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Childhood Cerebral Vasculitis : A Multidisciplinary Approach
Neetika Gupta1,2, Shivaprakash B Hiremath3,4, Richard I Aviv3
1Department of Medical Imaging, Children's Hospital of Eastern Ontario (CHEO), University of Ottawa, Ottawa, Ontario, Canada. drneetikagupta@gmail.com.
Insights
Childhood cerebral vasculitis (CCV) is a common cause of pediatric stroke, presenting with diverse neurological symptoms. This review details CCV diagnosis, emphasizing imaging and clinical findings for effective management.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Cerebral vasculitis is a significant cause of arterial stroke in children.
- It presents with varied neurological symptoms, including focal deficits, seizures, and encephalopathy.
- The multifactorial etiopathogenesis of childhood cerebral vasculitis (CCV) complicates classification.
Purpose of the Study:
- To review common and uncommon forms of CCV.
- To discuss the etiopathogenesis, diagnostic imaging, and advanced techniques for CCV.
- To highlight clinical, laboratory, and genetic findings crucial for diagnosis.
Main Methods:
- Comprehensive review of literature on CCV.
- Discussion of diagnostic roles of MRI, MR angiography, and vessel wall imaging.
- Emphasis on clinical manifestations, CSF, and blood results.
- Evaluation of brain and leptomeningeal biopsy utility.
Main Results:
- A stepwise diagnostic approach based on clinicoradiological findings is proposed.
- Magnetic resonance imaging (MRI) and related angiography techniques are primary imaging tools.
- Catheter angiography serves as a problem-solving tool.
- Biopsy is essential for definitive diagnosis and excluding mimics.
Conclusions:
- Accurate diagnosis of CCV requires integrating clinical, laboratory, genetic, and imaging data.
- A structured diagnostic approach aids in identifying CCV and differentiating it from other conditions.
- Advanced imaging and biopsy are key to managing childhood cerebral vasculitis effectively.
Abstract:
Cerebral vasculitis is increasingly recognized as a common cause of pediatric arterial stroke. It can present with focal neurological deficits, psychiatric manifestations, seizures, and encephalopathy. The etiopathogenesis of childhood cerebral vasculitis (CCV) is multifactorial, making an inclusive classification challenging. In this review, we describe the common and uncommon CCV with a comprehensive discussion of etiopathogenesis, the role of various imaging modalities, and advanced techniques in diagnosing CCV. We also highlight the implications of relevant clinical, laboratory, and genetic findings to reach the final diagnosis. Based on the clinicoradiological findings, a stepwise diagnostic approach is proposed to facilitate CCV diagnosis and rule out potential mimics. Identification of key clinical manifestations, pertinent blood and cerebrospinal fluid results, and evaluation of central nervous system vessels for common and disease-specific findings will be emphasized. We discuss the role of magnetic resonance imaging, MR angiography, and vessel wall imaging as the imaging investigation of choice, and reservation of catheter angiography as a problem-solving tool. We emphasize the utility of brain and leptomeningeal biopsy for diagnosis and exclusion of imitators and masqueraders.
