Acute disseminated encephalomyelitis: a long-term prospective study and meta-analysis

• Published in: Neuropediatrics Vol. 41; no. 6; p. 246
• Main Authors: Pavone, P, Pettoello-Mantovano, M, Le Pira, A, Giardino, I, Pulvirenti, A, Giugno, R, Parano, E, Polizzi, A, Distefano, A, Ferro, A, Pavone, L, Ruggieri, M
• Format: Journal Article
• Language: English
• Published: Germany 01.12.2010
• Subjects: Adrenal Cortex Hormones - therapeutic use; Age of Onset; Brain - pathology; Brain - physiopathology; Child; Electroencephalography; Encephalomyelitis, Acute Disseminated - diagnosis; Encephalomyelitis, Acute Disseminated - drug therapy; Encephalomyelitis, Acute Disseminated - physiopathology; Female; Glucocorticoids - therapeutic use; Humans; Male; Prognosis; Prospective Studies; Treatment Outcome
• ISSN: 1439-1899
• DOI: 10.1055/s-0031-1271656

There are only a few series in the literature on acute disseminated encephalomyelitis (ADEM) in children. 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). 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). ADEM is generally a benign condition that mosly affects boys more than girls and rarely recurs.