell B, Tenembaum S. for the International Pediatric MS Study Group . Consensus definitions proposed for pediatric multiple sclerosis and related disorders.  Neurology. 2007;  68 7-12
    CrossrefPubMedGoogle Scholar
  • 15 Leake JAD, Albani S, Kao AS. et al . Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features.  Pediatr Infect Dis J. 2004;  23 756-764
    CrossrefPubMedGoogle Scholar
  • 16 Lucas J. An account of uncommon symptoms succeeding the measles with additional remarks on the infection of measles and smallpox.  London Med J. 1790;  11 325-331
    PubMedGoogle Scholar
  • 17 Menge T, Hemmer B, Nessler S. et al . Acute disseminated encephalomyelitis: An update.  Arch Neurol. 2005;  62 1673-1680
    CrossrefPubMedGoogle Scholar
  • 18 Menge T, Kieseier BC, Nessler S. et al . Acute disseminated encephalomyelitis: an acute hit against the brain.  Curr Opin Neurol. 2007;  20 247-254
    CrossrefPubMedGoogle Scholar
  • 19 Mikaeloff Y, Suissa S, Vallèe L. et al . First episode of acute CNS inflammatory demyelination in childhood : prognostic factors for multiple sclerosis and disability.  J Pediatr. 2004;  144 246-252
    CrossrefPubMedGoogle Scholar
  • 20 Mikaeloff Y, Caridade G, Husson B. et al . Neuropediatric KIDSEP Study Group of the French Neuropediatric Society. Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse.  Eur J Paediatr Neurol. 2007;  11 90-95
    CrossrefPubMedGoogle Scholar
  • 21 Miller DH, Robb SA, Ormerod IEC. et al . Magnetic resonance imaging of inflammatory and demyelinating white-matter diseases of childhood.  Dev Med Child Neurol. 1990;  32 97-107
    PubMedGoogle Scholar
  • 22 Murthy KSN, Faden HS, Cohen ME. et al . Acute disseminated encephalomyelitis in children.  Pediatrics. 2002;  110 21-28
    CrossrefPubMedGoogle Scholar
  • 23 Noorbakhsh F, Johnson RT, Emery D. et al . Acute disseminated encephalomyelitis: clinical and pathogenesis features.  Neurol Clin. 2008;  26 759-780
    CrossrefPubMedGoogle Scholar
  • 24 O’Riordan JI, Thompson AJ, Kingsley DP. et al . The prognostic value of brain MRI in clinically isolated syndromes of CNS: A 10-year follow-up.  Brain. 1998;  121 495-503
    CrossrefPubMedGoogle Scholar
  • 25 Ruggieri M, Polizzi A, Pavone L. et al . Thalamic syndrome in children with measles infection and selective, reversible thalamic involvement.  Pediatrics. 1998;  101 112-119
    CrossrefPubMedGoogle Scholar
  • 26 Ruggieri M, Polizzi A, Pavone L. et al . Multiple sclerosis in children under 6 years of age.  Neurology. 1999;  53 478-484
    PubMedGoogle Scholar
  • 27 Schulze R. Meta-analysis: a comparison of approaches.. Hogrefe & Huber, Gottingen, Germany; 2004
    Google Scholar
  • 28 Stonehouse M, Gupte G, Wassmer E. et al . Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians.  Arch Dis Child. 2003;  88 122-124
    CrossrefPubMedGoogle Scholar
  • 29 Suppiej A, Vittorini R, Fontanin M. et al . Acute disseminated encephalomyelitis in children: Focus on relapsing patients.  Pediatr Neurol. 2008;  39 12-17
    CrossrefPubMedGoogle Scholar
  • 30 Tardieu M, Mikaeloff Y. Multiple sclerosis in children.  Int MS J. 2004;  11 36-42
    PubMedGoogle Scholar
  • 31 Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients.  Neurology. 2002;  59 1224-1231
    CrossrefPubMedGoogle Scholar
  • 32 Tenembaum S, Chitnis T, Ness J et al for the International Pediatric MS Study Group. Acute disseminated encephalomyelitis.  Neurol. 2007;  68 23-36
    PubMedGoogle Scholar
  • 33 Tenembaum SN. Disseminated encephalomyelitis in children.  Clin Neurol Neurosurg. 2008;  1119 928-938
    PubMedGoogle Scholar
  • 34 Torisu H, Kira R, Ishizaki Y. et al . Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis, and acute transverse myelitis in Fukuoka Prefecture, Japan.  Brain Dev. 2010;  32 454-462
    CrossrefPubMedGoogle Scholar
  • 35 Weng WC, Peng SS, Lee WT. et al . Acute disseminated encephalomyelitis in children: one medical center experience.  Acta Paediatr Taiwan. 2006;  47 67-71
    PubMedGoogle Scholar
  • 36 Wingerchuk DM. Postinfectious encephalomyelitis.  Curr Neurol Neurosci Rep. 2003;  3 256-264
    CrossrefPubMedGoogle Scholar

Correspondence

Martino RuggieriMD, PhD 

Chair of Pediatrics

Department of Formative

Processes

University of Catania

Via Biblioteca 4

95124 Catania

Italy

Phone: +39/095/746 6301

Fax: +39/095/316 792

Email: m.ruggieri@isn.cnr.it

Email: m.ruggieri@unict.it