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Published on: September 9, 2022
Multiphasic acute disseminated encephalomyelitis or pediatric multiple sclerosis: report of an atypical case
Agnese Suppiej1, Renzo Manara, Luca De Palma
1Department of Paediatrics, University of Padova, Padova, Italy. suppiej@pediatria.unipd.it
Insights
This case study highlights a child with recurrent central nervous system demyelination, initially diagnosed as multiphasic acute disseminated encephalomyelitis, who later developed symptoms suggestive of secondary progressive multiple sclerosis.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Consensus definitions for pediatric multiple sclerosis (MS) and related disorders have been proposed.
- Understanding the clinical overlap between pediatric MS and acute disseminated encephalomyelitis (ADEM) is crucial.
- Long-term follow-up studies are valuable for refining diagnoses in atypical cases.
Observation:
- A case of a 3-year-old girl with recurrent central nervous system demyelination, initially consistent with multiphasic ADEM, is presented.
- Over an 8-year period, the patient developed cognitive deterioration, mood disorder, and headaches at age 10.
- Diagnostic workup, including MRI and CSF analysis, was equivocal, with only visual evoked potentials favoring a diagnosis of MS.
Findings:
- The patient's presentation evolved from multiphasic ADEM to symptoms suggestive of secondary progressive multiple sclerosis.
- This case underscores the diagnostic challenges in differentiating pediatric MS from ADEM, especially in atypical presentations.
- Visual evoked potentials served as a key diagnostic indicator in this complex case.
Implications:
- A multifaceted diagnostic approach is essential for atypical demyelinating diseases in young children.
- Further research and long-term studies are needed to clarify the relationship between pediatric MS and ADEM.
- Improved diagnostic criteria may enhance patient outcomes and management strategies for pediatric demyelinating disorders.
Abstract:
An international panel has recently proposed consensus definitions for pediatric multiple sclerosis and related disorders. These are important diagnostic improvements, but exceptions have been acknowledged. Further insight about clinical overlap between pediatric multiple sclerosis and all forms of relapsing acute disseminated encephalomyelitis may be gained from long-term follow-up. We report an 8-year follow-up of a girl who developed multiple episodes of central nervous system demyelination at the age of 3 years consistent with multiphasic acute disseminated encephalomyelitis. At 10 years of age (7 years after the first clinical event), she developed progressive cognitive deterioration, mood disorder, and headache, suggesting a secondary progressive form of multiple sclerosis. Magnetic resonance imaging and cerebrospinal fluid analysis were equivocal while visual evoked potentials were the sole test in favor of a diagnosis of multiple sclerosis. A multifaceted approach may be needed when dealing with atypical cases of demyelinating disease in young children.
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