ACUTE DISSEMINATED ENCEPHALOMYELITIS – A CASE SERIES

Maria Miguel Gomes, Ariana Afonso, João Soares-Fernandes, Henedina Antunes, Célia Barbosa

Abstract


Background: Acute disseminated encephalomyelitis (ADEM) is a demyelinating monophasic disorder of the central nervous system characterized by diffuse neurologic signs and symptoms coupled with multifocal demyelinating lesions on neuroimaging.

Aims: To describe the epidemiology, symptoms and signs, laboratory features, neuroimaging, treatment and outcome in a pediatric cohort with the initial diagnosis of ADEM.

Methods and Materials: A retrospective review, between 2010-2015, of children/adolescents admitted at a level II hospital.

Results: Eleven cases were identified with male predominance and ages between 8 months and 16 years. Seven patients presented in winter/spring. More than half of the cases had a recent infectious disease. Patients presented most often with motor deficits, cranial neuropathy and stiff neck. All patients had spinal fluid abnormalities. A definitive microbiologic diagnosis was established in two children with Epstein-Barr virus disease and probable/possible diagnosis in two children with previous rotavirus disease or varicella. Brain magnetic resonance imaging (MRI) identified multifocal lesions in every case. All patients were treated with corticosteroids, and two were also treated with human immunoglobulin. There was one case with multiphasic ADEM (after one year follow-up) and two cases developed criteria of multiple sclerosis (MS). All patients survived. Two patients had neurologic sequelae. The differential diagnosis between ADEM and MS at first presentaion may be challenging.

Conclusions: Our case series suggests that infections are associated with the etiology of ADEM. MRI was the neuroimaging study of choice for diagnosis and follow-up. Prognosis was excellent. It remains difficult to differentiate ADEM and multiple sclerosis.


Keywords


Acute Disseminated Encephalomyelitis; pediatrics; magnetic resonance imaging

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