title: Childhood primary angiitis of the central nervous system.
abstract: OBJECTIVE
  To analyze the clinical course and magnetic resonance angiographic (MRA) abnormalities in children with primary angiitis of the central nervous system (cPACNS).
  
  
  METHODS
  Cohort study.
  
  
  METHODS
  Neurosciences and Neuroradiology Department of the Children's Hospital, Lahore, from January 2009 to December 2010.
  
  
  METHODS
  The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical findings and identification of arterial stenosis on magnetic resonance angiography (MRA) in the absence of an underlying condition that could cause these findings. The treatment protocol for ischaemic infarcts consisted of induction therapy with intravenous steroids pulses and intravenous immunoglobulin followed by maintenance therapy with azathioprine and low dose aspirin. When indicated, they were treated with anticoagulants at least for 4 weeks along with induction therapy. Patients were followed at a single centre and systemically assessed for clinical presentation, classification of disease as progressive or non-progressive, adverse effects of anticoagulants, aspirin, azathioprine and their hospital course.
  
  
  RESULTS
  Sixty-eight children with medium-large vessel cPACNS (62% boys, 38% girls) with mean age of 8.5 ± 3.5 years were enrolled in this study. Motor deficit (70%); headache (64%) and fever (20%) were the commonest symptoms; whereas hemiparesis (60%); seizures 55% (focal 35%, generalized 20%) and decreased conscious level (30%), were the commonest neurological findings. Neuroradiological findings were ischaemic strokes in 50 (73.5%), haemorrhagic strokes in 10 (14.7%) and ischaemic haemorrhagic lesions in 8 cases (11.8%). Angiographically 51 (51/68, 75%) of the cohort had non-progressive (obliterative) and 17 (17/68, 25%) had evidence of progressive arteriopathy at the time of admission. No secondary haemorrhagic lesions were documented among infarcts strokes, which were treated with heparin and oral anticoagulants. Outcome was survival in 56 cases (81.5%) and death in 12 cases (18.5%). All survivors were discharged on long-term oral aspirin; 15 of them were also commenced on azathioprine. Neurological findings among the 56 survivors were; normal 20%, minor disabilities in 25%, moderate disabilities in 20% and severe disabilities in 35%.
  
  
  CONCLUSIONS
  The spectrum of cPACNS includes both progressive and non-progressive forms with significant morbidity and mortality. This treatment protocol of immunosuppressive therapy may improve long-term neurological outcome in children with medium-large vessel childhood primary angiitis of the CNS.
authors.affiliation: Department of Paediatrics Neurology, The Children's Hospital, Lahore. docmalikpk2000@yahoo.co.in
authors.forename: Muhammad Akbar
authors.initials: MA
authors.lastname: Malik
authors.forename: Muhammad
authors.initials: M
authors.lastname: Zia-ur-Rehman
authors.forename: Malik Muhammad
authors.initials: MM
authors.lastname: Nadeem
authors.forename: Farooq Rasool
authors.initials: FR
authors.lastname: Chaudhry
authors.forename: Abid Ali
authors.initials: AA
authors.lastname: Qureshi
authors.forename: Muhammad
authors.initials: M
authors.lastname: Nawaz
authors.forename: Hamza
authors.initials: H
authors.lastname: Malik
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000925:Anticoagulants / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D002545:Brain Ischemia / Q000188:drug therapy / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004359:Drug Therapy, Combination; D005260:Female; D005334:Fever / Q000209:etiology; D006261:Headache / Q000209:etiology; D006784:Hospitals, Teaching; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D018810:Magnetic Resonance Angiography; D008297:Male; D011241:Prednisone / Q000627:therapeutic use*; D017678:Sex Distribution; D020521:Stroke / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*
chemical_list: D000893:Anti-Inflammatory Agents; D000925:Anticoagulants; D007166:Immunosuppressive Agents; D001379:Azathioprine; D011241:Prednisone