Cerebrospinal fluid and therapy of isolated angiitis of the central nervous system.

Abstract

Serial cerebral angiograms, computed tomography, and magnetic resonance imaging are among the proposed methods for monitoring disease activity and response to therapy in isolated angiitis of the central nervous system. Cerebrospinal fluid has not proved to be useful in monitoring clinical course. We describe a 45-year-old man with histological diagnosis of isolated angiitis of the central nervous system that was treated with prednisone plus azathioprine and monitored for 2 years. Samples of the cerebrospinal fluid were obtained for cytological and routine chemical examination, as well as albumin and immunoglobulin content. Before treatment, cerebrospinal fluid showed marked plasmatic transudation of albumin and intrathecal synthesis of immunoglobulins. During the first year of immunosuppression no events were noticed, and the previously abnormal aspects of the cerebrospinal fluid showed improvement. During the weaning of azathioprine, a new stroke occurred in conjunction with a marked deterioration of cerebrospinal fluid parameters. Immunosuppression was resumed at previous levels, and during the following year no further events occurred. Once again, abnormal cerebrospinal fluid values improved significantly. We report a case of isolated angiitis of the central nervous system in which the serial cerebrospinal fluid examinations (albumin and immunoglobulin content) showed a close correlation with clinical course. This method may be useful in monitoring response to therapy.

Primary Angiitis of the Central Nervous System: A Misinterpreted Clinical Onset of CNS Vasculitis

“…Several examinations are necessary to rule out neurological conditions that may mimic PACNS [2]. Serological tests are generally normal; however, abnormalities such as antinuclear antibodies or intrathecal oligoclonal IgG bands in our patient do not exclude PACNS diagnosis [2,7]. Similarly, MRI may be abnormal in more than 80% of PACNS patients, but this is not specific [2,8,9].…”

Primary central nervous system vasculitis: clinical experiences with 21 new European cases

“…The ideal diagnostic brain biopsy is a 1 cm 3 brain tissue with both gray and white matter as well as leptomeninges and preferably a cortical vessel [29,42,46]. The risk of false-negative brain biopsy is not small and can be explained by the segmental involvement of vessels and a possible mismatch between radiological abnormalities and histological predominant lesions [32,46]. It is consensus that open biopsy lessens chances of a false-negative result compared with stereotactic needle biopsy [42,46].…”

“…The major importance of CSF examinations also lies in excluding alterative diagnoses [42]. As usually in PCNSV, the typically -but unfortunately-nonspecific finding was mild lymphocytic pleocytosis and elevated protein [32,42]. In the recent published Chinese series of 8 cases the majority even of biopsy-approved patients presented with normal CSF results [5,45].…”

“…To our experience, the activity of the disease under treatment is appreciable only by combined clinical, CSF and neuroradiological monitoring. Drop of CSF abnorma lities is reported corresponds with clinical improvement [32]. The successful use of serial conventionally angiography has been reported [2], but in clinical practise also MRI combined with color duplex sonography studies is increasingly used for follow-up [30,34].…”

Primary isolated vasculitis of central nervous system (part 1)

“…Литературные данные об из менении лабораторных показателей при ИВ приведены в таблице. Для проведения контроля за эффективностью ле чения рекомендуется повторное исследование альбуминов и иммуноглобулинов спинномозговой жидкости (СМЖ) [31], анализ антинейтрофильных цитоплазматических ан тител (АНЦА), сывороточных цитокинов, антител к эндотелиальным антигенам [18].…”