Acute disseminated encephalomyelitis: A retrospective study of 20 children in a pediatrics department in Tunisia.


Journal

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
ISSN: 1769-664X
Titre abrégé: Arch Pediatr
Pays: France
ID NLM: 9421356

Informations de publication

Date de publication:
Nov 2021
Historique:
received: 22 11 2017
revised: 03 08 2021
accepted: 26 09 2021
pubmed: 26 10 2021
medline: 15 12 2021
entrez: 25 10 2021
Statut: ppublish

Résumé

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system. Little information is available about the clinical and neuroradiological profile or the follow-up of this disease in Tunisian children. To determine the clinical, laboratory, and radiological features and the outcome of ADEM in children admitted to the pediatrics department of a university hospital in Tunisia. All children ≤ 18 years old presenting with ADEM and admitted to the tertiary referral center for pediatrics at Sahloul University Hospital from January 2000 to December 2020 were included in the study. The diagnosis of ADEM was confirmed according to the international pediatric multiple sclerosis study group criteria. A total of 20 patients (13 girls and 7 boys) fulfilled the diagnostic criteria for ADEM. The mean age at diagnosis was 5.6 years. The clinical presentation included polyfocal neurological signs such as cranial hypertension (45%), seizures (35%), and motor weaknesses (55%). Pyramidal tract signs and cranial nerve palsies were noted in 55% of cases. Brain magnetic resonance imaging showed particular features, namely, a relapsing tumor-like form in one case, and optic neuritis and demyelinating lesions of the white matter in the brain and the spinal cord with gadolinium cerebral ring-like enhancement in another case. The treatment consisted of intravenous immunoglobulin in 16 cases (80%) and corticosteroid in 19 cases (95%). Plasmapheresis was used for one patient. Complete recovery was observed in 12 patients (60%); 19 patients (95%) had a monophasic course of the disease while only one patient developed multiphasic ADEM. ADEM remains a difficult diagnosis in children. Nevertheless, after prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with restitutio ad integrum.

Sections du résumé

BACKGROUND BACKGROUND
Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system. Little information is available about the clinical and neuroradiological profile or the follow-up of this disease in Tunisian children.
AIM OBJECTIVE
To determine the clinical, laboratory, and radiological features and the outcome of ADEM in children admitted to the pediatrics department of a university hospital in Tunisia.
METHODS METHODS
All children ≤ 18 years old presenting with ADEM and admitted to the tertiary referral center for pediatrics at Sahloul University Hospital from January 2000 to December 2020 were included in the study. The diagnosis of ADEM was confirmed according to the international pediatric multiple sclerosis study group criteria.
RESULTS RESULTS
A total of 20 patients (13 girls and 7 boys) fulfilled the diagnostic criteria for ADEM. The mean age at diagnosis was 5.6 years. The clinical presentation included polyfocal neurological signs such as cranial hypertension (45%), seizures (35%), and motor weaknesses (55%). Pyramidal tract signs and cranial nerve palsies were noted in 55% of cases. Brain magnetic resonance imaging showed particular features, namely, a relapsing tumor-like form in one case, and optic neuritis and demyelinating lesions of the white matter in the brain and the spinal cord with gadolinium cerebral ring-like enhancement in another case. The treatment consisted of intravenous immunoglobulin in 16 cases (80%) and corticosteroid in 19 cases (95%). Plasmapheresis was used for one patient. Complete recovery was observed in 12 patients (60%); 19 patients (95%) had a monophasic course of the disease while only one patient developed multiphasic ADEM.
CONCLUSIONS CONCLUSIONS
ADEM remains a difficult diagnosis in children. Nevertheless, after prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with restitutio ad integrum.

Identifiants

pubmed: 34690025
pii: S0929-693X(21)00203-7
doi: 10.1016/j.arcped.2021.09.027
pii:
doi:

Substances chimiques

Adrenal Cortex Hormones 0
Immunoglobulins, Intravenous 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

638-646

Informations de copyright

Copyright © 2021 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare that they have no competing interests.

Auteurs

Houda Ajmi (H)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia. Electronic address: houda.ajmi@rns.tn.

Nesrine Jammeli (N)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Saida Hassayoun (S)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Nadia Mama (N)

Department of Radiology, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Sameh Mabrouk (S)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Nadia Arifa (N)

Department of Radiology, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Jalel Chemli (J)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Noura Zouari (N)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Hela Jemni (H)

Department of Radiology, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

Saoussan Abroug (S)

Department of Pediatrics, Sahloul Teaching Hospital, Sousse 4054, Tunisia.

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