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An unusual central nervous system involvement in rheumatoid arthritis: combination of pachymeningitis and cerebral vasculitis

Identifieur interne : 001A28 ( Main/Exploration ); précédent : 001A27; suivant : 001A29

An unusual central nervous system involvement in rheumatoid arthritis: combination of pachymeningitis and cerebral vasculitis

Auteurs : Asl Kurne [Turquie] ; Rana Karabudak [Turquie] ; Ömer Karadag [Turquie] ; Gül Yalcin-Cakmakli [Turquie] ; Kader Karli-Oguz [Turquie] ; Kivilcim Yavuz [Turquie] ; Meral Calgüneri [Turquie] ; Mehmet Akif Topcuoglu [Turquie]

Source :

RBID : ISTEX:A9488DAA3AE9ECB414E0780936BB21581E6A2820

English descriptors

Abstract

Abstract: Severe primary central nervous system (CNS) involvement such as vasculitis and pachymeningitis can rarely occur in rheumatoid arthritis (RA) even in the absence of systemic disease activation. The authors illustrate a female patient with well-controlled RA who presented with headaches, encephalopathy, seizures and relapsing focal neurological deficits. Primary rheumatoid cerebral vasculitis and pachymeningitis were diagnosed based on suggestive brain magnetic resonance (MR) imaging, MR angiography, cerebrospinal fluid analysis and cerebral angiography. MR showed abnormal leptomeningeal enhancement and hyperintense FLAIR signal in the cortical subarachnoid spaces consistent with pachymeningitis. Cerebral angiography findings were consistent with vasculitis. Aggressive treatment resulted in significant clinicoradiological resolution. Cerebral vasculitis is a rare but certain manifestation of RA. This complication can be diagnosed in the presence of suggestive angiographic and CSF findings. The condition may be steroid resistant, and needs to be treated more aggressively.

Url:
DOI: 10.1007/s00296-008-0810-6


Affiliations:


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<div type="abstract" xml:lang="en">Abstract: Severe primary central nervous system (CNS) involvement such as vasculitis and pachymeningitis can rarely occur in rheumatoid arthritis (RA) even in the absence of systemic disease activation. The authors illustrate a female patient with well-controlled RA who presented with headaches, encephalopathy, seizures and relapsing focal neurological deficits. Primary rheumatoid cerebral vasculitis and pachymeningitis were diagnosed based on suggestive brain magnetic resonance (MR) imaging, MR angiography, cerebrospinal fluid analysis and cerebral angiography. MR showed abnormal leptomeningeal enhancement and hyperintense FLAIR signal in the cortical subarachnoid spaces consistent with pachymeningitis. Cerebral angiography findings were consistent with vasculitis. Aggressive treatment resulted in significant clinicoradiological resolution. Cerebral vasculitis is a rare but certain manifestation of RA. This complication can be diagnosed in the presence of suggestive angiographic and CSF findings. The condition may be steroid resistant, and needs to be treated more aggressively.</div>
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