Mid-to long-term disability and functional outcomes in pediatric patients with monophasic acute disseminated encephalomyelitis

Abstract

Background

Acute disseminated encephalomyelitis (ADEM) is a rare disease characterized by encephalopathy, polyfocal symptoms and demyelination. Although its prognosis is generally favorable, there is growing evidence that subtle neuropsychological and motoric sequelae may persist years after the initial episode.

Aim

To assess the relationship between clinical, laboratory and radiological characteristics of the acute monophasic ADEM episode in children, and its immediate outcome, and long(er) term disability/functional status.

Methods

Retrospective chart review embraced all children managed for monophasic ADEM over 18 years at a single tertiary center. They were invited for a follow-up medical, neurological assessment, and disability/functional outcomes assessment [modified Rankin scale (mRS), Extended Disability Status Scale and Functional Systems Scores (EDSS-FSS)].

Results

Of the 65 children (age 10–250 months, median 90; 46.2 % boys), 40 (61.5 %) were judged as fully recovered, and 25 had sequelae, two of whom (aged 23 and 13 months) suffered severe ocular or cortical symptoms. Follow-up assessment 21–211 months (median 154) after hospital discharge embraced 41 children, 27 discharged as recovered, 14 with sequelae. Of the latter, the two children with severe sequelae were seriously disabled with decrease in mentation, while 11/14 had mRS 0, and EDSS-FSS 0. Of the former, 7 had mRS = 1 and one had mRS = 2, whereas 5 had EDSS-FSS = 2, and 2 had EDSS-FSS = 3, including 2 children with mood alterations and 2 with mild decrease in mentation.

Conclusions

Unless truly severe, acute episode sequelae do not predict long-term disability/functional deficiency. Children recovered after the acute episode may have mild symptoms/deficiencies in long term.