Isolated Angiitis/Angiopathy of the Central Nervous System

Abstract

One hundred cases of isolated angiopathy of the central nervous system (CNS) have been described, the majority of whom (71%) were diagnosed histologically. The pathological findings were those of angiitis, most frequently granulomatous angiitis. These patients should be classified as isolated angiitis of the CNS. The minority of patients (29%) were diagnosed according to clinical and angiographic findings which lack sensitivity and specificity. They represent a heterogeneous group, comprising several cases of probable isolated angiitis of the CNS, some clinical and angiographic syndromes associated with acute hypertension, subarachnoid haemorrhage or migraine and other more obscure ''entities'' such as post-partum cerebral angiopathy and isolated benign cerebral angiopathy. In the absence of a histological diagnosis, it is preferable to classify them as isolated angiopathy of the CNS. Although isolated angiitis of the CNS can be considered a discrete, or at least definable, subset of isolated angiopathy of the CNS, it is unclear whether it is a specific disorder or part of a spectrum of disease or even one of a number of reactions to various antigens. The finding of granulomatous angiitis of the CNS (GACNS) in several patients with various systemic disorders suggests that GACNS may be a non-specific pathological reaction. Until the last decade, the prognosis of isolated angiitis of the CNS was extremely poor. Most patients now survive and return to active lives. The major influence on outcome appears to have been the use of combination corticosteroid and cyclophosphamide therapy, although no randomized controlled trials have been undertaken. Before prolonged treatment with such potent medication is prescribed, it recommended that other causes of CNS vasculitis be carefully excluded and the diagnosis of isolated angiitis of the CNS be established histologically by combined leptomeningeal and parenchymal biopsy. The risks of biopsy would not appear to exceed those of untreated isolated angiitis of the CNS. Multicentre collaboration of interested groups should be encouraged if controlled therapeutic trials are to be achieved.

Inflammation and Infection in Clinical Stroke

“…Antiphospholipid antibodies of other specificities such as those to ␤ 2 -glycoprotein 1, phosphatidyl serine, or phosphatidyl inositol may be equally or more important (Tanne et al, 1998). Lossos et al, 1995bSarcoidosis Brown et al, 1989 In addition to these systemic vasculitides and inflammatory conditions, various rare primary CNS vasculitides, including granulomatous primary angiitis of the CNS, have been associated with cerebrovascular syndromes (Hankey, 1991). Isolated angiitis of the CNS with cerebral infarction has also been described in children (Lanthier et al, 2001).…”

Vasculitis as a Cause of First-Ever Stroke

“…Most authors have stated that vasculitis usually manifests as headache, meningeal signs, encephalopathy, psychiatric syndromes, dementia, cranial nerve palsies, and seizures, and only rarely as stroke [1, 13,14,17]. There are also generally non-specific systemic symptoms, such as fever, fatigue and weight loss [19][20][21]25].…”

“…Arterial lesion can lead to stenosis, occlusion, and ischemic infarction [1,13]. The diagnosis of vasculitis is difficult in most cases because of the insidious nature of the disease and the fact that various parts of the circulation can be affected without definitive signs or symptoms [1, [13][14][15]. More often than not, the diagnosis of vasculitis is made indirectly [1, 15,16].…”

Vasculitis of the Central Nervous System – A Rare Cause of Stroke

“…The prognosis of PACNS is uncertain and careful; most patients dying within 1 year. Altered consciousness or mental state and diffuse cerebral dysfunction are associated with poor outcome, while focal symptoms are associated with better outcome [12,18,30,37]. In this disease it should remember that a substantial number of patients suspected of having PACNS will have signs and symptoms caused by vasospasm rather than true vasculitis of intracerebral vessels.…”