Heidi J. Haapala

Functional outcomes of childhood dorsal rhizotomy in adults and adolescents with cerebral palsy: Clinical article

OBJECT In this descriptive study the authors evaluated medical outcomes, interventions, satisfaction with life, and subjective impressions about selective dorsal rhizotomy (SDR) in older adolescents and adults who had undergone the procedure as children. METHODS A survey was administered to older adolescents (16-20 years old) and adults with CP who had undergone SDR between 1986 and 2000 at two academic centers. The patients or their caregivers participated in telephone or clinic interviews. Subjective impressions about the SDR and a history of post-SDR medical interventions were obtained. Current functional status, history and ratings of pain, educational achievement, living situation, and subjective health status were also recorded. The Diener Satisfaction with Life Scale (SWLS) was administered. RESULTS Eighty-eight participants, mean age 25.6 ± 4.8 years (mean ± standard deviation), were interviewed at a mean of 19.6 ± 3.0 years after surgery. The distribution of current reported Gross Motor Function Classification System levels was as follows: I, 7%; II, 18%; III, 23%; IV, 36%; and V, 16%. Moreover, 56% of respondents were living with parents and 25% were living alone. Thirty-five percent were employed, and 39% were still in school. The mean overall SWLS score was 26.0 ± 7.3, indicating a high level of satisfaction with life. According to 65% of the patients, the SDR was helpful; 31% were uncertain about the procedures efficacy. Sixty-five percent would recommend the procedure to others. Fifty-eight percent reported excellent to very good health. Forty-four percent reported pain in the past week. Fifty-one percent reported chronic back pain in general. Logistic regression analysis suggested that an increased satisfaction with life was a predictor (p = 0.01) of an affirmative response to the question about recommending the procedure to others and that better overall health showed a trend toward being such a predictor (p = 0.08). Additional interventions were frequently performed after the SDR. Seventy-four percent of participants underwent orthopedic surgery. Thirty-eight percent were currently taking oral medications for tone, and 53% had received botulinum toxin injections for spasticity treatment. Thirteen patients (15%) had an intrathecal baclofen pump placed. CONCLUSIONS The majority of adults who had undergone SDR as children would recommend the procedure to others. Very few reported negative impressions of the procedure. Levels of satisfaction with life were generally high. Pain prevalence was similar to what has been reported in the literature for adults with cerebral palsy. Despite the SDR, further interventions, both surgical and nonsurgical, were used in the majority of patients.

Predictors of Cardiometabolic Risk Among Adults With Cerebral Palsy

OBJECTIVE To examine the independent association between various anthropometric indicators and standard clinical markers of cardiometabolic health risk among adults with cerebral palsy (CP). DESIGN Cross-sectional study. SETTING Clinical center for CP treatment and rehabilitation. PARTICIPANTS Adults with CP (N=43) with a mean age ± SD of 37.3±13.2 years, and Gross Motor Function Classification System (GMFCS) levels of I-V. INTERVENTIONS Not applicable. MAIN OUTCOME MEASURES Adults with CP were assessed for body mass index (BMI), waist circumference (WC), hip circumference (HC), waist-to-hip ratio (WHR), waist-to-height ratio (WtHR), and serum lipid profiles. Data were analyzed with multiple regression analysis and general linear models, and are reported as means ± SDs. RESULTS Mean BMI was 29.1±7.8kg/m(2). BMI was not associated with any measures of cardiometabolic risk. Using GMFCS categories (2 groups: GMFCS levels I-III and IV-V), BMI was significantly lower among GMFCS levels IV-V (24.2±6.2kg/m(2)) versus GMFCS levels I-III (30.1±7.6kg/m(2)). WC and WtHR were not correlated with any cardiometabolic outcomes. Conversely, measures of WHR were independently associated with various indices of risk, including total cholesterol to high-density lipoprotein (HDL) cholesterol ratio (r=.45; P<.05), HDL cholesterol (r=-.51; P<.01), and triglycerides (r=.40; P<.05), suggesting that greater WHR was indicative of elevated risk. CONCLUSIONS It is likely that WHR represents a stronger predictor of risk, because this measure was robustly and independently associated with 3 primary clinical markers of cardiometabolic health in adults 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 (β=-63.41 Hu, P<.001) and trabecular (β=-42.24 Hu, P<.001) BMDs and psoas major areas (β=-374.51mm(2), P<.001) and attenuation (β=-9.21 Hu, P<.001) after controlling for age, sex, and body mass. Adults with CP had greater VAT (β=3914.81mm(2), P<.001) and SAT (β=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 (β=-.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.

Agreement between Actual Height and Estimated Height Using Segmental Limb Lengths for Individuals with Cerebral Palsy

ObjectiveThe purpose of this study was to determine the agreement between actual height or segmental length and estimated height from segmental measures among individuals with cerebral palsy. DesignA convenience sample of 137 children and young adults with cerebral palsy (age 2–25 yrs) were recruited from a tertiary care center. Height, body mass, recumbent length, knee height, tibia length, and ulna length were measured. Estimated height was calculated using several common prediction equations. Agreement between measured and estimated height was determined using the Bland-Altman method. ResultsLimits of agreement were wide for all equations, usually in the range of ±10 cm. Repeatability of the individual measures was high, with a coefficient of variation of 1%–2% for all measures. The equation using knee height demonstrated a nonuniform difference in which height estimation worsened as overall height increased. ConclusionsAccurate measurement of height is important but very difficult in individuals with cerebral palsy. Segmental measures are highly repeatable and thus may be used on their own to monitor growth. However, when an accurate measure of height is needed to monitor nutritional status (i.e., for body mass index calculation), caution is warranted because there is only fair-to-poor agreement between actual height and estimated height.

