Central Nervous System Vasculitis: Still More Questions than Answers

Abstract

The central nervous system (CNS) may be involved by a variety of inflammatory diseases of blood vessels. These include primary angiitis of the central nervous system (PACNS), a rare disorder specifically targeting the CNS vasculature, and the systemic vasculitides which may affect the CNS among other organs and systems. Both situations are severe and convey a guarded prognosis. PACNS usually presents with headache and cognitive impairment. Focal symptoms are infrequent at disease onset but are common in more advanced stages. The diagnosis of PACNS is difficult because, although magnetic resonance imaging is almost invariably abnormal, findings are non specific. Angiography has limited sensitivity and specificity. Brain and leptomeningeal biopsy may provide a definitive diagnosis when disclosing blood vessel inflammation and are also useful to exclude other conditions presenting with similar findings. However, since lesions are segmental, a normal biopsy does not completely exclude PACNS. Secondary CNS involvement by systemic vasculitis occurs in less than one fifth of patients but may be devastating. A prompt recognition and aggressive treatment is crucial to avoid permanent damage and dysfunction. Glucocorticoids and cyclophosphamide are recommended for patients with PACNS and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis. CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids (giant-cell arteritis) or glucocorticoids and immunosuppressive agents (Takayasu’s disease). However, in large vessel vasculitis, where CNS symptoms are usually due to involvement of extracranial arteries (Takayasu’s disease) or proximal portions of intracranial arteries (giant-cell arteritis), revascularization procedures may also have an important role.

Development of intracerebral hemorrhage in the short-term clinical course of a patient with microscopic polyangiitis without neurological symptoms at diagnosis: an autopsy case

“…We suspect that there is no relationship between the presence of cerebral vasculitis and the severity of other organ symptom. This is because in our case and in other reported cases, the severity of MPA before developing cerebral hemorrhage varied [3][4][5][6][7][8][9][10][11].…”

Granulomatosis with polyangiitis

“…6 A prompt recognition and aggressive treatment is crucial to avoid paramount damage and dysfunction. Despite lack of specific findings in MRI of brain, with the typical cavitary lung lesions and upper airway involvement along with positive c-ANCA, cerebral vasculitis was the likeliest explanation for Parkinsonism in this patient.…”

Primary Central Nervous System Vasculitis with Negative Brain Biopsy: Case Report

“…1,4 Kırk altı hastayı içeren bir PSSV çalışmasında beyin biyopsisinin duyarlılığı %63 olarak saptanmıştır. Bu seride histopatolojik morfolojik paterne bakıldığında; akut nekrotizan form (%14), saf lenfositik form (%28) ve granülo-matöz form (%58) olarak saptanmıştır.…”

“…Buna rağmen, doğru tanı konmasının güç olduğu durumda risk-yarar durumu açı-sından bakıldığında anjiyografi tanısal işlem olarak yapılması gereken bir incelemedir. 2,4,7,8 Beyin biyopsisi kesin tanı için gereklidir. Ancak biyopsi %25-50 hastada negatif bulunmakta …”