title: Cognitive and functional outcome in spina bifida-Chiari II malformation.
abstract: OBJECTIVE
  The long-term outcome in spina bifida-Chiari II-hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth.
  
  
  METHODS
  Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated.
  
  
  RESULTS
  Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 ± 0.20; mean ± standard deviation (SD)) than those for physical (0.64 ± 0.15) and social-emotional (0.65 ± 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 ± 17.0; mean ± SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function.
  
  
  CONCLUSIONS
  Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction.
authors.affiliation: Department of Neurosurgery, The Walton Centre Foundation NHS Trust, Lower Lane, Liverpool, L9 7LJ, UK. michael.jenkinson@liv.ac.uk
authors.forename: Michael D
authors.initials: MD
authors.lastname: Jenkinson
authors.forename: Sophie
authors.initials: S
authors.lastname: Campbell
authors.forename: Caroline
authors.initials: C
authors.lastname: Hayhurst
authors.forename: Simon
authors.initials: S
authors.lastname: Clark
authors.forename: Jothy
authors.initials: J
authors.lastname: Kandasamy
authors.forename: Maggie K
authors.initials: MK
authors.lastname: Lee
authors.forename: Ann
authors.initials: A
authors.lastname: Flynn
authors.forename: Peter
authors.initials: P
authors.lastname: Murphy
authors.forename: Conor L
authors.initials: CL
authors.lastname: Mallucci
mesh_terms: D000203:Activities of Daily Living / Q000523:psychology*; D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000503:physiopathology* / Q000523:psychology*; D003072:Cognition Disorders / Q000209:etiology / Q000503:physiopathology / Q000523:psychology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000503:physiopathology / Q000523:psychology; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D016135:Spinal Dysraphism / Q000150:complications / Q000503:physiopathology* / Q000523:psychology*; D016896:Treatment Outcome; D055815:Young Adult