Conflict of Interest: The authors report no conflict of interest.
Central Nervous System Venulitis Presenting as Migraine
Article first published online: 23 JUL 2013
© 2013 American Headache Society
Headache: The Journal of Head and Face Pain
Volume 54, Issue 3, pages 541–544, March 2014
How to Cite
Orr, S. L., dos Santos, M. P., Jurencak, R., Michaud, J., Miller, E. and Doja, A. (2014), Central Nervous System Venulitis Presenting as Migraine. Headache: The Journal of Head and Face Pain, 54: 541–544. doi: 10.1111/head.12188
Funding: This case report has no sponsors, industry or otherwise.
- Issue published online: 4 MAR 2014
- Article first published online: 23 JUL 2013
- Manuscript Accepted: 4 JUN 2013
- central nervous system vasculitis;
- central nervous system venulitis;
- primary angiitis of the central nervous system
To describe a case of pediatric central nervous system (CNS) venulitis.
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.
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.
We describe a case of pediatric CNS venulitis presenting with migraine.