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[Brain imaging in a case of early-onset acute disseminated encephalomyelitis]
Insights
Early-onset acute disseminated encephalomyelitis (ADEM) in an infant presented with neurological deficits after infection. Steroid treatment showed efficacy, but relapse necessitated further intervention, leading to recovery with residual weakness.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Neuroradiology
Background:
- Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated demyelinating disease affecting the central nervous system.
- Early-onset ADEM in infants presents diagnostic and therapeutic challenges due to nonspecific initial symptoms.
Observation:
- A 14-month-old girl developed severe neurological deficits, including spastic paraplegia, aphasia, and mental deterioration, two weeks post-upper respiratory infection.
- Clinical relapse occurred upon steroid withdrawal, characterized by meningeal signs, necessitating reinstitution of corticosteroid therapy.
Findings:
- Brain imaging (CT and MRI) revealed extensive, symmetric white matter lesions in the cerebrum, consistent with ADEM.
- Significant improvement in both clinical symptoms and neuroimaging abnormalities was observed following prolonged steroid treatment and subsequent rehabilitation.
Implications:
- This case highlights the potential for recovery from early-onset ADEM with timely diagnosis and aggressive immunosuppressive therapy.
- The findings underscore the importance of vigilant monitoring for relapse and the role of rehabilitation in optimizing functional outcomes in pediatric ADEM.
Abstract:
A case of early-onset acute disseminated encephalomyelitis was presented with her brain imaging. Two weeks after a nonspecific upper respiratory infection, a 14-month-old girl developed spastic paraplegia, difficulty in using left upper extremity and mental deterioration with aphasia. Steroid improved her clinical symptoms. However, 9 weeks later, when steroid was almost withdrawn, relapse with meningeal signs appeared. Reinstitution of steroid was started and 8 weeks later when meningeal signs disappeared, rehabilitation by physical therapist was started. Eighteen months later she recovered into normal intelligence and slight gait difficulty walking with left lower extremity weakness. CT showed extensive symmetric low density lesions in bilateral cerebral white matters and they almost resolved 8 months later. MRI showed extensive symmetric high signal lesions in bilateral cerebral white matters which were demonstrable in the sagittal image. Abnormalities in MRI were much improved 4 months later when she began to speak several words again.