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

P. Pavone; M. Pettoello-Mantovano; A. Le Pira; I. Giardino; A. Pulvirenti; R. Giugno; E. Parano; A. Polizzi; A. Distefano; A. Ferro; L. Pavone; M. Ruggieri

doi:10.1055/s-0031-1271656

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

Original Article

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

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

Volltext

Referenzen

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Correspondence

Martino RuggieriMD, PhD

Chair of Pediatrics

Department of Formative

Processes

University of Catania

Via Biblioteca 4

95124 Catania

Italy

Telefon: +39/095/746 6301

Fax: +39/095/316 792

eMail: m.ruggieri@isn.cnr.it

eMail: m.ruggieri@unict.it