PT  - JOURNAL ARTICLE
AU  - K T Gallagher
AU  - B Shaham
AU  - A Reiff
AU  - A Tournay
AU  - J P Villablanca
AU  - J Curran
AU  - M D Nelson, Jr
AU  - B Bernstein
AU  - D J Rawlings
TI  - Primary angiitis of the central nervous system in children: 5 cases.
DP  - 2001 Mar 01
TA  - The Journal of Rheumatology
PG  - 616--623
VI  - 28
IP  - 3
4099  - http://www.jrheum.org/content/28/3/616.short
4100  - http://www.jrheum.org/content/28/3/616.full
SO  - J Rheumatol2001 Mar 01; 28
AB  - 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.