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Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
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Related Experiment Video

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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
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Summary

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.

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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.