Primary angiitis of the central nervous system: emerging variants
ABSTRACT Primary angiitis of the central nervous system (PACNS), a serious disease, has not featured prominently in the spectrum of multi-organ disease seen in vasculitis clinics.
To evaluate the presentation, natural history and features of PACNS variants and compare to those of systemic vasculitides.
Patients (n=105) presented during 1988-2003 to a tertiary regional vasculitis clinic receiving unselected disease types. Data were collected from a clinical database, patient and laboratory records.
The frequency of PACNS presentation rose over the study period, compared with most of the other vasculitides. When PACNS was divided into small- and middle-sized vessel disease (SVD/MVD), their clinical courses differed substantially. SVD PACNS was responsive to immunosuppressive drugs, but relapsed during prolonged periods in all patients on maintenance immunosuppressives, or after withdrawal of treatment, causing recurrent, severe and irreversible CNS injury. MVD PACNS had isolated episodes at presentation, with a paucity of relapses during prolonged follow-up.
Similarities between SVD PACNS and microscopic polyarteritis suggest the former may represent a limited form of the latter. MVD PACNS has a distinctly more benign relapse pattern than its multisystem counterpart polyarteritis nodosa. Acute-phase serology was useful in designating inflammatory processes at presentation of patients presenting with encephalopathy caused by SVD only, but were unhelpful in defining relapses in this form of PACNS, the definition of which in all cases rested on clinical assessment and MR scanning. Direct cerebral angiography was not diagnostic in any case of SVD PACNS; positive brain biopsy is diagnostically unequivocal, but the total clinical syndrome with imaging may establish a diagnosis with highest probability. In MVD PACNS, angiography with MR scan proved diagnostic. We suggest an algorithm for a rational, minimally invasive approach to investigation. In PACNS, SVD and MVD are important variants, and decisions about therapy should incorporate these distinctions.
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ABSTRACT: Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis of unknown aetiology. Multifaceted clinical manifestations, non-specific MRI findings, a broad range of differential diagnoses and diverse pathological appearances prove to be a diagnostic challenge. However, a prompt diagnosis and aggressive treatment are crucial to avoid permanent damage. Hence, we present the clinico-pathological spectrum of this entity and highlight the limitations of currently available diagnostic modalities. We describe in detail the histopathological findings of eight cases of PACNS diagnosed at the Department of Pathology, AIIMS, over a period of eight years. Eight cases of PACNS were identified during this period. Five cases (62.5%) showed features of granulomatous vasculitis, two (25%) showed lymphocytic vasculitis and one case (12.5%) showed a predominantly necrotizing pattern of vasculitis. Diagnosis of PACNS is a challenge and requires a high index of clinical suspicion. Appropriate work-up to exclude other conditions is mandatory. Brain biopsy is useful in making the diagnosis and ruling out mimicking conditions.Folia Neuropathologica 01/2014; 52(2):187-96. DOI:10.5114/fn.2014.43790 · 1.67 Impact Factor
Clinical Neurology and Neurosurgery 08/2013; · 1.25 Impact Factor
Article: CNS vasculitis.[Show abstract] [Hide abstract]
ABSTRACT: Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV. Infectious, neoplastic, and autoimmune conditions should be carefully evaluated. Knowledge of CNSV mimics is crucial given the therapeutic and prognostic implications. Reversible cerebral vasoconstriction syndrome is now recognized as the most common mimicker of PACNS. Much progress has been made in the understanding of PACNS histopathology, and specific clinical subsets and their response to treatment. Contrary to historical reports, recent PACNS cohorts achieved favorable clinical outcomes when early diagnosis and prompt treatment was initiated. Successful treatment with newer drugs has also been reported in PACNS cases. Newer imaging modalities hold promise for distinguishing inflammatory from noninflammatory vascular lesions. In this review, the authors provide a broad overview with updates on the types of adult CNSV along with its clinical assessment, diagnosis, and treatment.Seminars in Neurology 09/2014; 34(4):405-12. DOI:10.1055/s-0034-1390389 · 1.78 Impact Factor