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 Printable version |
Primary angiitis of the central nervous system |
Médecine thérapeutique / Pédiatrie. Volume 14, Number 5-6, 387-95, Septembre-Décembre 2011, Dossier
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 Résumé
Article gratuit
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Author(s) : Jean-Louis Stéphan, Stéphane Darteyre, Dominique Allard, Stéphane Chabrier |
Summary : Primary angiitis of the central nervous system (PACNS) is an idiopathic vasculitis confined to the central nervous system. This review summarizes the recent data on diagnosis, classification, treatment, and outcomes in cPACNS. Diagnosis is often challenging. Angiography is a key diagnostic modality that can aid in differentiating between large and medium vessel disease, and small vessel disease. Small vessel involvement is characterized clinically by progressive neurological symptoms, multifocal lesions on brain imaging, occasional pseudo-tumor presentation, and normal angiogram results in most patients. Confirmation of small vessel cPACNS requires brain biopsy. A thorough diagnostic evaluation for large and medium sized cerebral vessel vasculites includes a workup for prothrombotic diseases, exclusion of infection and arterial dissection. Early diagnosis and aggressive treatment with immunosuppressants may improve neurological outcomes and lower mortality rates in this potentially devastating disease. |
Keywords : primary angiitis of the central nervous system, cerebral biopsy, angiography |
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