Objectives 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.
Study design Observational Cohort Study.
Settings Tertiary Care Children Hospital at Pakistan.
Methods 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.
Results 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.
Conclusions 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.
- Primary Angiitis
- Intra-cerebral Hemorrhage
- Central Nervous System
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