Background

Childhood primary angiitis of central nervous system (cPACNS) is rare idiopathic vasculitis most frequently in adults. Children with this disorder can present with a range of neurological symptoms and signs including decreased consciousness, seizures, hemiparesis, cranial nerve deficits, and cognitive deficits. Delayed diagnosis and treatment may compromise the outcome. Therapeutic modalities including Anti-Platelet agents, Corticosteroids, Azathioprine, Cyclophosphamide and other Immunomodulatory agents have been used with variable success.

Purpose

We wanted to study a cohort of children with childhood primary angiitis of Central Nervous System (cPACNS); and evaluate efficacy and safety of their management.

Methods

Current study is an observational cohort study that included 68 patients admitted with acute ischemic strokes (AIS) within 14 days of symptoms onset at Department of Neurosciences at Children’s Hospital, Lahore, Pakistan from January 2009 to December 2010 with an age≤ 16 years. They were subjected to physical examination laboratory and neuroimaging evaluation. They received pulses of intravenous steroids and/or Immunoglobulins for 4 weeks with maintenance dose of Azathioprine and low dose Aspirin for 24 months and kept on follow for 2 years.

Results

Sixty eight patients were included; 42 (62.76%) boys and 26 (38.23%) girls whose mean age was 8.5±3.5 years. Presenting symptoms and signs included fever (20%), headache (64%), disturbed consciousness (30%), seizures 55%, hemiparesis (60%), and motor deficit (70%). Neuroimaging studies revealed ischemic strokes in 50 patients (73.5%), hemorrhagic strokes in 10 (14.7%) and ischemic-hemorrhagic lesions in 8 (11.8%). Males with, deep coma and raised intracranial pressure were poor prognostic signs. Mortality was encountered in 12 patients (17.64%) with normal outcome in 11 (16.17%), minor disabilities in 14 (20.59%), moderate disabilities in 11 (16.17%) and severe disabilities in 20 (29.41%).

Conclusions

Characteristic features of cPACNS on presentation may predict later progression and outcome, identify high-risk patients which may guide selection of patients for immunosuppressive therapy. Further studies are required to substantiate our findings regarding immunosuppressive therapy for such patients.