Post-fracture rehabilitation pathways and association with mortality among adults with cerebral palsy.

OBJECTIVE: Rehabilitation may mitigate the high mortality rates and health declines post-fracture for adults with cerebral palsy, but this is understudied. The objectives were to characterize the post-fracture rehabilitation pathways and identify their association with 1-year survival among adults with cerebral palsy. METHODS: A retrospective cohort study of adults with cerebral palsy with a fragility fracture with continuous health plan enrollment ≥1-year prior to and ≥1 day after their fracture date was performed using a random 20% Medicare fee-for-service dataset. Participants were categorized as a home discharge or inpatient rehabilitation admission post-fracture. For the home discharge cohort, weekly exposure to outpatient physical/occupational therapy (PT/OT) was examined up to 6-month post-fracture. Cox regression examined the association between time-varying PT/OTuse within 6-month post-fracture and mortality from 30 days to 1-year post-fracture before and after adjusting for confounders (e.g. medical complexity). RESULTS: Of 3598 adults with cerebral palsy with an incident fragility fracture, 74% were discharged home without inpatient rehabilitation; they were younger, but more medically complex compared to the 26% admitted to inpatient rehabilitation. Among the home discharge cohort (n = 2662), 43.1% initiated PT/OTwithin 6-month post-fracture, and cumulative PT/OTexposure post-fracture was associated with improved survival; for example, per 3 weeks of PT/OTexposure, the adjusted mortality rate was 40% lower (95% confidence interval (CI) = 0.41-0.89). CONCLUSIONS: Most adults with cerebral palsy with a fragility fracture were discharged home rather than to inpatient rehabilitation, and only 43.1% of that group initiated outpatient PT/OTwithin 6 months post-fracture. Receiving outpatient PT/OTwas associated with improved 1-year survival.

Adults with Cerebral Palsy Require Ongoing Neurologic Care: A Systematic Review.

Cerebral palsy (CP) neurologic care and research efforts typically focus on children. However, most people with CP are adults. Adults with CP are at increased risk of new neurologic conditions, such as stroke and myelopathy, that require ongoing neurologic surveillance to distinguish them from baseline motor impairments. Neurologic factors could also contribute to the motor function decline, chronic pain, and chronic fatigue that are commonly experienced by adults with CP. Based on a systematic literature review, we suggest (1) guidelines for neurologic surveillance and neurologist referral and (2) clinical research questions regarding the evolving neurologic risks for adults with CP. ANN NEUROL 2021;89:860-871.

Clinical bone health among adults with cerebral palsy: moving beyond assessing bone mineral density alone.

AIM: To understand associations among bone mineral density (BMD), bone mineral content (BMC), and bone area, and their association with fractures in adults with cerebral palsy (CP). METHOD: This retrospective cohort study included 78 adults with CP with a hip dual energy X-ray absorptiometry (DXA) from 1st December 2012 to 3rd May 2021 performed at the University of Michigan. Data-driven logistic regression techniques identified which, if any, DXA-derived bone traits (e.g. age/sex/ethnicity-based z-scores) were associated with fracture risk by sex and severity of CP. BMC-area associations were examined to study the structural mechanisms of fragility. RESULTS: Femoral neck area was associated with lower age-adjusted odds ratios (ORs) of fracture history (OR 0.72; 95% confidence interval [CI] 0.49-1.06; p=0.098), while higher BMD was associated with higher odds of incident fracture (OR 3.08; 95% CI 1.14-8.33; p=0.027). Females with fracture had lower area than females without fracture but similar BMC, whereas males with fracture had larger area and higher BMC than males without fracture. The paradoxical BMD-fracture association may be due to artificially elevated BMD from BMC-area associations that differed between females and males (sex interaction, p˂0.05): males had higher BMC at lower area values and lower BMC at higher area values compared to females. INTERPRETATION: BMD alone may not be adequate to evaluate bone strength for adults with CP. Further research into associations (or integration) between BMC and area is needed.

A core outcome set for multimorbidity risk in individuals with cerebral palsy.

