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Updated: May 9, 2026

3D-Neuronavigation In Vivo Through a Patient's Brain During a Spontaneous Migraine Headache
Published on: June 2, 2014
Central nervous system venulitis presenting as migraine
Serena L Orr1, Marlise P Dos Santos, Roman Jurencak
1Children's Hospital of Eastern Ontario, ON, Canada; University of Ottawa, ON, Canada.
Insights
This case study highlights pediatric central nervous system (CNS) venulitis, a rare condition presenting as severe migraines and hemiplegia. Early diagnosis and treatment with immunosuppressants led to significant clinical improvement in a young patient.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Background:
- Primary angiitis of the CNS is a rare cause of pediatric morbidity, typically affecting arteries.
- Isolated central nervous system (CNS) venulitis in pediatric patients is exceptionally rare, with limited documented cases.
Observation:
- A 17-year-old female presented with worsening migraines and right-sided hemiplegia.
- Brain MRI revealed multiple rim-enhancing lesions suggestive of calcifications in the deep white matter.
- Brain biopsy confirmed an inflammatory process affecting small veins and venules.
Findings:
- The patient's symptoms, including migraines and hemiplegia, were attributed to pediatric CNS venulitis.
- Diagnostic workups for infectious, hematologic, and rheumatologic causes were negative.
- Histopathological examination of brain biopsy revealed venulitis.
Implications:
- This case expands the understanding of CNS venulitis presentation in pediatric populations.
- Prompt immunosuppressive therapy, including steroids and cyclophosphamide, can effectively manage pediatric CNS venulitis.
- Migraine presentation should prompt consideration of CNS venulitis in pediatric differential diagnoses.
Objective:
To describe a case of pediatric central nervous system (CNS) venulitis.
Background:
Primary angiitis of the CNS is a rare but increasingly well-recognized cause of morbidity in children. It primarily involves the arteries and arterioles of the CNS, with only 1 published case of a pediatric patient found to have isolated CNS venulitis on brain biopsy.
Case Report:
A 17-year-old female with a 4-year history of migraines presented with increasingly frequent migraines and right-sided hemiplegia. Infectious, hematologic, and rheumatologic work-ups were negative. Brain magnetic resonance imaging showed multiple rim-enhancing lesions consistent with calcifications affecting the deep left white matter. On brain biopsy, there was evidence of an inflammatory process involving small veins and venules. The patient displayed clinical improvement with a course of high-dose steroids and 6 monthly cyclophosphamide infusions followed by maintenance therapy with mycophenolate mofetil.
Discussion:
We describe a case of pediatric CNS venulitis presenting with migraine.
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