title: Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: a longitudinal case study.
abstract: Myelomeningocele is often accompanied by hydrocephalus (MMH), making it a potentially unstable neurological condition requiring shunt placement and possible revisions. Serial neuropsychological assessment is an important tool in monitoring children with MMH, as cognitive changes can indicate shunt malfunction and hydrocephalus. We present the case of a girl with MMH who had five neuropsychological assessments (ages 5, 7, 11, 12, and 14). Despite a lack of overt neurological symptoms or report of behavioral decline, testing at age 11 revealed decline in multiple neurobehavioral domains, and imaging at that time showed increased hydrocephalus, requiring shunt revision. Subsequent neuropsychological assessment conducted after a 2-year period of medical stability showed improvement and/or a return to baseline levels in some skill areas (i.e., working memory, verbal memory, visuomotor integration, and sustained attention), yet more lasting impairments in others (i.e., Verbal IQ, processing speed, organization, and response inhibition). These lasting cognitive deficits potentially impact independent completion of complex medical self-care tasks. This pattern of recovery highlights vulnerability of brain systems supporting executive functions in children with hydrocephalus and shunt failure.
authors.affiliation: Department of Neuropsychology, Kennedy Krieger Institute and Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA.
authors.forename: Melissa A
authors.initials: MA
authors.lastname: Matson
authors.forename: E Mark
authors.initials: EM
authors.lastname: Mahone
authors.forename: T Andrew
authors.initials: TA
authors.lastname: Zabel
mesh_terms: D000293:Adolescent; D061085:Agenesis of Corpus Callosum; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000601:surgery; D002557:Cerebrospinal Fluid Shunts; D003072:Cognition Disorders / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000150:complications; D008591:Meningomyelocele / Q000150:complications / Q000601:surgery*; D009483:Neuropsychological Tests; D010348:Patient Care Team; D012720:Severity of Illness Index; D016136:Spina Bifida Occulta / Q000150:complications / Q000601:surgery*