Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease.
Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.
Benseler
Susanne M
SM
The Hospital for Sick Children, Toronto, Ontario, Canada.
deVeber
Gabrielle
G
Hawkins
Cynthia
C
Schneider
Rayfel
R
Tyrrell
Pascal N
PN
Aviv
Richard I
RI
Armstrong
Derek
D
Laxer
Ronald M
RM
Silverman
Earl D
ED
D000293:Adolescent; D001241:Aspirin / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D001921:Brain / Q000473:pathology*; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000188:drug therapy
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine; D001241:Aspirin; D011241:Prednisone