AIM: To: (1) investigate the importance of outcome measurement instruments (OMIs) within a core outcome set (COS) for multimorbidity (at least two chronic health conditions) risk in individuals with cerebral palsy (CP); (2) investigate the feasibility of OMIs within the COS in international clinical research settings in adolescents and adults with CP; and (3) describe the associations between the COS data and Gross Motor Function Classification System (GMFCS) levels. METHOD: Eighty-three individuals with CP completed a survey on health outcomes: physical behaviour, nutrition, sleep, endurance, body composition, blood pressure, blood lipids, and glucose. A cross-sectional study assessed the feasibility of the COS in 67 adolescents and adults with CP (mean age 30y, SD 15y 1mo, min-max: 14-68y, 52.2% male) at four centres. Prevalence of multimorbidity risk and associations with GMFCS levels are described. RESULTS: Most participants rated physical behaviour, nutrition, sleep, and endurance as very important. Body composition, blood pressure, nutrition, and sleep were highly feasible since data were collected in 88% or more participants who consented to having the assessments. Physical behaviour, cardiorespiratory endurance, and blood draws were collected in less than 60% of participants. Total time sedentary (ρ=0.53, p<0.01) and endurance (ρ=-0.46, p<0.01) were significantly associated with GMFCS level. INTERPRETATION: The COS identified that most participants had poor sleep quality and endurance, did not have healthy diets, and showed increased sedentary behaviour. Individuals with CP valued these outcomes as most important, suggesting a need to assess these modifiable behaviours in this population. Objective measures of physical behaviour and cardiorespiratory endurance in the COS required additional personnel, time, and participant burden. We recommend that healthcare providers should perform a simpler first screen using questionnaire-based assessments and then focus the use of the remainder of the COS if required for the patient.

UM DESFECHO CENTRAL DEFINIDO PARA RISCO DE MULTIMORBIDADE EM INDIVÍDUOS COM PARALISIA CEREBRAL: OBJETIVO: (1) Investigar a importância dos instrumentos de medição de desfechos (IMD) dentro de um conjunto de desfechos principais (CDS) para risco de multimorbidade (pelo menos duas condições crônicas de saúde) em indivíduos com paralisia cerebral (PC); (2) Investigar a viabilidade de IMD dentro do CDS em cenários internacionais de pesquisa clínica em adolescentes e adultos com PC; e (3) Descrever as associações entre os dados do CDS e os níveis do Sistema de Classificação da Função Motora Grossa (GMFCS). MÉTODO: Oitenta e três indivíduos com PC completaram uma pesquisa sobre desfechos em saúde: comportamento físico, nutrição, sono, resistência, composição corporal, pressão arterial, lipídios no sangue e glicose. Um estudo transversal avaliou a viabilidade do COS em 67 adolescentes e adultos com PC (idade média de 30 anos, desvio padrão de 15 anos e 1 mês, min-max: 14-68 anos, 52,2% do sexo masculino) em quatro centros. São descritas a prevalência do risco de multimorbidade e as associações com os níveis de GMFCS. RESULTADOS: A maioria dos participantes classificou o comportamento físico, nutrição, sono e resistência como muito importantes. Composição corporal, pressão arterial, nutrição e sono foram altamente viáveis, uma vez que os dados foram coletados em 88% ou mais dos participantes que consentiram em realizar as avaliações. Comportamento físico, resistência cardiorrespiratória e coleta de sangue foram coletados em menos de 60% dos participantes. O tempo total de sedentarismo (ρ = 0,53, p < 0,01) e resistência (ρ = −0,46, p < 0,01) foram significativamente associados ao nível de GMFCS. INTERPRETAÇÃO: O CDS identificou que a maioria dos participantes tinha má qualidade e resistência do sono, não tinha dietas saudáveis e apresentava um comportamento sedentário aumentado. Indivíduos com PC valorizaram esses desfechos como mais importantes, sugerindo a necessidade de avaliar esses comportamentos modificáveis nessa população. Medidas objetivas de comportamento físico e resistência cardiorrespiratória no CDS exigiram pessoal adicional, tempo e sobrecarga do participante. Recomendamos que os profissionais de saúde realizem uma primeira triagem mais simples usando avaliações baseadas em questionários e, em seguida, concentrem o uso do restante do CDS, se necessário para o paciente.

Psychological morbidity among adults with cerebral palsy and spina bifida.

BACKGROUND: Very little is known about the risk of developing psychological morbidities among adults living with cerebral palsy (CP) or spina bifida (SB). The objective of this study was to compare the incidence of and adjusted hazards for psychological morbidities among adults with and without CP or SB. METHODS: Privately insured beneficiaries were included if they had an International Classification of Diseases, Ninth revision, Clinical Modification diagnostic code for CP or SB (n = 15 302). Adults without CP or SB were also included (n = 1 935 480). Incidence estimates of common psychological morbidities were compared at 4-years of enrollment. Survival models were used to quantify unadjusted and adjusted hazard ratios for incident psychological morbidities. RESULTS: Adults living with CP or SB had a higher 4-year incidence of any psychological morbidity (38.8% v. 24.2%) as compared to adults without CP or SB, and differences were to a clinically meaningful extent. Fully adjusted survival models demonstrated that adults with CP or SB had a greater hazard for any psychological morbidity [hazard ratio (HR): 1.60; 95% CI 1.55-1.65], and all but one psychological disorder (alcohol-related disorders), and ranged from HR: 1.32 (1.23, 1.42) for substance disorders, to HR: 4.12 (3.24, 5.25) for impulse control disorders. CONCLUSIONS: Adults with CP or SB have a significantly higher incidence of and risk for common psychological morbidities, as compared to adults without CP or SB. Efforts are needed to facilitate the development of improved clinical screening algorithms and early interventions to reduce the risk of disease onset/progression in these higher-risk populations.

