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Gadolinium ring enhancement and mass effect in acute disseminated encephalomyelitis
C H van der Meyden1, J F de Villiers, B D Middlecote
1Department of Neurology, University of the Orange Free State, Bloemfontein, South Africa.
Abstract:
A 9-year-old boy presented with a subacute history of optic neuritis followed by brainstem involvement, with fever and a lymphocytic pleocytosis in the cerebrospinal fluid. Gadolinium-enhancing ring lesions were demonstrated in the white matter of the cerebrum, brain-stem and cerebellum on day 17 of the illness, all appearing simultaneously as part of a monophasic illness. A parietal lesion exerted mass effect. Needling and biopsy yielded no evidence of a pyogenic lesion, tumour or tuberculosis and showed vasculitis. There was insufficient material for myelin staining. Dexamethasone therapy lead to rapid improvement of the radiological lesions: MRI and CT on day 34 of the illness showed complete clearing of the lesions except for residual abnormality at the biopsy site.
Insights
A pediatric case of acute disseminated encephalomyelitis (ADEM) presented with optic neuritis and brainstem lesions. Dexamethasone therapy rapidly resolved the brain lesions, suggesting ADEM as a diagnosis.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimmunology
Background:
- A 9-year-old boy experienced subacute optic neuritis and brainstem dysfunction.
- Fever and lymphocytic pleocytosis were noted in the cerebrospinal fluid.
Observation:
- Simultaneous, gadolinium-enhancing ring lesions appeared in the brain's white matter, brainstem, and cerebellum.
- A parietal lesion caused mass effect, and biopsy revealed vasculitis, excluding infection or tumor.
Findings:
- The illness presented as a monophasic event.
- Dexamethasone treatment led to rapid radiological improvement, with near-complete lesion clearing on MRI and CT scans.
Implications:
- This case highlights the presentation and favorable response to corticosteroids in a child with possible acute disseminated encephalomyelitis (ADEM).
- Early diagnosis and treatment are crucial for managing such neuroinflammatory conditions in children.