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Stroke and mixed connective tissue disease

W D Graf¹, J M Milstein, D D Sherry

¹Department of Pediatrics, Children's Hospital and Medical Center, University of Washington, Seattle 98105.

Journal of Child Neurology

|July 1, 1993

Summary

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Cerebrovascular disease can be a primary sign in children with mixed connective tissue disease, presenting with varied neurological outcomes. Prompt treatment may lead to recovery, but severe cases can be fatal.

Area of Science:

Neurology
Rheumatology
Pediatrics

Background:

Mixed connective tissue disease (MCTD) is an autoimmune disorder with overlapping features of other connective tissue diseases.
Cerebrovascular disease, encompassing conditions affecting brain blood vessels, is a known complication in some autoimmune disorders.

Observation:

Two pediatric cases of MCTD with cerebrovascular events are presented.
Case 1: A girl with rapid onset hemiparesis and aphasia due to internal carotid artery occlusion, who recovered with immunosuppressive therapy.
Case 2: A girl with MCTD who experienced fatal neurologic deterioration from intracerebral hemorrhage, confirmed as small-vessel fibrinoid necrosis on autopsy.

Findings:

This study highlights cerebrovascular disease as a primary manifestation in MCTD, distinct from its association with systemic lupus erythematosus.

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The cases illustrate a spectrum of cerebrovascular complications in pediatric MCTD, ranging from arterial occlusion to hemorrhage.

Histopathological findings in one fatal case indicated small-vessel vasculitis.

Implications:

Recognizing cerebrovascular disease as a potential primary sign in pediatric MCTD is crucial for timely diagnosis and intervention.
These findings may prompt further research into the pathogenesis and management of cerebrovascular complications in MCTD.
Early detection and aggressive treatment strategies are essential to improve outcomes for children with MCTD and neurological involvement.