Pediatric wegener’s granulomatosis complicated by central nervous system vasculitis

An unusual case of central nervous system vasculitis in pediatric Wegener’s granulomatosis, a rare disorder that infrequently presents during childhood, is reported. A 13-year-old girl with Wegener’s granulomatosis, whose initial presentation resembled Henoch-Schönlein purpura, developed recurring s...

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Bibliographic Details
Published in:Pediatric neurology 1998-10, Vol.19 (4), p.317-319
Main Authors: von Scheven, Emily, Lee, Christine, Berg, Bruce O
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
Brain - pathology
Cerebrovascular Disorders - complications
Cerebrovascular Disorders - diagnosis
Diagnosis, Differential
Female
Granulomatosis with Polyangiitis - complications
Granulomatosis with Polyangiitis - diagnosis
Granulomatosis with Polyangiitis - pathology
Humans
Kidney - pathology
Magnetic Resonance Imaging
Medical sciences
Purpura, Schoenlein-Henoch - diagnosis
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Seizures - diagnosis
Seizures - etiology
Vasculitis - complications
Vasculitis - diagnosis
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Summary:An unusual case of central nervous system vasculitis in pediatric Wegener’s granulomatosis, a rare disorder that infrequently presents during childhood, is reported. A 13-year-old girl with Wegener’s granulomatosis, whose initial presentation resembled Henoch-Schönlein purpura, developed recurring seizures. MRI of the brain demonstrated multiple areas of increased signal in the occipital, parietal, and frontal lobes, consistent with central nervous system vasculitis. Although both peripheral and cranial neuropathies have been reported in patients with Wegener’s granulomatosis, cerebral vasculitis is unusual, particularly in childhood. This case emphasizes the need to consider Wegener’s granulomatosis in the differential diagnosis of both unexplained seizures and central nervous system vasculitis in children with systemic illness.
ISSN:0887-8994
1873-5150
DOI:10.1016/S0887-8994(98)00071-X