Recurrence Prevention of Childhood Primary Angiitis of Central Nervous System by Combination of Azathioprine and Aspirin

Abstract

The objectives of the study were to determine the frequency of various presenting features and its effect on final outcome in childhood primary angiitis of central nervous system (cPACNS). The study also aimed to determine the frequency of complications with use of anticoagulants followed by long term Aspirin and Azathioprine in patients with ischemic infarcts. The study was conducted at the department of the Neurosciences and the Neuroradiology of the Children's Hospital from 1st Jan 2009 to 31st December 2010. Over the period of 2 years, 68 patients with acute ischemic strokes were admitted, who presented within 14 days of onset of the symptoms. Patients with ischaemic infarcts were treated with anticoagulants at least for 04 weeks and this was followed by long term use of Aspirin and Azathioprine. Patient were followed in Hospital based cohort study at single center and were systemically assessed for clinical presentation, classification of cPACNS, adverse effects of anticoagulants, aspirin, azathioprine and their hospital course. Data were analyzed using the Statistical Package for the Social Sciences (SPSS) version 12.0 (Chicago, IL). 68 children with cAIS (boys 62%, girls 38%) with mean age of 8.5 years (median age 7.4 + 3.5 years), were enrolled in this study. Motor deficit (70%); headache (64%) and fever (20%) were the commonest symptoms, whereas, hemiparesis (60%); seizure 55 % (focal 35%, generalized 20%); and decreased conscious level (30%), were the commonest neurological findings. Neuroradiological findings of head revealed; ischemic strokes 50 (73.5%); hemorrhagic strokes 10 (14.7%) and ischaemic- haemorrhagic lesions 8(11.8%).Conventional angiography and/or MRA revealed that at the time of admission 51 (51/68, 75%) of the cohort had non-progressive (obliterative) and 17 (17/68,25%) had evidence of progressive arteriopathy. No secondary hemorrhagic was documented among infarcts strokes, which were treated with heparin and anticoagulants. Hospital outcome was as; survivors 56 (81.5%) and deaths 12 (18.5%). 40patints discharged on long term oral aspirin, and 14 children of these were commenced also on Azathioprine and are on follow-up. Male sex, deep conscious level and intra cerebral bleed causing severe raised intracranial pressure were the poor prognostic factor. The Neurological findings among 56 survivors were; normal 20%; minor disabilities 25%; moderate disabilities 20% and severe disabilities 35%.