Effect of levetiracetam and oxcarbazepine on 4-year fragility fracture risk among prepubertal and pubertal children with epilepsy.

OBJECTIVE: The objective of this study was to determine whether two commonly prescribed antiseizure medications (ASMs), levetiracetam (LEV) and oxcarbazepine (OXC), were associated with an increased risk of fragility fracture in children with epilepsy when initiating therapy during a crucial period of bone development, namely, pre- and midpuberty. METHODS: Claims data from January 1, 2009 to December 31, 2018 were extracted from the Optum Clinformatics Data Mart. Children aged 4-13 years at baseline with at least 5 years of continuous health plan enrollment were included to allow for a 1-year baseline (e.g., pre-ASM exposure) and 4 years of follow-up. Children with epilepsy who were ASM naïve were grouped based on whether ASM treatment initiation included LEV or OXC. The comparison group included children without epilepsy and without ASM exposure. Crude incidence rate (IR; n per 1000 person-years) and IR ratio (IRR; with 95% confidence interval [CI]) were estimated for nontrauma fracture (NTFx), a claims-based proxy for fragility fracture, for up to 4 years of follow-up. Cox proportional hazards regression estimated the hazard ratio (HR; with 95% CI) after adjusting for demographic variables, motor impairment, and baseline fracture. RESULTS: The crude IR (95% CI) of NTFx was 21.5 (21.2-21.8) for non-ASM-users without epilepsy (n = 271 346), 19.8 (12.3-27.2) for LEV (n = 358), and 34.4 (21.1-47.7) for OXC (n = 203). Compared to non-ASM-users, the crude IRR of NTFx was similar for LEV (IRR = .92, 95% CI = .63-1.34) and elevated for OXC (IRR = 1.60, 95% CI = 1.09-2.35); the crude IRR of NTFx was elevated for OXC compared to LEV (IRR = 1.74, 95% CI = 1.02-2.99). The findings were consistent after adjusting for covariates, except when comparing OXC to LEV (HR = 1.71, 95% CI = .99-2.93), which was marginally statistically insignificant (p = .053). SIGNIFICANCE: Initiating OXC, but not LEV, therapy among 4-13-year-olds with epilepsy is associated with an elevated risk of fragility fracture. Studies are needed to determine whether these children could benefit from adjunct bone fragility therapies.

The mortality burden attributable to nontrauma fracture for privately insured adults with epilepsy.

OBJECTIVE: Individuals with epilepsy have poor bone development and preservation throughout the lifespan and are vulnerable to nontrauma fracture (NTFx) and post-NTFx complications. However, no studies have examined the contribution of NTFx to mortality among adults with epilepsy. The objective was to determine whether NTFx is a risk factor for mortality among adults with epilepsy. METHODS: Data from 2011 to 2016 were obtained from Optum Clinformatics Data Mart, a nationwide claims database from a single private payer in the United States. Diagnosis codes were used to identify adults (≥18 years old) with epilepsy, NTFx, and covariates (demographics and pre-NTFx cardiovascular disease, respiratory disease, diabetes, chronic kidney disease, cancer). Crude mortality rate per 100 person-years was estimated. Cox regression estimated hazard ratios (HRs) and 95% confidence intervals (CIs) were determined for mortality, comparing epilepsy and NTFx (EP + NTFx; n = 11 471), epilepsy without NTFx (EP without NTFx; n = 50 384), without epilepsy and with NTFx (without EP + NTFx; n = 423 041), and without epilepsy and without NTFx (without EP without NTFx; n = 6.8 million) after adjusting for covariates. RESULTS: The 3-, 6-, and 12-month crude mortality rates were highest among EP + NTFx (12-month mortality rate = 8.79), followed by without EP + NTFx (12-month mortality rate = 4.80), EP without NTFx (12-month mortality rate = 3.06), and without EP without NTFx (12-month mortality rate = 0.47). After adjustments, the mortality rate was elevated for EP + NTFx for all time points compared to EP without NTFx (eg, 12-month HR = 1.70, 95% CI = 1.58-1.85), without EP + NTFx (eg, 12-month HR = 1.41, 95% CI = 1.32-1.51), and without EP without NTFx (eg, 12-month HR = 5.23, 95% CI = 4.88-5.60). Stratified analyses showed higher adjusted HRs of 12-month mortality for EP + NTFx for all NTFx sites (ie, vertebral column, hip, extremities), all age categories (young, middle-aged, older), and for both women and men. SIGNIFICANCE: Among adults with epilepsy and compared to adults without epilepsy, NTFx is associated with a higher 12-month mortality rate. Findings suggest that NTFx may be a robust risk factor for mortality among adults with epilepsy.

