Systemic and central nervous system vasculitides

Abstract

The vasculitides are a collection of diseases sharing the central feature of tissue injury due to inflammation of blood vessels. Although some occur frequently, many are encountered uncommonly or rarely. Nonetheless, because peripheral or central nervous system abnormalities may be a prominent part of these disorders, neurologists should know or be able to refer to studies on the classification, pathophysiology, treatments, and long-term complications of vasculitis. Two central issues that confront us now are 1) improving the accuracy of diagnosis, including identifying any underlying infectious causes, and 2) limiting the long-term damage from both the disease and its therapies.

Vasculitis of the nervous system

“…However, it has also been used with success as the first line therapy in PACNS [63]. The use of antiplatelet agents has been recommended by some as additional maintenance therapy [90].…”

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

“…The diagnosis of PACNS was supported by angiography, an examination more sensitive than MRI [9]. As recommended, our patient received high doses of steroid [1][2][3], with combined antiplatelet therapy [10]. During the follow-up, the clinical course improved dramatically and MRA, DSA and Doppler ultrasound confirmed the complete lysis of the parietal thrombus on the right ICA.…”

“…The gold standard for diagnosis is the histological analysis; however, in most cases, the diagnosis can be hypothesised after the exclusion of disorders mimicking clinical, serological and imaging findings associated with systemic or CNS vascular diseases [1]. Two patterns of the disease with different outcomes have been defined: (a) PACNS, being more frequent in males at any age [1]; high-dose intravenous steroid is the drug of choice [2,3] and (b) benign angiopathy, frequently affecting young women with a history of headaches, nicotine or caffeine abuse or oestrogen intake [1,4]. Calcium channel blockers with steroids are the drug of choice [2,3].…”

“…Two patterns of the disease with different outcomes have been defined: (a) PACNS, being more frequent in males at any age [1]; high-dose intravenous steroid is the drug of choice [2,3] and (b) benign angiopathy, frequently affecting young women with a history of headaches, nicotine or caffeine abuse or oestrogen intake [1,4]. Calcium channel blockers with steroids are the drug of choice [2,3].…”

“…Although MS is defined clinically, MRI is the strongest paraclinical method to establish the diagnosis [3,4] and serves as a disease monitor during the course of MS, both in studies (as a surrogate outcome marker) and practise (for therapeutic decisions). MRI is far more sensitive in detecting new lesions than clinical signs and symptoms [3].…”