title: Cognitive dysfunctions and cerebral microbleeds in adult patients with haemophilia A: a clinical and MRI pilot-study.
abstract: BACKGROUND
  Studies providing information about the cognitive profile of adult haemophiliacs are lacking.
  
  
  OBJECTIVE
  To assess the neuropsychological profile in a group of Haemophiliac patients; to detect asymptomatic cerebral microbleeds (CMBs) and any correlation between CMBs and cognitive dysfunctions; to verify how several contributing factors may determine cognitive dysfunctions and/or Magnetic Resonance Imaging (MRI) findings.
  
  
  METHODS
  Adult haemophiliacs without history of brain bleeding were prospectively enrolled on Padua Haemophilia Centre. Patients underwent: i) "Short Neuropsychological Test" assessing cognitive functions (Short Neuropsychological Examination) to obtain an overall cognitive performance (OCP) profile standardised on a cohort matched for age, sex, cultural profile; ii) MRI of the brain to evaluate areas of brain atrophy or haemorrhagic lesions. We collected information on anti-haemorrhagic treatment, cardiovascular risk profile, viral infections, birth trauma.
  
  
  RESULTS
  49 adults with haemophilia (31 severe-moderate, 18 mild) were enrolled. 73% of patients presented a reduction in OCP. According to OCP, no significant difference between severe and mild haemophilia was observed though scores tended to be worse in severe haemophilia (mean Z score 0.20 ± 0.10 vs s0.15 ± 0.11). Considering risk factors, OCP correlated significantly with coronary artery disease (p=0.02). MRI findings in 44 patients, indicated CMBs were inversely related to OCP (R=-0.32 p<0.05). CMBs were associated with cardiovascular risk factors (p=0.018).
  
  
  CONCLUSIONS
  Adult haemophiliacs seem to present high prevalence of mild cognitive dysfunctions that doesn't correlate with the severity of haemophilia probably for the few number of patients evaluated. OCP impairment seems to be related to the presence of CMBs and of risk factors for cardiovascular disease.
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, 2nd Chair of Internal Medicine, University of Padua, Italy. Electronic address: ezio.zanon@unipd.it.
authors.forename: Ezio
authors.initials: E
authors.lastname: Zanon
authors.affiliation: Department of Neuroradiology, University of Padua, Italy.
authors.forename: Renzo
authors.initials: R
authors.lastname: Manara
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, 2nd Chair of Internal Medicine, University of Padua, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Milan
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, 2nd Chair of Internal Medicine, University of Padua, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Brandolin
authors.affiliation: CIRMANMEC, University of Padua, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Mapelli
authors.affiliation: Department of Neuroradiology, University of Padua, Italy.
authors.forename: Rodica
authors.initials: R
authors.lastname: Mardari
authors.affiliation: Department of General Psychology, University of Padua, Italy.
authors.forename: Sandra
authors.initials: S
authors.lastname: Rosini
authors.affiliation: Department of Clinical and Experimental Medicine, University of Padua, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Amodio
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000098:blood supply* / Q000473:pathology*; D002318:Cardiovascular Diseases / Q000150:complications / Q000209:etiology; D002543:Cerebral Hemorrhage / Q000150:complications* / Q000473:pathology; D003072:Cognition Disorders / Q000150:complications* / Q000473:pathology; D005260:Female; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D012307:Risk Factors; D055815:Young Adult
keywords: Cognition; Haemophilia; Microbleeds