title: 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: Department of Rheumatology, Children's Hospital Los Angeles, California, USA.
authors: K T
authors: KT
authors: Gallagher
authors: B
authors: B
authors: Shaham
authors: A
authors: A
authors: Reiff
authors: A
authors: A
authors: Tournay
authors: J P
authors: JP
authors: Villablanca
authors: J
authors: J
authors: Curran
authors: M D
authors: MD
authors: Nelson
authors: B
authors: B
authors: Bernstein
authors: D J
authors: DJ
authors: Rawlings
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D018501:Antirheumatic Agents / Q000008:administration & dosage*; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008881:Migraine Disorders / Q000209:etiology; D020521:Stroke / Q000209:etiology; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D000305:Adrenal Cortex Hormones; D018501:Antirheumatic Agents; D003520:Cyclophosphamide