Effect of pain on mood affective disorders in adults with cerebral palsy.

AIM: To determine if pain is associated with 12-month incidence of mood affective disorders (MAD) in adults with cerebral palsy (CP). METHOD: Data from Optum Clinformatics® Data Mart (2013-2016) were used for this retrospective cohort study. Diagnostic codes were used to identify adults (≥18y) with CP, incident cases of MAD, and covariates (other neurodevelopmental conditions, sleep disorders, arthritis). Pain (any type, location) was identified between 1st October 2014 and 30th September 2015. The pain group was divided into new or consistent pain if they had a history of pain (i.e. consistent) in the 12 months before their first pain claim date. Crude incidence rates of MAD (expressed per 100 person-years) were estimated. Cox regression was used to estimate hazard ratio (95% confidence interval [CI]) of MAD after adjusting for covariates. RESULTS: Adults that had new pain (n=859; incidence rate=15.5) or consistent pain (n=1303; incidence rate=17.9) had greater crude incidence rate of MAD compared to adults without pain (n=3726; incidence rate=5.9). The elevated rate of MAD remained after adjusting for covariates, for new pain (hazard ratio=2.4; 95% CI=1.9-3.0) and consistent pain (hazard ratio=2.1; 95% CI=1.7-2.7). INTERPRETATION: Pain is associated with greater incidence of MAD in adults with CP. This association remained after accounting for potential confounding factors. WHAT THIS PAPER ADDS: What this paper adds Pain was associated with higher 12-month incidence of mood affective disorders (MAD). The 12-month MAD incidence was similar between new and consistent pain groups. The MAD incidence remained higher adjusting for neurodevelopmental comorbidities, sleep disorders, and arthritis.

Prevalence of Mental Health Disorders Among Adults With Cerebral Palsy: A Cross-sectional Analysis.

Background: Persons with cerebral palsy (CP) have an increased risk for secondary chronic conditions during childhood, including mental health disorders. However, little is known about how these disorders affect adults with CP. Objective: To determine the prevalence of mental health disorders among adults with CP compared with those without CP. Design: Cross-sectional. Setting: 2016 Optum Clinformatics Data Mart. Patients: 8.7 million adults (including 7348 adults with CP). Measurements: Other neurodevelopmental comorbid conditions (intellectual disabilities, autism spectrum disorders, epilepsy) and 37 mental health disorders (as 6 categories) were identified on the basis of diagnosis codes. Direct age-standardized prevalence of the mental health disorder categories was estimated by sex for adults with CP alone, adults with CP and neurodevelopmental disorders, and adults without CP. Results: Men with CP alone had higher age-standardized prevalence than men without CP for schizophrenic disorders (2.8% [95% CI, 2.2% to 3.4%] vs. 0.7%), mood affective disorders (19.5% [CI, 18.0% to 21.0%] vs. 8.1%), anxiety disorders (19.5% [CI, 18.0% to 21.0%] vs. 11.1%), disorders of adult personality and behavior (1.2% [CI, 0.8% to 1.6%] vs. 0.3%), and alcohol- and opioid-related disorders (4.7% [CI, 3.9% to 5.5%] vs. 3.0%). The same pattern was observed for women. Compared with adults with CP alone, those with CP and neurodevelopmental disorders had similar or higher age-standardized prevalence of the 6 mental health disorder categories, except for the lower prevalence of alcohol- and opioid-related disorders in men. Limitations: Single claims code was used to define the cohort of interest. Information on the severity of CP was not available. Conclusion: Compared with adults without CP, those with CP have an elevated prevalence of mental health disorders, some of which may be more pronounced in patients with comorbid neurodevelopmental disorders. Primary Funding Source: National Institute on Disability, Independent Living, and Rehabilitation Research.

Age-related trends in cardiometabolic disease among adults with cerebral palsy.

AIM: To examine the longitudinal trends of cardiometabolic diseases in a large sample of adults with cerebral palsy (CP). METHOD: The Optum Clinformatics Data Mart is a de-identified nationwide claims database of beneficiaries from a single private payer. Beneficiaries were included if they had an International Classification of Diseases, Ninth Revision, Clinical Modification code for a diagnosis of CP. Adults with at least 3 years of continuous enrollment on a single plan between 2002 and 2009 were included in the final analyses (n=2659). We examined the longitudinal trends of incident diabetes mellitus, hypercholesterolemia, hypertension, cardiac dysrhythmias, and atherosclerosis, stratified by age categories: 18 to 39 years, 40 to 59 years, and 60 years and over. Kaplan-Meier product-limit survival curves were compared across age categories for each of the cardiometabolic outcomes, and a Cox proportional hazards regression was run to determine adjusted hazard ratios. RESULTS: The cumulative incidence of each of the cardiometabolic diseases ranged from 6.0% for atherosclerosis to 34.4% for hypercholesterolemia at 3 years and over. Risk-adjusted Cox proportional hazard models revealed that age was a robust predictor of survival for each outcome, with higher hazard ratio ranges in middle age (hazard ratio 1.41-2.72) and older adults (hazard ratio 2.20-5.93) compared with young adults. INTERPRETATION: Adults with CP have high rates of cardiometabolic diseases; and disease-free survival shortens significantly with higher ages. WHAT THIS PAPER ADDS: Adults with cerebral palsy have high rates of cardiometabolic diseases. Disease-free survival of all cardiometabolic diseases shortens significantly with higher ages. The highest rates were for hypercholesterolemia and hypertension.

