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Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
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Childhood central nervous system vasculitis

Susanne Benseler1, Daniela Pohl

  • 1Division of Rheumatology, Department of Pediatrics, University of Toronto and Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, Toronto, Canada.

Handbook of Clinical Neurology
|April 30, 2013
PubMed

Insights

Childhood primary angiitis of the CNS (cPACNS) is a serious condition requiring prompt diagnosis. Early recognition and treatment of this rare neurological disorder significantly improve patient outcomes.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Vascular Neurology

Background:

  • Childhood CNS vasculitis is a rare neurological disorder.
  • It can be primary (cPACNS) or secondary to systemic diseases.
  • Early diagnosis and treatment are crucial for better outcomes.

Purpose of the Study:

  • To highlight the diagnostic challenges and key modalities for childhood CNS vasculitis.
  • To emphasize the importance of timely intervention in cPACNS.
  • To provide a comprehensive overview of diagnostic evaluation.

Main Methods:

  • Review of clinical presentation, diagnostic imaging (MRI, angiography), and biopsy.
  • Discussion of laboratory markers and CSF analysis.
  • Consideration of secondary causes and mimics of CNS vasculitis.

Main Results:

  • Clinical presentation is highly variable, including neurological and psychiatric symptoms.
  • MRI is sensitive but not specific; angiography is key for large/medium vessel cPACNS.
  • Brain biopsy is necessary for small vessel cPACNS with normal angiography.

Conclusions:

  • Timely diagnosis of cPACNS is essential due to variable presentation and diagnostic limitations.
  • A thorough evaluation including imaging, biopsy, and exclusion of mimics is required.
  • Early recognition and treatment can significantly alter the course of this devastating disease.

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