Abstract
<h3>Objectives</h3> To describe cohort of children with childhood Primary Angiitis of Central Nervous System (cPACNS); report their neurological outcome; evaluate efficacy and safety of used therapies. <h3>Study design</h3> Observational Cohort Study. <h3>Settings</h3> Tertiary Care Children Hospital at Pakistan. <h3>Methods</h3> The study included patients presented with acute ischaemic strokes (AIS) to Department of Neurosciences at Children’s Hospital, Lahor, Pakistan over 2 years (January 2009–December 2010). Their age ≤ 16 years; admitted within 14 days of symptoms onset and they were subjected to through history taking; complete physical examination; laboratory and neuroimaging evaluation. They received pulses of intravenous Steroids and/or Immunoglobulin and Anticoagulant for 4 week followed by maintenance therapy with Azathioprine and low dose Aspirin for 24 months. <h3>Results</h3> Sixty Eight patients were included, 42 (62.76%) boys and 26 (38.23%) girls, mean age was 8.5 ± 3.5 years. Presenting symptoms and signs were; fever (20%), headache (64%), disturbed consciousness (30%), seizures 55%, hemiparesis (60%), and motor deficit (70%). Neuroimaging studies revealed: ischaemic strokes in 50 (73.5%), haemorrhagic strokes in 10 (14.7%) and ischaemic-haemorrhagic lesions in 8 (11.8%). Male sex, deep coma and raised intracranial pressure were poor prognostic signs. Outcome revealed; 12 deaths (17.64%), 11 normal (16.17%), 14 (20.59%) had minor disabilities, 11 (16.17%) with moderate disabilities and 20 (29.41%) had severe disabilities. <h3>Conclusions</h3> Characteristic features of cPACNS on presentation may predict progression and outcome; identify high-risk patients; and guide selection of patients for immunosuppressive therapy. Further studies are required to substantiate our findings regarding immunosuppressive therapy for such patients.
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