Childhood primary angiitis of the central nervous system
- Marinka Twilt, MD, PhD
Marinka Twilt, MD, PhD
- Assistant Professor of Pediatrics
- University of Calgary, Alberta
- Susanne Benseler, MD, PhD
Susanne Benseler, MD, PhD
- Associate Professor of Pediatrics
- University of Calgary
- Calgary, Alberta, Canada
- Section Editors
- Robert Sundel, MD
Robert Sundel, MD
- Section Editor — Pediatric Rheumatology
- Associate Professor of Pediatrics
- Harvard Medical School
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
Childhood primary angiitis of the central nervous system (cPACNS) is a rare form of central nervous system (CNS) vasculitis or inflammatory brain disease affecting children. It typically presents with neurologic deficits, often a stroke-like event, and/or psychiatric symptoms. The diagnostic evaluation focuses on ruling out other forms of inflammatory brain disease and noninflammatory vasculopathies in addition to performing confirmatory studies, such as brain magnetic resonance imaging (MRI) and angiography. Treatment includes anticoagulation and/or antiinflammatory therapy to prevent disease progression and complications.
A general overview of childhood vasculitis is discussed in detail separately, as is primary angiitis of the central nervous system (PACNS) in adults. (See "Vasculitis in children: Classification and incidence" and "Vasculitis in children: Evaluation" and "Vasculitis in children: Management overview" and "Primary angiitis of the central nervous system in adults".)
cPACNS criteria are based upon the modified Calabrese criteria for adult PACNS and include [1-3]:
●Newly acquired, otherwise unexplained neurologic or psychiatric symptom in a patient less than 18 years of age
●Angiographic and/or histopathologic features of central nervous system (CNS) angiitis
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- CLINICAL FEATURES
- Angiography-positive, nonprogressive cPACNS
- Angiography-positive, progressive cPACNS
- Angiography-negative, brain biopsy-positive, small-vessel cPACNS
- DIFFERENTIAL DIAGNOSIS
- Psychosis and behavioral complications