TENDENCIAS RELACIONADAS CON LA EDAD EN LA ENFERMEDAD CARDIO-METABÓLICA EN ADULTOS CON PARÁLISIS CEREBRAL: OBJETIVO: Examinar las tendencias longitudinales de las enfermedades cardio-metabólicas en una gran muestra de adultos con parálisis cerebral (PC). MÉTODO: El Data Mart de Optum Clinformatics es una base de datos de reclamaciones nacional anónima de beneficiarios de un único proveedor de servicios de salud privado. Se incluyeron los beneficiarios si tenían un Código de Clasificación Internacional de Enfermedades, Novena Revisión, Modificación Clínica para un diagnóstico de PC. Los adultos con al menos 3 años de inscripción continua en un solo plan entre 2002 y 2009 se incluyeron en los análisis finales (n=2659). Examinamos las tendencias longitudinales de diabetes mellitus incidente, hipercolesterolemia, hipertensión, disritmias cardíacas y aterosclerosis, estratificadas por categorías de edad: de 18 a 39 años, de 40 a 59 años y mayores de 60 años. Se compararon las curvas de supervivencia del límite de producto de Kaplan-Meier para cada categoría de edad para cada resultado cardio-metabólico, y se realizó una regresión de riesgos proporcionales de Cox para determinar los cocientes de riesgo ajustados. RESULTADOS: La incidencia acumulada de cada una de las enfermedades cardio-metabólicas varió del 6.0% para la aterosclerosis al 34.4% para la hipercolesterolemia a los 3 años y más. Los modelos de riesgo proporcional de Cox ajustados por riesgo revelaron que la edad era un predictor robusto de supervivencia para cada resultado, con rangos de razón de riesgo más altos en la mediana edad (razón de riesgo 1.41-2.72) y adultos mayores (razón de riesgo 2.20-5.93) en comparación con los adultos jóvenes. INTERPRETACIÓN: Los adultos con PC tienen altas tasas de enfermedades cardio-metabólicas; y la supervivencia libre de enfermedad se acorta significativamente con las edades más altas.

TENDÊNCIAS RELACIONADAS À IDADE EM DOENÇA CARDIO-METABÓLICA ENTRE ADULTOS COM PARALISIA CEREBRAL: OBJETIVO: Examinar as tendências longitudinais de doenças cardio-metabólicas em uma grande amostra de adultos com paralisia cerebral (PC). MÉTODO: O Optum Clinformatics™ Data Mart é uma base de dados nacional não-identificada de beneficiários de um convênio privado. Os beneficiários foram incluídos se tivessem uma Classificação internacional de doenças, nona revisão, códico de modificação clínica para diagnóstico de PC. Adultos com pelo menos 3 anos de envolvimento contínuo em um único plano entre 2002 e 2009 foram incluídos na análise final (n=2659). Examinamos as tendências longitudinais de incidência de diabetes mellitus, hipercolesterolemia, hipertensão, disritmias cardíacas e aterosclerose, estratificadas por categorias etárias: 18 a 39 anos, 40 a 59 anos, e acima de 60 anos. Curvas de sobreviência produto-limite de Kaplan-Meier foram comparadas entre categorias etárias para cada uma das condições cardio-metabólicas, e uma regressão de riscos proporcionais de Cox foi realizada para determinar as taxas ajustadas de risco. RESULTADOS: A incidência cumulativa de cada doença cardio-metabólica variou de 6.0% para aterosclerose a 34.4% para hipercolesterolemia em 3 anos ou mais. Os modelos de risco proporcionais de Cox ajustados revelaram que a idade foi um preditor robusto da sobrevivência para cada desfecho, com maior taxa de risco na meia idade (taxa de risco 1.41-2.72) e em adultos mais velhos (taxa de risco 2.20-5.93) em comparação com jovens adultos. INTERPRETAÇÃO: Adultos com PC tem taxas altas de doenças cardio-metabólicas; a sobrevivência sem doenças se reduz significantemente em maiores idades.

Focus on Risk Factors for Cardiometabolic Disease in Cerebral Palsy: Toward a Core Set of Outcome Measurement Instruments.

