Susan M Reid1,2,3, Elaine M Meehan1,2,3, Sarah J Arnup4, Dinah S Reddihough1,2,3. 1. Developmental Disability and Rehabilitation Research, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. 2. Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia. 3. Neurodevelopment and Disability, The Royal Children's Hospital, Melbourne, Victoria, Australia. 4. Clinical Epidemiology and Biostatistics Unit, Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
Abstract
AIM: A population-based observational study design was used to describe the epidemiology of intellectual disability in cerebral palsy (CP) in terms of clinical and neuroimaging associations, and to report the impact of intellectual disability on utilization of health services and length of survival. METHOD: Population CP registry data were used to retrospectively assess the frequency of intellectual disability and strength of associations between intellectual disability and mobility, epilepsy, vision, hearing, communication, and neuroimaging patterns (n=1141). Data linkage was undertaken to assess usage of hospital inpatient and emergency department services. Survival analysis was performed in a 30-year birth cohort (n=3248). RESULTS: Intellectual disability, present in 45% of the cohort, was associated with non-ambulation (47% vs 8%), later walking (mean 2y 7mo vs 1y 9mo), hypotonic (8% vs 1%) or dyskinetic (9% vs 5%) CP, a quadriplegic pattern of motor impairment (42% vs 5%), epilepsy (52% vs 12%), more emergency and multi-day hospital admissions, and reduced 35-year survival (96% vs 71%). Grey matter injuries (13% vs 6%), malformations (18% vs 6%), and miscellaneous neuroimaging patterns (12% vs 4%) were more common in people with intellectual disability. INTERPRETATION: Intellectual disability adds substantially to the overall medical complexity in CP and may increase health and mortality disparities. WHAT THIS STUDY ADDS: Cerebral maldevelopments and grey matter injuries are associated with higher intellectual disability rates. Health care is more 'crisis-driven' and 'reactive' in children with co-occurring intellectual disability. Length of survival is reduced in individuals with CP and co-occurring intellectual disability.
AIM: A population-based observational study design was used to describe the epidemiology of intellectual disability in cerebral palsy (CP) in terms of clinical and neuroimaging associations, and to report the impact of intellectual disability on utilization of health services and length of survival. METHOD: Population CP registry data were used to retrospectively assess the frequency of intellectual disability and strength of associations between intellectual disability and mobility, epilepsy, vision, hearing, communication, and neuroimaging patterns (n=1141). Data linkage was undertaken to assess usage of hospital inpatient and emergency department services. Survival analysis was performed in a 30-year birth cohort (n=3248). RESULTS: Intellectual disability, present in 45% of the cohort, was associated with non-ambulation (47% vs 8%), later walking (mean 2y 7mo vs 1y 9mo), hypotonic (8% vs 1%) or dyskinetic (9% vs 5%) CP, a quadriplegic pattern of motor impairment (42% vs 5%), epilepsy (52% vs 12%), more emergency and multi-day hospital admissions, and reduced 35-year survival (96% vs 71%). Grey matter injuries (13% vs 6%), malformations (18% vs 6%), and miscellaneous neuroimaging patterns (12% vs 4%) were more common in people with intellectual disability. INTERPRETATION: Intellectual disability adds substantially to the overall medical complexity in CP and may increase health and mortality disparities. WHAT THIS STUDY ADDS: Cerebral maldevelopments and grey matter injuries are associated with higher intellectual disability rates. Health care is more 'crisis-driven' and 'reactive' in children with co-occurring intellectual disability. Length of survival is reduced in individuals with CP and co-occurring intellectual disability.
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Authors: Sheng Chih Jin; Sara A Lewis; Somayeh Bakhtiari; Xue Zeng; Michael C Sierant; Sheetal Shetty; Sandra M Nordlie; Aureliane Elie; Mark A Corbett; Bethany Y Norton; Clare L van Eyk; Shozeb Haider; Brandon S Guida; Helen Magee; James Liu; Stephen Pastore; John B Vincent; Janice Brunstrom-Hernandez; Antigone Papavasileiou; Michael C Fahey; Jesia G Berry; Kelly Harper; Chongchen Zhou; Junhui Zhang; Boyang Li; Hongyu Zhao; Jennifer Heim; Dani L Webber; Mahalia S B Frank; Lei Xia; Yiran Xu; Dengna Zhu; Bohao Zhang; Amar H Sheth; James R Knight; Christopher Castaldi; Irina R Tikhonova; Francesc López-Giráldez; Boris Keren; Sandra Whalen; Julien Buratti; Diane Doummar; Megan Cho; Kyle Retterer; Francisca Millan; Yangong Wang; Jeff L Waugh; Lance Rodan; Julie S Cohen; Ali Fatemi; Angela E Lin; John P Phillips; Timothy Feyma; Suzanna C MacLennan; Spencer Vaughan; Kylie E Crompton; Susan M Reid; Dinah S Reddihough; Qing Shang; Chao Gao; Iona Novak; Nadia Badawi; Yana A Wilson; Sarah J McIntyre; Shrikant M Mane; Xiaoyang Wang; David J Amor; Daniela C Zarnescu; Qiongshi Lu; Qinghe Xing; Changlian Zhu; Kaya Bilguvar; Sergio Padilla-Lopez; Richard P Lifton; Jozef Gecz; Alastair H MacLennan; Michael C Kruer Journal: Nat Genet Date: 2020-09-28 Impact factor: 41.307