RT Journal Article
SR Electronic
T1 Primary angiitis of the central nervous system in children: 5 cases.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 616
OP 623
VO 28
IS 3
A1 K T Gallagher
A1 B Shaham
A1 A Reiff
A1 A Tournay
A1 J P Villablanca
A1 J Curran
A1 M D Nelson, Jr
A1 B Bernstein
A1 D J Rawlings
YR 2001
UL http://www.jrheum.org/content/28/3/616.abstract
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.