Stroke and mixed connective tissue disease

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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
PubMed

Insights

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.
  • 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.

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