Primary central nervous system vasculitis: analysis of 101 patients

Abstract

Objective: 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. Methods: 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. Results: 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.

The Emerging Link Between Autoimmune Disorders and Neuropsychiatric Disease

“…57–59 As with other vasculitides, this syndrome is thought to result from autoimmune dysfunction, based largely on the response to immunotherapy. Affected patients are usually middle-aged men (mean age, 50 years), with diffuse or focal neurologic findings resulting from injury to intracerebral vessels.…”

“…Affected patients are usually middle-aged men (mean age, 50 years), with diffuse or focal neurologic findings resulting from injury to intracerebral vessels. 59 Psychiatric symptoms can include memory dysfunction, confusion, and affective changes. 57,59 Multifocal lesions or infarcts on MRI, inflammatory changes in CSF, and cerebral angiography showing vasculitic changes (alternating vessel narrowing and dilations) lead to diagnosis.…”

“…57,59 Multifocal lesions or infarcts on MRI, inflammatory changes in CSF, and cerebral angiography showing vasculitic changes (alternating vessel narrowing and dilations) lead to diagnosis. 60 Although sensitivity varies among studies, 59 brain biopsy is generally considered the gold standard of diagnosis (particularly to rule out infectious processes) and often reveals granulomatous changes. 57,60 Treatment is with corticosteroids alone or in combination with cyclophosphamide.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The annual incidence is approximately 2 per million of the population, and it occurs more commonly in men, with the onset usually in the fifth decade of life [113,114]. Patients generally present with headache and encephalopathy; strokes may also occur.…”

“…Treatment is generally initiated with high-dose corticosteroids and is often followed by cyclophosphamide [119]. Relapse is common and occurs in up to 25% of patients [113]. Successful therapy with tumor necrosis factor (TNF)-α inhibitors, rituximab, and MMF has been described [120][121][122].…”