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Isolated angiitis of CNS: treatment with pulse cyclophosphamide
T F Barron1, B E Ostrov, R A Zimmerman
1Department of Pediatrics (Neurology and Rheumatology), Pennsylvania State University School of Medicine, Milton S. Hershey Medical Center, Hershey 17033.
Insights
Isolated angiitis of the central nervous system (IACNS) can affect children, presenting as severe headaches. Aggressive immunosuppression, including steroids and cyclophosphamide, can effectively treat this rare condition.
Area of Science:
- Neurology
- Pediatric Neurology
- Vascular Neurology
Background:
- Isolated angiitis of the central nervous system (IACNS) is a rare condition.
- It can manifest in pediatric patients, often presenting with severe headaches.
Observation:
- A 12-year-old boy experienced severe headaches, emesis, and unsteadiness.
- Cerebral angiography revealed segmental narrowing in cerebral arteries, indicative of IACNS.
Findings:
- Initial high-dose steroid therapy provided temporary relief for 6 months.
- Recurrence of symptoms and persistent vascular narrowing necessitated pulse cyclophosphamide therapy.
- Six treatments with cyclophosphamide led to symptom resolution and angiographic normalization.
Implications:
- IACNS, though rare, must be considered in the differential diagnosis of pediatric headaches with neurological deficits.
- Aggressive immunosuppressive therapy, including combination therapy, is crucial for managing pediatric IACNS.
- Prompt diagnosis and treatment can lead to favorable outcomes and prevent long-term sequelae.
Abstract:
A 12-year-old boy with a history of "migraine" headache presented with an increasingly severe headache accompanied by emesis and unsteadiness. Evaluation revealed an acute cerebral hemorrhage with subsequent angiographic studies demonstrating multiple areas of segmental narrowing in both the anterior and posterior cerebral circulations. He was diagnosed with isolated angiitis of the central nervous system and high-dose steroid therapy was administered to which he responded for 6 months. Symptoms recurred and repeat angiography demonstrated persistent segmental narrowing. Pulse cyclophosphamide therapy was begun with resolution of symptoms and normalization of angiography following 6 treatments. Although rare in children, isolated angiitis of the central nervous system can occur in children and aggressive immunosuppression should be considered as the mode of therapy.