The recognition, diagnosis and management of cerebral vasculitis: a European survey

Abstract

We have completed a survey of European neurological practice concerning cerebral vasculitis. Twenty-nine respondents from 15 countries provided information concerning the diagnosis and management. The results confirmed the anticipated low frequency of the disease, but also illustrated the power of any putative collaborative effort. Interestingly, there was a wide variation in clinical practice, in particular concerning the perceived importance of cerebral angiography as a diagnostic test and the very common use of steroids as first-line treatment, rather than more potent immunosuppressive agents. This variation is probably to be explained at least, in part, by the absence of any firm evidence base to inform clinical practice. A European collaborative effort -in which there has emerged considerable interest -offers a realistic opportunity to generate sound clinical evidence and thence scientifically robust practical guidelines.

Primary Angiitis of the Central Nervous System Mimicking Tumor-Like Lesion -Case Report-

“…3,7,11,[14][15][16][17]19) Our patient did not receive steroid or immunosuppressive therapies, yet he did not present with any new clinical symptoms or new lesions on MR imaging in the 6 months after surgery. We did not administer steroid therapy even after the small intracerebral hemorrhage occurred, because the diagnosis of PACNS had not been established at that time.…”

“…15,16) The etiology and pathogenesis of PACNS remain unclear. PACNS is characterized by inflammation of the intracranial small and medium arteries without systemic disease.…”

Primary angiitis of the central nervous system: Report of eight cases from a single Italian center

“…However, in the presence of a "constellation" of clinical and neuroradiological signs fitting for vasculitis, we consider that positive angiographic findings strongly support diagnosis [20,24,30,31]. In these selected cases our diagnostic iter does not proceed to a biopsy.…”

Multifocal Hemorrhagic Vasculopathy: Possibly a Manifestation of Central Nervous System Vasculitis

“…Although no prospective placebo-controlled trial of immunosuppressive treatment in CNS vasculitis is currently available, large retrospective series of patients with Wegener's granulomatosis and microscopic polyangiitis provide clear support for their use. 20 A Japanese case study reported a 58-year-old woman presenting with sudden headache who had a frontal lobe subcortical hemorrhage that was evaluated only by serial MR angiography. The findings were felt to be consistent with isolated angiitis of the CNS, and corticosteroid treatment was initiated, with resolution of symptoms and favorable outcome on a 7-month follow-up.…”