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[Can acute disseminated encephalomyelitis progress in a deferred way?]
B Gener1, C Garaizar-Axpe, C Ruiz Espinosa
1Hospital de Cruces, Baracaldo, 48903, España.
Objective:
To report on the heterogeneity with regard to the clinical course of the acute disseminated encephalomyelitis (ADEM).
Case Report:
A 5 year old boy suffered of acute disseminated encephalomyelitis of unknown origin. This child suffered two episodes of different neurologic symptoms separated by several weeks. Based on the clinical manifestations and typical appearance of magnetic resonance imaging findings and the absence of oligoclonal bands in CSF immunoglobulins, multiple sclerosis (MS) was ruled out.
Conclusion:
We postulate that the recurrent symptoms in our patient could be explained as a multiphasic disseminated encephalomyelitis (MDEM). Favourable outcome after simultaneous treatment with methylprednisolone and intravenous immunoglobulin is emphasized in this report.
Insights
Acute disseminated encephalomyelitis (ADEM) can present with recurrent symptoms, suggesting a multiphasic course. This case highlights favorable outcomes with combined methylprednisolone and immunoglobulin therapy for recurrent ADEM.
Area of Science:
- Neurology
- Pediatric Neurology
- Immunology
Background:
- Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disease.
- ADEM typically presents as a monophasic illness, but heterogeneous clinical courses are increasingly recognized.
- Differentiating recurrent ADEM from other demyelinating conditions like multiple sclerosis (MS) is crucial for appropriate management.
Observation:
- A 5-year-old boy experienced two distinct episodes of neurological symptoms separated by several weeks.
- Clinical presentation and magnetic resonance imaging (MRI) findings were consistent with ADEM.
- Cerebrospinal fluid (CSF) analysis showed no oligoclonal bands, aiding in the exclusion of multiple sclerosis (MS).
Findings:
- The recurrent neurological symptoms in this pediatric patient suggest a multiphasic disseminated encephalomyelitis (MDEM).
- The case demonstrates that ADEM can exhibit a multiphasic or relapsing-remitting course.
- Exclusion of MS was based on clinical course, MRI, and CSF findings.
Implications:
- Recognition of MDEM is important for accurate diagnosis and prognosis in pediatric demyelinating diseases.
- Prompt and aggressive treatment, including corticosteroids and immunoglobulins, may lead to favorable outcomes in MDEM.
- This case underscores the importance of considering multiphasic presentations of ADEM in children.