Soumya Sundaram1, Deepak Menon1, Pooja Khatri2, Sapna Erat Sreedharan1, E R Jayadevan3, Prabhakaran Sarma4, Christian Pagnoux5, P N Sylaja1. 1. Comprehensive Stroke Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India. 2. Department of Neurology, University of Cincinnati, Cincinnati, OH, USA. 3. Department of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India. 4. Achutha Menon Centre for Health Sciences, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India. 5. Department of Rheumatology, Vasculitis Clinic, Mount Sinai Hospital, Toronto, Ontario, Canada.
Abstract
OBJECTIVE: To describe the clinical profile, treatment response and predictors of outcome in patients with primary angiitis of the central nervous system (PACNS) from a single tertiary care center. METHODOLOGY: Retrospective analysis of consecutive patients diagnosed with PACNS from January 2000 to December 2015. Outcome was defined as poor when the 6-month modified Rankin scale (mRS) was ≥3. RESULTS: The median age of the 45 patients included in this study was 36 (range 19-70) years at disease onset and 31 (68.9%) were males. The initial presentation was ischemic stroke in 15 (33.3%), hemorrhagic stroke in 4 (8.9%), headache in 11 (24.4%), seizures in 8 (17.8%) and cognitive dysfunction in 5 (11.1%) patients. Diagnosis was confirmed by a four vessel cerebral digital subtraction angiogram (DSA), biopsy and by both biopsy and DSA in 26 (57.8%), 15 (33.3%) and 4 (8.9%) patients, respectively. All patients received glucocorticoids and 14 patients received in addition either cyclophosphamide or azathioprine as their first treatment. The median duration of follow-up was 33.1 (0.7-356) months. A poor 6-month outcome was observed in 12 (26.7%) patients. Relapse occurred in 25 (55.6%) patients and 7 (15.6%) died. Predictors of a poor outcome consisted of cognitive dysfunction at diagnosis (80% vs 20%; P = 0.014) and NIHSS ≥5 (62.5% vs 37.5%; P <.0005). None of the patients with a normal EEG had a poor outcome (P = 0.046). Predictors of relapse were a higher NIHSS at admission (P =.032) and a normal DSA (P = 0.002). CONCLUSION: In this cohort, severe deficits and cognitive symptoms at onset and an abnormal EEG were associated with a poor 6-month outcome.
OBJECTIVE: To describe the clinical profile, treatment response and predictors of outcome in patients with primary angiitis of the central nervous system (PACNS) from a single tertiary care center. METHODOLOGY: Retrospective analysis of consecutive patients diagnosed with PACNS from January 2000 to December 2015. Outcome was defined as poor when the 6-month modified Rankin scale (mRS) was ≥3. RESULTS: The median age of the 45 patients included in this study was 36 (range 19-70) years at disease onset and 31 (68.9%) were males. The initial presentation was ischemic stroke in 15 (33.3%), hemorrhagic stroke in 4 (8.9%), headache in 11 (24.4%), seizures in 8 (17.8%) and cognitive dysfunction in 5 (11.1%) patients. Diagnosis was confirmed by a four vessel cerebral digital subtraction angiogram (DSA), biopsy and by both biopsy and DSA in 26 (57.8%), 15 (33.3%) and 4 (8.9%) patients, respectively. All patients received glucocorticoids and 14 patients received in addition either cyclophosphamide or azathioprine as their first treatment. The median duration of follow-up was 33.1 (0.7-356) months. A poor 6-month outcome was observed in 12 (26.7%) patients. Relapse occurred in 25 (55.6%) patients and 7 (15.6%) died. Predictors of a poor outcome consisted of cognitive dysfunction at diagnosis (80% vs 20%; P = 0.014) and NIHSS ≥5 (62.5% vs 37.5%; P <.0005). None of the patients with a normal EEG had a poor outcome (P = 0.046). Predictors of relapse were a higher NIHSS at admission (P =.032) and a normal DSA (P = 0.002). CONCLUSION: In this cohort, severe deficits and cognitive symptoms at onset and an abnormal EEG were associated with a poor 6-month outcome.
Entities:
Keywords:
Brain biopsy; CNS vasculitis; PACNS; cognitive dysfunction; outcome; primary angiitis of central nervous system; relapse; stroke
Authors: Soumya Sundaram; P Naveen Kumar; Dev Prakash Sharma; Chandrasekharan Kesavadas; Sapna Erat Sreedharan; B Arun Prasad; P N Sylaja Journal: Ann Indian Acad Neurol Date: 2021-05-21 Impact factor: 1.383
Authors: Gelsomina Mansueto; Giuseppe Lanza; Francesco Fisicaro; Danielle Alaouieh; Emily Hong; Sara Girolami; Marco Montella; Alessandro Feola; Mario Di Napoli Journal: Curr Neurol Neurosci Rep Date: 2022-02-09 Impact factor: 6.030