A case report: primary CNS angiitis
Keywords:Primary angiitis of CNS (PACNS), stroke, Vasculitis, Methylprednisolone
The primary angiitis of the central nervous system (PACNS) is an entity with a very low incidence and prevalence. It affects small and medium sized arteries of the brain parenchyma, spinal cord and leptomeniges resulting in CNS dysfunction. It is defined by inflammation of the cerebral vasculature without angiitis of other organ. Its clinical manifestations are very heterogeneous and make clinical diagnosis difficult. In most cases, a brain biopsy is required. Only the clinical suspicion and the ability to recognize the possible clinical and imagenological patterns of presentation make an accurate diagnosis possible.
We hereby report a case of Primary angiitis of CNS in 35 year old right handed male who presented with ischemic stroke with Left Hemiplegia. The diagnosis of PACNS was made after ruling out most of the causes of secondary CNS angiitis.
Younger DS. Vasculitis of the nervous system. Curr Opin Neurol. 2004;17:317.
Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Annals of Neurology. 2007;62:442–51.
Alba NA, Espígol-Frigolé G, Sergio Prieto-González S, Garcia-Martinez ITA, Butjosa M, amd Mariaccid JHR. Central Nervous System Vasculitis: Still More Questions than Answers. Current Neuropharmacol. 2011;9(3):437-48.
Miller DV, Salvarani C, Hunder GG, Brown RD, Parisi JE, Christianson TJ. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol. 2009;33(1):35-43.
Bonilla-Abadía NCF, Cortes OA, Izquierdo JH, Shinchi AM, Bravo JC, Tobón GJ, et al. Primary Angiitis of the Central Nervous System: A Report of Three Cases from a single Colombian Center. Case Reports in Neurological Medicine. 2013 (2013), Article ID 940438, 4 pageshttp://dx.doi.org/10.1155/2013/940438
Parisi JE, Moore PM. The role of biopsy in vasculitis of the central nervous system. Seminar Neurol. 1994;14:341-8.
Calabrese LH, Mallek JA. Primartangitis of central nervous system report of the 8 new Cases review of the literature & proposal for diagnostic criteria. Medicine (Baltimore). 1988;67:20.
Amara AW, Bashir K, Palmer CA, Walker HC. Challenges in diagnosis of isolated central nervous system vasculitis. Brain and Behavior. 2011;1:57–61.