OBJECTIVE: To identify existing outcome measurement instruments (OMIs) assessing risk factors for cardiometabolic disease in adolescents and adults with cerebral palsy (CP) reported on in the literature or used in the field. DATA SOURCES: The COnsensus-based Standards for the selection of health Measurement Instruments database of systematic reviews and 4 electronic databases (Embase, MEDLINE/Ovid, MEDLINE/Pubmed, PsychINFO) were searched up to June 19, 2017, that yielded 2594 articles. Experts in the field were consulted to identify any additional OMIs. STUDY SELECTION: Two reviewers independently applied inclusion criteria to select eligible studies using or evaluating measurement properties of OMIs assessing 1 of 8 outcomes: cardiorespiratory endurance, body size, body composition, physical behavior, sleep, nutrition, blood pressure, and blood lipids and glucose. Studies with an experimental or observational design including ≥10 adolescents or adults with CP were included. DATA EXTRACTION: One reviewer extracted data that were summarized for study and sample characteristics, outcomes, OMIs used, and if applicable data on measurement properties. Two reviewers rated the methodological quality and the quality of the OMIs. Feasibility for clinical practice and research was rated by experts in the field. DATA SYNTHESIS: Ninety OMIs were identified from 56 included articles and by the experts. Seventy OMIs pertained to cardiorespiratory endurance, body size, body composition, and physical behavior, whereas only 5 were identified for sleep and nutrition. Overall synthesis revealed that there is moderate to poor evidence for good quality of OMIs in this population. Based on feasibility for clinical practice, experts agreed on a single OMI per outcome (and 2 for cardiorespiratory endurance) to be included in a core set. CONCLUSION: Despite the range of available OMIs to assess risk factors for cardiometabolic disease in adolescents and adults with CP, evidence of good quality is often lacking. Nonetheless, a preliminary core set of 9 OMIs was systematically developed.

The risk, burden, and management of non-communicable diseases in cerebral palsy: a scoping review.

AIM: To examine the risk, burden, and management of non-communicable diseases (NCDs) among people with cerebral palsy (CP). METHOD: Databases (Ovid MEDLINE, Embase Ovid, CINAHL Plus) were systematically searched up to August 2017. Data on the prevalence of risk factors for, and the burden and management of, cardiovascular diseases, diabetes, cancers, and respiratory diseases were extracted. RESULTS: Thirty-six studies that examined the prevalence of risk factors among people with CP were identified. There was inconsistent evidence that people with CP had higher prevalence of metabolic risk factors such as hypertension, hyperlipidaemia, and obesity, but strong evidence that they participated in low levels of physical activity, compared with people without CP. Seven studies reported on the burden of NCDs. Adults with CP had a higher risk of NCDs, including stroke, chronic obstructive pulmonary disease, and other heart conditions, and death due to NCDs, including cancers, chronic obstructive pulmonary disease, stroke, and ischaemic heart disease, compared with the general population. Only one study reported on the management of NCD, specifically the uptake of breast cancer screening among females. INTERPRETATION: The burden of NCDs is higher among adults with CP compared with the general population. Further research is required to determine the prevalence of metabolic risk factors and management of NCDs among people with CP. WHAT THIS PAPER ADDS: Adults with cerebral palsy (CP) have an increased risk of non-communicable diseases (NCDs) and increased risk of death because of NCDs. Evidence is inconsistent about the elevated prevalence of metabolic risk factors for NCDs. Evidence is consistent that people with CP participate in reduced physical activity. Only one study reported on management of NCD among people with CP. Available evidence suggests people with CP are less likely to receive preventive medicine.

Setting a patient-centered research agenda for cerebral palsy: a participatory action research initiative.

AIM: To establish a patient-centered research agenda for cerebral palsy (CP). METHOD: We engaged a large cross-section of the extended community of people living with CP and those providing healthcare to people with CP ('the community') in an educational series and collaborative survey platform to establish an initial list of prioritized research ideas. After online workshops, a facilitated Delphi process was used to select the 20 highest priorities. Select participants attended an in-person workshop to provide comment and work toward consensus of research priorities. RESULTS: A research agenda for CP was developed by the community, which included consumers, clinicians, and researchers interested in advancing the established research agenda. The results included the top 16 research concepts produced by the process to shape and steward the research agenda, and an engaged cross-section of the community. INTERPRETATION: It has been shown that proactively engaging consumers with clinical researchers may provide more meaningful research for the community. This study suggests that future research should have more focus on interventions and outcomes across the lifespan with increased emphasis on the following outcome measures: function, quality of life, and participation. WHAT THIS PAPER ADDS: A patient-centered research agenda for cerebral palsy was established. Comparative effectiveness of interventions, physical activity, and understanding ageing were leading themes. Longitudinal studies across the lifespan, clinical spectrum, and ages were highly ranked. Participants reported high value for participation outcomes. Participants reported great appreciation for the engagement between consumers and clinician researchers.

