title: Consciousness without cortex: a hydranencephaly family survey.
abstract: OBJECTIVE
  Hydranencephaly is commonly taken to exemplify the developmental vegetative state, based largely on the assumption that radical loss of cortical tissue is incompatible with consciousness. The aim of the study reported here was to survey primary caregivers of children born with hydranencephaly for behavioural evidence indirectly informative about the conscious status of these children.
  
  
  METHODS
  The survey recruited 108 primary caregivers through a parent support group and was conducted online via a commercial survey hosting facility. As part of a more extensive questionnaire, participants answered 106 questions bearing on the environmental responsiveness, emotional reactivity, mood and agency of the child in their care.
  
  
  RESULTS
  The survey elicited a many-facetted and detailed set of caregiver answers and written observations regarding the child's behaviour. A conservative measure of agreement among respondents' answers yielded a generic portrait of the responsiveness and expressive behaviour of a hydranencephaly child.
  
  
  CONCLUSIONS
  The generic behavioural characteristics of hydranencephaly thus assessed are incompatible on multiple counts with the unconsciousness characteristic of the vegetative state. This bears on what is included under the concept of quality of life for children with hydranencephaly, and hence on appropriate forms of treatment and care in their case.
authors.affiliation: Hydranencephaly Information Network, Maple Ridge, BC, Canada.
authors.forename: Barb
authors.initials: B
authors.lastname: Aleman
authors.forename: Bjorn
authors.initials: B
authors.lastname: Merker
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003243:Consciousness; D003625:Data Collection; D005190:Family; D006801:Humans; D006832:Hydranencephaly / Q000523:psychology*; D007223:Infant; D055815:Young Adult
keywords: Consciousness; Cortex; Hydranencephaly; Life expectancy; Vegetative state