[Acute disseminated encephalomyelitis in Tunisia: Report of a pediatric cohort]

Abstract

Introduction: Acute disseminated encephalomyelitis (ADEM) is an inflammatory, demyelinating disorder of the central nervous system whose clinical features, management and outcome are incompletely understood in Tunisian population.

Objective: To describe clinical, neuroimaging and laboratory features; treatment and outcome in a cohort of Tunisian children with ADEM.

Methods: We conducted a retrospective review of the medical records of all children attending the Department of Child and Adolescent Neurology (Tunis) with ADEM between 2005 and 2015. Clinical, neuroimaging and laboratory features, therapeutic data and outcome were analyzed.

Results: There were 15 children (7 males and 8 females). The mean age at onset was 6.9 years. Thirteen (86.6%) patients had a prodromal event. The onset of neurological symptoms occurred within 17.6 days (4-30). Limb weakness was the most common presenting symptom (53.3%). Extrapyramidal syndrome was noticed in 6 patients (40%). Initial MRI showed a deep gray matter involvement in 7 cases (46.6%). Gadolinium enhancement at acute stage was observed in only 2 patients (13%). Cerebrospinal fluid findings did not show intrathecal oligoclonal bands. The use of high-dose IV methylprednisolone followed by oral steroid taper was associated with rapid recovery. Additional treatment with intravenous immunoglobulin was necessary in 2 patients. Complete recovery was obtained in 11 patients (73.3%). A monophasic course was noticed in 14 cases. Only one patient (5%) developed multiple sclerosis.

Conclusion: The high frequency of prodromal events and extrapyramidal syndrome in addition to the low rate of gadolinium enhancement at acute stage seem to be the main features in our patients. Larger ADEM multicenter cohort studies in Tunisia and North Africa could provide more detailed information about this entity.

Keywords: Acute disseminated encephalomyelitis; Auto-immune diseases; Children; Encéphalomyélite aiguë disséminée; Enfants; Maladies auto-immunes; Postinfectieux; Postinfectious.