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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients
Silvia Tenembaum1, Nestor Chamoles, Natalio Fejerman
1Department of Neurology, Hospital Nacional de Pediatría Dr. J. P. Garrahan, Buenos Aires, Argentina. solier@ciudad.com.ar
Insights
Childhood acute disseminated encephalomyelitis (ADEM) is typically benign, with most boys recovering well after treatment. Optic nerve involvement at onset is linked to disability, but ADEM can be distinguished from MS over time.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system (CNS).
- Limited pediatric studies exist, often with retrospective designs and short follow-up periods.
Purpose of the Study:
- To characterize a cohort of pediatric ADEM patients.
- To identify clinical and neuroimaging predictors of patient outcomes.
Main Methods:
- Prospective study of 84 consecutive pediatric ADEM cases from 1988-2000.
- Clinical assessment, neuroimaging (MRI), and cerebrospinal fluid (CSF) analysis were performed.
- Long-term follow-up evaluated clinical course and disability (Expanded Disability Status Scale).
Main Results:
- Mean age at onset was 5.3 years, with male predominance. Most cases (74%) followed viral illness or vaccination.
- Prominent symptoms included hemiparesis (76%), long tract signs (85%), and altered mental state (69%).
- High-dose corticosteroids, especially IV methylprednisolone, correlated with good recovery and MRI lesion resolution. 90% had a monophasic course; 11% had moderate to severe disability (EDSS 3-6.5).
Conclusions:
- Childhood ADEM is generally a benign condition with a favorable prognosis, particularly in boys.
- No MRI-defined groups predicted disability; optic nerve involvement at presentation was associated with poorer outcomes.
- Long-term follow-up and CSF analysis (absence of oligoclonal bands) helped differentiate ADEM from multiple sclerosis (MS), even in relapsing cases.
Background:
Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the CNS. Few pediatric series have been published, with retrospective and short-term follow-up studies.
Objectives:
To describe a cohort of pediatric patients with ADEM and to determine whether clinical and neuroimaging findings predict outcome.
Methods:
A prospective study was conducted between March 1988 and July 2000 on 84 consecutive children with ADEM at the National Pediatric Hospital "Dr. J. P. Garrahan."
Results:
Mean age at onset was 5.3 +/- 3.9 years, with a significant male predominance. Sixty-two patients (74%) had a preceding viral illness or vaccination. Acute hemiparesis (76%), unilateral or bilateral long tract signs (85%), and changes in mental state (69%) were the most prominent presenting features. Four MRI groups were identified: ADEM with small lesions (62%), with large lesions (24%), with additional bithalamic involvement (12%), and acute hemorrhagic encephalomyelitis (2%). Of the 54 children whose CSF samples were analyzed, none showed intrathecal oligoclonal bands. The use of high-dose corticosteroid treatment, particularly IV methylprednisolone, was associated with good recovery and resolution of MRI lesions. After a mean follow-up of 6.6 +/- 3.8 years, 90% of children showed a monophasic course, and 10% a biphasic disease. Eighty-nine percent of patients show at present Expanded Disability Status Scale scores of 0 to 2.5. Eleven percent have disability scores of 3 to 6.5.
Conclusions:
Childhood acute disseminated encephalomyelitis is a benign condition, affecting boys more frequently. No association was found between MRI groups and disability. Disability was related to optic nerve involvement at presentation. Even in relapsing cases, the distinction between acute disseminated encephalomyelitis and MS was possible on the basis of long-term clinical and neuroimaging follow-up and the absence of oligoclonal bands in CSF.
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