Abstract TP111: Primary Angiitis of Central Nervous System . Clinical Profile and Outcome of Forty Seven Patients

2017 
Introduction: Primary angiitis of central nervous system (PACNS) is a rare and serious disorder of uncertain cause, with significant morbidity and mortality. Objective: To determine the clinical profile, response to treatment and outcome in patients with PACNS. Methodology: This is retrospective study of 47 patients from January 2000 to Dec 2015 with either angiographically or biopsy proven PACNS. Outcome at 6 months was considered poor, if modified rankin scale was > 2. Relapse was defined as occurrence of new neurological deficits after clinical improvement, progression of existing disability or radiological worsening. Results: The median age at diagnosis was 36 years (IQR 17) and 33 (70.2%) were males. Twenty-nine patients (61.7%) had angiography positive, 14 patients (38.3%) had biopsy confirmed and 4 patients (8.5%) had both angiography and biopsy positive PACNS .Stroke was the initial presentation in 33 patients (70.2%) followed by rapidly progressive dementia in 11 patients (23.4%) and myelopathy...
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