Long-term impact of childhood selective dorsal rhizotomy on pain, fatigue, and function: a case-control study.

AIM: Selective dorsal rhizotomy (SDR) is a surgical treatment for spasticity in children with cerebral palsy (CP). Studies suggest long-lasting effects of SDR on spasticity; long-term effects on symptoms and function are not clear. This study tested whether adults with CP (average 22y after SDR) report less pain, fatigue, and functional decline than a retrospectively assessed non-surgical comparison group. METHOD: This was a case-control study. Eighty-eight adults with CP (mean age 27y; SDR=38 male/female/missing=20/16/2; non-surgical [comparison]=50, male/female=19/31) recruited from a tertiary care center and the community completed a battery of self-reported outcome measures. Regression models were used to test whether SDR status predicted pain, fatigue, functional change, and hours of assistance (controlling for Gross Motor Function Classification System level). RESULTS: SDR status did not significantly predict pain interference (p=0.965), pain intensity (p=0.512), or fatigue (p=0.404). SDR related to lower decline in gross motor functioning (p=0.010) and approximately 6 fewer hours of daily assistance than for those in the comparison group (p=0.001). INTERPRETATION: Adults with CP who had SDR in childhood reported less gross motor decline and fewer daily assistance needs than non-surgically treated peers, suggesting the functional impact of SDR persists long after surgery.

Exercise and physical activity recommendations for people with cerebral palsy.

Physical activity and its promotion, as well as the avoidance of sedentary behaviour, play important roles in health promotion and prevention of lifestyle-related diseases. Guidelines for young people and adults with typical development are available from the World Health Organisation and American College of Sports Medicine. However, detailed recommendations for physical activity and sedentary behaviour have not been established for children, adolescents, and adults with cerebral palsy (CP). This paper presents the first CP-specific physical activity and exercise recommendations. The recommendations are based on (1) a comprehensive review and analysis of the literature, (2) expert opinion, and (3) extensive clinical experience. The evidence supporting these recommendations is based on randomized controlled trials and observational studies involving children, adolescents, and adults with CP, and buttressed by the previous guidelines for the general population. These recommendations may be used to guide healthcare providers on exercise and daily physical activity prescription for individuals with CP.

Greater Adipose Tissue Distribution and Diminished Spinal Musculoskeletal Density in Adults With Cerebral Palsy.

OBJECTIVES: To examine differences in adipose tissue distribution, lumbar vertebral bone mineral density (BMD), and muscle attenuation in adults with and without cerebral palsy (CP), and to determine the associations between morphologic characteristics. DESIGN: Cross-sectional, retrospective analyses of archived computed tomography scans. SETTING: Clinical treatment and rehabilitation center. PARTICIPANTS: Adults (N=352) with CP (age, 38.8±14.4y; body mass, 61.3±17.1kg; Gross Motor Function Classification System levels, I-V) and a matched cohort of neurotypical adults. Of the 41 adults with CP included in the study, 10 were not matchable because of low body masses. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Computed tomography scans were assessed for visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) areas, psoas major area and attenuation in Hounsfield units (Hu), and cortical and trabecular BMDs. RESULTS: Adults with CP had lower cortical (ß=-63.41 Hu, P<.001) and trabecular (ß=-42.24 Hu, P<.001) BMDs and psoas major areas (ß=-374.51mm(2), P<.001) and attenuation (ß=-9.21 Hu, P<.001) after controlling for age, sex, and body mass. Adults with CP had greater VAT (ß=3914.81mm(2), P<.001) and SAT (ß=4615.68mm(2), P<.001). Muscle attenuation was significantly correlated with trabecular (r=.51, P=.002) and cortical (r=.46, P<.01) BMD, whereas VAT was negatively associated with cortical BMD (ß=-.037 Hu/cm(2), r(2)=.13, P=.03). CONCLUSIONS: Adults with CP had lower BMDs, smaller psoas major area, greater intermuscular adipose tissue, and greater trunk adiposity than neurotypical adults. VAT and cortical BMD were inversely associated.

Obesity misclassification and the metabolic syndrome in adults with functional mobility impairments: Nutrition Examination Survey 2003-2006.

