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[A case of early-onset acute disseminated encephalomyelitis]
A Nishimura1, T Fuchigami, H Izumi
1Department of Pediatrics, Nihon University School of Medicine, Tokyo.
Abstract:
We reported a rare case of early-onset acute disseminated encephalomyelitis (ADEM). After a nonspecific upper respiratory infection, a 14-month-old boy developed oculomotor nerve paralysis, nystagmus, intention tremor and ataxic gait. MRI showed extensive symmetric high signal lesions in th bilateral cerebellar and cerebral white matters. We made the diagnosis of ADEM on the basis of high CSF myelin basic protein, clinical course, symptoms and MRI findings. Following the administration of steroid hormone, his clinical symptoms rapidly improved.
Insights
This case report details a rare instance of early-onset acute disseminated encephalomyelitis (ADEM) in a 14-month-old boy. Prompt steroid treatment led to rapid symptom improvement, highlighting effective therapeutic options.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune disorder.
- Early-onset ADEM in infants is exceptionally uncommon.
- Post-infectious neurological complications pose diagnostic challenges in young children.
Observation:
- A 14-month-old boy presented with oculomotor nerve paralysis, nystagmus, intention tremor, and ataxic gait following an upper respiratory infection.
- Cerebral magnetic resonance imaging (MRI) revealed extensive, symmetric white matter lesions in the cerebellum and cerebrum.
- Cerebrospinal fluid (CSF) analysis showed elevated myelin basic protein levels.
Findings:
- The clinical presentation, MRI findings, and elevated CSF myelin basic protein confirmed a diagnosis of ADEM.
- The patient experienced a rapid improvement in neurological symptoms after steroid hormone therapy.
- This case underscores the importance of considering ADEM in infants with acute neurological deficits post-infection.
Implications:
- Early diagnosis and prompt treatment with corticosteroids can significantly improve outcomes in pediatric ADEM.
- This case contributes to the understanding of rare demyelinating diseases in early childhood.
- Further research into the specific triggers and long-term prognosis of early-onset ADEM is warranted.