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Central nervous system complications in two cases of juvenile onset dermatomyositis
A V Ramanan1, S Sawhney, K J Murray
1Juvenile Dermatomyositis Research Centre, Rheumatology Unit, Great Ormond Street Hospital and the Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK.
Insights
Central nervous system (CNS) complications are rare in juvenile inflammatory myopathies. This report details two pediatric cases of juvenile dermatomyositis with severe CNS involvement, highlighting potential cerebral vasculopathy and under-recognition of these serious neurological manifestations.
Area of Science:
- Neurology
- Pediatrics
- Rheumatology
Background:
- Inflammatory myopathies, including juvenile dermatomyositis (JDM), typically present with muscle and skin inflammation.
- Central nervous system (CNS) involvement is considered a rare complication in JDM, with limited documented cases.
Observation:
- Two pediatric patients diagnosed with JDM, aged 4 and 10 years, presented with severe neurological symptoms.
- Clinical features suggested CNS involvement, potentially indicative of cerebral vasculopathy or vasculitis.
Findings:
- One child experienced fatal brainstem involvement, while the other developed seizures, pseudoseizures, and depression.
- The patient with seizures and depression showed improvement following aggressive immunosuppressive therapy.
Implications:
- This case series suggests that CNS complications in JDM may be under-recognized.
- Cerebral vasculopathy should be considered in the differential diagnosis of neurological symptoms in pediatric patients with JDM.
- Early recognition and aggressive immunosuppression may be crucial for managing CNS involvement in JDM.
Abstract:
Central nervous system (CNS) complications are rarely reported in either juvenile or adult onset inflammatory myositides, such as dermatomyositis and polymyositis. We report two children, aged 4 and 10 yr respectively, with a diagnosis of juvenile dermatomyositis, both of whom subsequently developed clinical features of severe CNS involvement, possibly consistent with cerebral vasculopathy. One child died from apparent brainstem involvement; the other developed seizures, pseudoseizures and clinical depression which responded to aggressive immunosuppression. Although the vasculopathy or vasculitis underlying this disorder is known to have a systemic distribution, CNS involvement has rarely been reported and may be under-recognized.