Primary angiitis of the central nervous system in children: 5 cases.

Abstract

We describe 5 children who meet criteria for primary angiitis of the central nervous system (PACNS). All patients presented with headache and/or focal neurologic deficits and exhibited clinical and/or radiographic evidence of disease progression. Two patients had disease progression prior to combined treatment with cyclophosphamide and corticosteroids; one progressed while receiving intravenous cyclophosphamide and stabilized after a change to daily oral dosing; one progressed after discontinuing therapy after less than 12 months and improved after retreatment; and one progressed on steroid therapy alone but was lost to followup. Children who have frequent or severe headaches or focal neurologic deficits should be carefully evaluated and those meeting criteria for PACNS should be treated aggressively.

• Authors: K T Gallagher, B Shaham, A Reiff, A Tournay, J P Villablanca, J Curran, M D Nelson Jr, B Bernstein, D J Rawlings
• Journal: The Journal of rheumatology (J Rheumatol) Vol. 28 Issue 3 Pg. 616-23 (Mar 2001) ISSN: 0315-162X [Print] Canada
• PMID: 11296969 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Adrenal Cortex Hormones
• Antirheumatic Agents
• Cyclophosphamide

Topics

• Adrenal Cortex Hormones (administration & dosage)
• Antirheumatic Agents (administration & dosage)
• Cerebral Angiography
• Child
• Child, Preschool
• Cyclophosphamide (administration & dosage)
• Drug Therapy, Combination
• Female
• Humans
• Magnetic Resonance Imaging
• Male
• Migraine Disorders (etiology)
• Stroke (etiology)
• Vasculitis, Central Nervous System (complications, diagnosis, drug therapy)