Cerebral vasculitis presenting with dementia

John V Bowler MD (Dr. Bowler of the Royal Free Hospital and University College Medical School in London has no relevant financial relationships to disclose.)
Martin R Farlow MD, editor. (Dr. Farlow of Indiana University received research grant support from Accera, Biogen, Eisai,  Eli Lilly, Genentech, Roche, Lundbeck, Chase Pharmaceuticals and Boehringer Ingelheim; honorariums from Eisai, Forest Laboratories, Pfizer, Eli Lilly and Company and Novartis for speaking engagements; and fees from Accera,  Alltech, Avanir,   Biogen, Eisai Med Res, Inc., Eli Lilly and Company, FORUM Pharmaceuticals, Genentech, Inc., Grifols, Helicon, INC Research, Lundbeck, Medavante, Medivation, Merck,  Neurotrope Biosciences, Novartis, Pfizer, Prana, QR Pharm., Riovant Sciences Inc., Roche, Sanofi-Aventis, Schering-Plough, Toyama Pharm and UCB Pharma for consultancy. His spouse was employed by Eli Lilly.)
Originally released June 5, 1995; last updated June 29, 2016; expires June 29, 2019

Overview

Cerebral vasculitis, particularly primary angiitis of the central nervous system, can be of subtle onset, the manifestations protean and the investigations often negative; consequently, the diagnosis can be very difficult to make. In this article, the author reviews the principal features of those vasculitides that may present with cognitive impairment and outlines the extent to which investigations may be falsely negative in primary angiitis of the central nervous system. An illustrative case history demonstrates many of these points. In addition, several other primary vasculitides are outlined.

Key points

 

• Catheter angiography has high false positive and false negative rates in diagnosing primary angiitis of the central nervous system.

 

• As the disease is patchy, biopsy may be falsely negative.

 

• A diagnosis must be made using all available information; it is the overall pattern and not usually just one investigation that secures an accurate diagnosis.

 

• Treatment requires steroids plus a potent immunosuppressant.

Historical note and terminology

Vasculitis rarely affects the central nervous system, as opposed to other organ systems and the peripheral nervous system, because it is an immunologically privileged site. Many arteritides can affect the CNS and ultimately progress to dementia, but the primary presentation of vasculitis as dementia is extremely rare. Dementia usually occurs late in the course of the disease and is preceded by a history of stroke attributable either to the vasculitis directly, to abnormal coagulation, or to cardiac embolism. Other vasculitides may cause a rapid decline in cognition and memory in the presence of altered consciousness; these conditions cannot be said, by definition, to cause dementia. This review does not discuss these and refers only to those conditions wherein the primary presentation may be dementia. Conditions in which dementia or cognitive impairment may occur but in which dementia has not been reported as the primary presenting feature, conditions in which the dementia is preceded by stroke, and conditions that cause an encephalopathic syndrome rather than a dementia are not discussed.

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