OBJECTIVES: The purpose of this study was to estimate the degree of obesity misclassification between body mass index (BMI) and body fat percentage in adults with functional mobility impairment, and to determine cardiometabolic risk profiles. METHODS: Data from the combined 2003-2006 National Health and Nutrition Examination Survey (NHANES) were incorporated. The representative sample included 852 individuals, aged 20-85years, reporting at least one major physical limitation related to mobility or lower body function, and 4724 individuals reporting no impairments. Body mass index, percent body fat (%BF) as determined by dual energy X-ray absorptiometry (DXA), objectively measured sedentary behavior and activity, and markers of cardiometabolic risk were compared between adults with and without functional mobility impairments. Among functional mobility impaired individuals, sensitivity, specificity, and receiver operating characteristic curves were used to evaluate the performance of BMI as a continuous variable, as well as various BMI thresholds to detect obesity defined by sex-specific %BF cutoffs. RESULTS: Adults with functional mobility impairments were older, had larger waist circumferences (WC), had greater prevalence of obesity according to BMI and %BF, were more sedentary, had less physical activity, and had higher overall cardiometabolic risk. The standard BMI cutoff for obesity had excellent specificity in both men (100%) and women (98.4%) with functional mobility impairment, but sensitivity was poor (<55%). Whereas approximately 36% and 43% of impaired men and women fell into the obese BMI category, over 80% of men and women were obese according to %BF. Individuals with high %BF who were misclassified as not obese, according to BMI, had a significantly higher prevalence of the metabolic syndrome (17.6%) compared to subjects with normal BMI and low %BF (2.1%). CONCLUSIONS: Obesity misclassification and cardiometabolic risk are prevalent among individuals with functional mobility impairments, and thus diagnostic screening for obesity should be modified to account for %BF and/or waist circumference. Behavioral interventions to decrease sedentary behavior, increase activity, and reduce abdominal obesity are warranted.

Age, sex, and multi-morbidity stratified mortality risk estimates for adults with cerebral palsy to inform clinical decision making.

BACKGROUND: While research has provided key insights into mortality rates and risks for individuals with cerebral palsy (CP), clinically useable mortality risk estimates remain unreported for adults with CP, especially by key patient-level factors. OBJECTIVE: The objective of this study was to generate clinically useable mortality risk estimates among adults with CP to inform clinical decision making. METHODS: This retrospective cohort study, using a fee-for-service Medicare database, identified adults ≥18-years-old with CP from 01/01/2008-12/31/2010 and followed through 12/31/2019 for death. Mortality risk at 1-, 3-, 5-, and 9-year intervals were selected based on common clinical length of time to reasonably benefit from preventive care. Sex-stratified analyses assessed risk estimates by narrow age group (18-25/26-34/35-44/45-54/55-64/65-74/≥75 years old) and multi-morbidity group (Whitney Comorbidity Index score 0-2/3/4-6/≥7). RESULTS: Of 24,767 adults with CP, n = 12,962 were men (mean [SD] age = 48.3 [15.0] years) and n = 11,805 were women (age = 49.7 [15.8] years). Loss to follow-up was rare. 1-year risk was similar between men and women (3.4 % vs. 3.3 %), but increased slightly more for men than women through 9-years (30.1 % vs. 28.0 %). As expected, the mortality risk increased with older age and higher WCI scores. The probability of death (and survival) is presented per age and multi-morbidity group for men and women with CP. CONCLUSIONS: Mortality risk estimates were reported at clinically relevant intervals by age, sex, and multi-morbidity status. This information can be used to weigh harm-to-benefit ratios of screening and treatment strategies based on mortality expectancy estimates.

Comorbidity Clusters Among Adults With Cerebral Palsy: A Latent Class Analysis.

INTRODUCTION: Adults with cerebral palsy are at risk for early multimorbidity onset, but little is known about the composition of multimorbidity profiles or how these profiles present across adulthood. The objective of this study was to identify multimorbidity profiles and association with mortality among adults with cerebral palsy. METHODS: This retrospective cohort study used a random 20% fee-for-service Medicare database from January 1, 2008 to December 31, 2019 from adults aged ≥18 years with cerebral palsy. Latent class analyses using 4-class models were conducted within each age cohort (young adults aged 18-39 years, middle adulthood aged 40-64 years, and older adults aged ≥65 years) to determine patterns of 30 comorbidities defined using the International Classification of Diseases, Ninth Revision codes, identified from January 1, 2008 to December 31, 2010, and their association with mortality through December 31, 2019 (up to 11 years of follow-up); statistical analysis was performed in 2023. RESULTS: Three classes were relatively consistent in the composition of comorbidities across young (n=7,020), middle (n=13,554), and older (n=4,193) cohorts: (1) low morbidity (low proportion of all comorbidities) representing 50.1% (young), 41.4% (middle), and 30.9% (older) of the cohorts; (2) neurologic multimorbidity (e.g., epilepsy, intellectual disabilities) representing 26.0% (young), 26.6% (middle), and 14.7% (older) of the cohorts; and complex multimorbidity (e.g., cardiorespiratory, nutritional, musculoskeletal, neurologic) representing 26.0% (young), 26.6% (middle), and 14.7% (older) of the cohorts. The fourth class varied by young (mental health disorders), middle (hypertension), and older (hypertension and osteoarthritis) age cohorts. Compared with the low morbidity class, other classes had an increased mortality rate for each age cohort (hazard ratio range=1.34-5.58, all p<0.001). CONCLUSIONS: Findings provide insight into varied multimorbidity profiles and associations with mortality across the life course for adults with cerebral palsy.