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Case Reports
. 2011:2011:495201.
doi: 10.1155/2011/495201. Epub 2011 Jul 14.

Isolated central nervous system vasculitis associated with antiribonuclear protein antibody

Affiliations
Case Reports

Isolated central nervous system vasculitis associated with antiribonuclear protein antibody

Amer M Awad et al. Case Rep Neurol Med. 2011.

Abstract

We describe the case of a young woman who was referred to a tertiary care center with unexplained subacute progressive encephalopathy preceded by long-standing severe headaches. Her extensive workup was remarkable for abnormal intracranial angiography suggestive of small- and medium-vessel vasculitis, persistently elevated protein in the cerebrospinal fluid and persistently high titers of antiribonuclear protein antibody. The patient showed a modest response to intravenous high-dose steroids. We propose that the patient's neurologic disease is secondary to immune-mediated central nervous system vasculitis, possibly as an initial manifestation of mixed connective tissue disease.

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Figures

Figure 1
Figure 1
(a) Three mm axial fluid attenuated inversion recovery (FLAIR) brain magnetic resonance images (MRIs) demonstrating extensive subcortical white matter hyperintensity. (b) Magnetic resonance angiogram (MRA) demonstrating beading of small and medium intracranial vessels.

References

    1. Matsui H, Udaka F, Oda M, Kubori T, Nishinaka K, Kameyama M. Encephalopathy and severe neuropathy due to probable systemic vasculitis as an initial manifestation of mixed connective tissue disease. Neurology India. 2006;54(1):83–85. - PubMed
    1. Prystowsky SD. Mixed connective tissue disease. Western Journal of Medicine. 1980;132(4):288–293. - PMC - PubMed
    1. Nadeau SE. Neurologic manifestations of connective tissue disease. Neurologic Clinics. 2002;20(1):151–178. - PubMed
    1. Salmhofer W, Hermann J, Joch M, Kerl H, Graninger W. High serum levels of antibodies against the recombinant 70 kDa ribonucleoprotein are useful for diagnosing mixed connective tissue disease. Journal of the European Academy of Dermatology and Venereology. 2007;21(8):1047–1053. - PubMed
    1. Alarcon-Segovia D, Villarreal M. Classification and diagnostic criteria for mixed connective tissue disease. In: Kasukawa R, Sharp GC, editors. Mixed Connective Tissue Diseases and Antinuclear Antibodies. Amsterdam, The Netherlands: Elsevier; 1987. pp. 33–40.

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