Primary central nervous system vasculitis: analysis of 101 patients.
ABSTRACT To analyze the clinical findings, response to therapy, outcome, and incidence of primary central nervous system vasculitis (PCNSV) in a large cohort from a single center.
We retrospectively studied 101 patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 21-year period. This was a collaborative study by five departments at a large multispecialty clinic. Clinical findings and outcomes were compared among patients categorized by method of diagnosis, response to therapy, survival, and degree of disability. An annual incidence rate was calculated.
Seventy patients were diagnosed by angiography and 31 by central nervous system biopsy. Three histological patterns were observed during biopsy. Although most patients responded to therapy, an increased mortality rate was observed. Relapses occurred in one fourth of patients. Mortality rate and disability at last follow-up were greater in those who presented with a focal neurological deficit, cognitive impairment, cerebral infarctions, and angiographic large-vessel involvement but were lower in those with prominent gadolinium-enhanced lesions when evaluated by magnetic resonance imaging. The annual incidence rate of PCNSV was 2.4 cases per 1,000,000 person-years.
PCNSV is a rare disease that may result in serious neurological outcomes or death. Angiography and brain biopsy may complement each other when determining the diagnosis. Early recognition and treatment may reduce poor outcomes. PCNSV is a variable syndrome that appears to consist of several subsets of heterogeneous diseases.
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ABSTRACT: Isolated angiitis of the central nervous system (IAC) is usually a fatal inflammatory disease with a predilection for small blood vessels. Recurrent cerebral infarction leading to death within a few years is the usual course, but this may be significantly altered by aggressive immunosuppressive therapy with prednisone and cyclophosphamide. Other diseases may, however, present with similar clinical and angiographic features. Because antemortem diagnosis suggests a therapy, establishing the criteria for diagnosis is important. This report describes clinical, angiographic, and biopsy features, and therapy of five successfully treated patients with IAC. The following specific criteria are recommended for establishing an antemortem diagnosis of IAC: (1) clinical pattern of headaches and multifocal neurologic deficits present for at least 6 months, unless the deficits are severe at onset or rapidly progressive; (2) cerebral angiography demonstrating segmental arterial narrowing; (3) exclusion of systemic inflammation or infection; and (4) leptomeningeal/parenchymal biopsy demonstrating vascular inflammation or exclusion of alternate diagnoses. Based upon the successful management of these five previously unreported patients, as well as others in the literature, the following treatment regimens are recommended for the initial 6 weeks of therapy: (1) prednisone 40 to 60 mg/day, and (2) cyclophosphamide 100 mg/day.Neurology 03/1989; 39(2 Pt 1):167-73. · 8.25 Impact Factor
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ABSTRACT: Primary angiitis of the central nervous system (PACNS) is a rare form of angiitis limited to the central nervous system. The diagnosis and classification of this disorder has been problematic, owing to the lack of uniform diagnostic criteria and the difficulty in obtaining pathologic material for diagnosis. This study proposes to establish diagnostic criteria for PACNS which would include 1) the presence of an unexplained neurologic deficit after thorough clinical and laboratory evaluation; 2) documentation by cerebral angiography and/or tissue examination of an arteritic process within the central nervous system; and 3) no evidence of a systemic vasculitide or any other condition to which the angiographic or pathologic features could be secondary. Utilizing these criteria, 8 new cases are reported and are combined with 40 previously diagnosed cases from the literature. The clinical findings of the combined series revealed that headache was the most common symptom (58%) with a combination of focal and diffuse neurologic deficits described in 79% of the group. The diagnostic approach to PACNS should include a variety of laboratory tests and examination of the cerebral spinal fluid primarily to rule out mimicking conditions. Special procedures including electroencephalography, computed axial tomography, and magnetic resonance imaging appear only marginally helpful in securing the diagnosis, but are extremely important in ruling out other conditions. Angiography appears to be the first invasive diagnostic procedure of choice and it has a high predictive value when properly interpreted. Leptomeningeal and cortical biopsy can be accomplished with acceptable mortality and should be performed along with a normal or non-diagnostic angiogram when the diagnostic likelihood is high. Therapy of PACNS with a combination of cytotoxic drugs and high-dose corticosteroids has greatly improved the prognosis for this condition.Medicine 02/1988; 67(1):20-39. · 4.23 Impact Factor
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ABSTRACT: Isolated angiitis of the central nervous system is an uncommon clinicopathologic entity characterized by vasculitis restricted to the vessels of the central nervous system without other apparent systemic vasculitis. Experience with the diagnosis, treatment, and follow-up evaluation in four patients with this disease is presented. Early manifestations of disease include severe headaches, altered mental function, and focal neurologic deficits. The pattern of progression from headaches and altered mental status to multifocal neurologic deficits is particularly suggestive of the diagnosis of vasculitis of the central nervous system. Systemic symptoms such as fever, myalgia, arthralgia, and arthritis, which occur frequently in other vasculitic syndromes, are generally not present in patients with isolated angiitis of the central nervous system. No single laboratory study can firmly establish or completely exclude the diagnosis; consequently, tissue diagnosis with biopsy of the brain parenchyma and leptomeninges may be required. In two patients, recurrent disease developed despite treatment with corticosteroids alone. Sustained clinical remission was induced in all four patients with a regimen of daily cyclophosphamide and alternate-day prednisone therapy. Cyclophosphamide and alternate-day prednisone therapy are considered the treatment of choice in severe, progressive, or corticosteroid-resistant isolated angiitis of the central nervous system.The American Journal of Medicine 02/1983; 74(1):97-105. · 4.77 Impact Factor