Primary Angiitis of the Central Nervous System in Children: 5 Cases

KERRY T. GALLAGHER, BRACHA SHAHAM, ANDREAS REIFF, ANNE TOURNAY, J. PABLO VILLABLANCA, JOHN CURRAN, MARVIN D. NELSON Jr, BRAM BERNSTEIN, and DAVID J. RAWLINGS

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. (J Rheumatol 2001;28:616-23)

Key Indexing Terms:

CENTRAL NERVOUS SYSTEM VASCULITIS
STROKE
JUVENILE MIGRAINE<
COMPUTER TOMOGRAPHIC ANGIOGRAPHY
CYCLOPHOSPHAMIDE