Primary angiitis of CNS

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Primary angiitis of the central nervous system.” (Rev Neurol. 2007 Feb 16-28;44(4):209-11). Authors are Jordao MJ, Almeida F, Moreira da Costa et al., from the Hospital Sao Marcos, Braga, Portugal. Primary angiitis of the central nervous system (CNS) is a rare disease. Clinical signs and symptoms include headache and cognitive disorders associated to multifocal neurological deficits. A definitive diagnosis can only be achieved by means of a cerebromeningeal biopsy. The authors describe the case of a 15-year-old male who first reported lower back pain and progressive paresis of the right lower limb, later followed by laterocollis on the right side. Primary angiitis of the CNS is an infrequent disease and its pathogenesis remains unknown. The definitive diagnosis of these patients is histological. It courses spontaneously and generally has a fatal outcome. Treatment, which consists in an association of cyclophosphamide and prednisone, must be started as early as possible. To access the full abstract of the article, click here.