Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy
Open Access
- 13 August 2008
- journal article
- research article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 47 (11), 1671-1677
- https://doi.org/10.1093/rheumatology/ken328
Abstract
Objectives. To describe the clinical features and outcomes of patients with primary central nervous system vasculitis (PCNSV) and cerebral amyloid angiopathy (CAA) from a large cohort of consecutive patients with PCNSV treated at a single institution. Methods. We identified 101 consecutive patients with PCNSV admitted between January 1983 and December 2003. PCNSV diagnoses were based on findings from a central nervous system (CNS) biopsy (n = 31) and conventional angiography (n = 70). CNS tissue specimens from 49 cases were examined histologically, and 49 were stained for amyloid deposits. Those with vascular amyloid deposits (CAA) were compared with those without histological evidence of amyloid deposition. Results. Eight cases (26%) with CNS biopsy specimens positive for PCNSV also showed findings of CAA. Compared with patients with PCNSV only, these patients were older at diagnosis, predominantly male, had a more acute onset, a higher frequency of cognitive dysfunction and showed prominent gadolinium-enhanced leptomeningeal lesions with MRI. Histologically, all had a granulomatous vascular inflammatory pattern. Six patients responded promptly to therapy. Outcomes at last follow-up were similar in the two groups. Conclusions. PCNSV with CAA appears to form a clinical subset of PCNSV. The vasculitis influences the clinical findings to a greater degree than the presence of amyloid deposits in the vessels.This publication has 21 references indexed in Scilit:
- Primary central nervous system vasculitis: analysis of 101 patientsAnnals of Neurology, 2007
- Amyloid Angiopathy–Related Vascular Cognitive ImpairmentStroke, 2004
- Cerebral amyloid angiopathyNeurology, 1998
- Diagnosis of Cerebral Amyloid AngiopathyStroke, 1997
- Vasculitis in the central nervous systemArthritis & Rheumatism, 1997
- The clinical spectrum of cerebral amyloid angiopathyNeurology, 1993
- Diagnosis and management of isolated angiitis of the central nervous systemNeurology, 1989
- Amyloid angiopathy and granulomatous angiitis of the central nervous system: a case responding to corticosteroid treatmentZeitschrift für Neurologie, 1988
- Primary Angiitis of the Central Nervous SystemMedicine, 1988
- Cerebral amyloid angiopathy. A critical review.Stroke, 1987