Recumbent Cross-Training Is a Feasible and Safe Mode of Physical Activity for Significantly Motor-Impaired Adults With Cerebral Palsy

OBJECTIVE To examine the feasibility and potential benefits of using recumbent cross-training for nonambulatory adults with cerebral palsy (CP). DESIGN Observational. SETTING Clinical center for CP treatment and rehabilitation. PARTICIPANTS Significantly motor-impaired adults with CP (N=11) with a mean age ± SD of 36.3±13.2 years and Gross Motor Function Classification System (GMFCS) levels III and IV. INTERVENTIONS Participants completed a 40-minute session of aerobic exercise using the NuStep Recumbent Cross Trainer, in which resistance was progressively increased at 5-minute intervals. MAIN OUTCOME MEASURES Every 5 minutes during the exercise session, heart rate, blood pressure, oxygen consumption (VO(2)), energy expenditure, and respiratory exchange ratios (RERs) were recorded along with rating of perceived exertion. Immediately after, and 24 hours postexercise, participants received a standard survey to assess levels of pain and discomfort. RESULTS All participants were able to complete the 40-minute exercise protocol. Five of the 11 participants achieved a heart rate of at least 60% maximum throughout the duration, 10 participants had a significant elevation in VO(2) from baseline, and all participants had elevated RER values. Six participants reported pain during exercise, but only 2 reported pain after exercise was over. CONCLUSIONS The NuStep Recumbent Cross Trainer is a feasible exercise modality for significantly motor-impaired adults with CP, GMFCS III and IV. Moreover, this mode was sufficient to stimulate a significant cardiorespiratory response in all participants, and thus it and similar devices may serve as a viable option for aerobic exercise interventions in this population, to prevent obesity and related cardiometabolic consequences.

Self-report of pain in young people and adults with spastic cerebral palsy: interrater reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale ratings

People with cerebral palsy (CP) are often unable to express pain owing to cognitive or speech impairments. Reports that rely on observation can be inaccurate, because behaviours such as grimacing, common in people with spastic CP, resemble pain expressions. We examined preliminary validity and reliability of the revised Face, Legs, Activity, Cry, and Consolability (r‐FLACC) scale in people with spastic CP.

Poster 261 The Prevalence of Constipation as Reported by Adults With Cerebral Palsy

cepts as well as general questions about physiatry. Setting: This study was conducted at a private medical school in the Chicago area. Participants: The participants were firstand second-year medical students who attended the Musculoskeletal and Neuromuscular lecture day of their Physical Diagnosis class, respectively. Interventions: Each medical student class was given a pretest questionnaire to evaluate their baseline knowledge. Each class received a 1-hour physiatry-based lecture on basic concepts of either the musculoskeletal examination for first-years medical students or the neuromuscular examination for second-year medical students. The class was then divided into small groups, and the students attended a 2-hour hands-on interactive experience with physiatry residents to practice performing their physical examination skills. Main Outcome Measures: The participants of each class were administered an identical questionnaire, first as a pretest questionnaire and then as a posttest questionnaire. The posttest results were compared to the pretest results. Results: The average for first-year medical students’ posttest results improved by 24%, and the average for second-year medical students’ posttest results improved by 14%. In addition, 11 secondyear medical students and 15 first-year medical students requested more information about PM&R. Conclusions: This study indicates that a physiatry-based lecture and an interactive experience with physiatrists is an effective way to teach medical students the musculoskeletal and neuromuscular examination and introduce them to the field of PM&R. A moreeffective approach to teaching students may include exposing medical students to PM&R during their first year of medical school by teaching the musculoskeletal examination and then returning during their second year to teach the neuromuscular examination.

Poster 367 Adiposity Moderates the Association Between Gross Motor Functional System and Vitamin D Status Among Adults With Cerebral Palsy

CSHCN with functional limitations reported all types of participation restrictions compared with other CSHCN and non-CSHCN. Among CSHCN with limitations, 25.0% did not participate in organized activities, 73.0% did not work for pay, and 28.3% had not volunteered in the past year. For CSHCN, the odds of certain participation restrictions were higher for those with functional limitations, in fair or poor health, with depressed mood, living at or near the federal poverty level, and living in homes not headed by 2 parents. Conclusions: CSHCN with functional limitations and those with poorer health status have participation restrictions. Social disadvantage furthers the likelihood that CSHCN experience participation restrictions. This study identifies factors that may be amenable to clinical and policy-related interventions.

Adults With Cerebral Palsy Who Had a Rhizotomy as a Child: Long-term Follow-up

Disclosures: E. A. Hurvitz, None. Objective: To describe medical, functional and psychosocial outcomes and satisfaction of adults with cerebral palsy (CP) who had a rhizotomy as a child. Design: Retrospective cohort. Setting: Academic medical center. Participants: Adults and adolescents (16 years or older) with a diagnosis of CP who had a selective dorsal rhizotomy as a child or young adolescent. Interventions: Surveys administered in person or on the phone. Main Outcome Measures: Subjects were administered the Satisfaction with Life Scale (SWLS), Gross Motor Functional Classification Scale (GMFCS) and Manual Abilities Classification System. Subjects were asked about general health, and if they felt that the rhizotomy had affected their QOL. They were also asked if they would recommend the rhizotomy to others. Results: There were 31 subjects ages 16-39 years (mean 24.1 years; SD 5.2), mean age at rhizotomy 6.6 years (SD 4.0), mean follow up of 17.5 years (SD 3.0), and 53% men. About half the informants were self and half were parent or caregiver. The GMFCS levels were I, 6%; II, 16%; III, 26%; IV, 36%; V, 16%. Forty-eight percent thought they were in excellent or very good health, whereas 42% reported good health. Seven baclofen pumps had been placed some time after rhizotomy, but 2 were removed. Thirty-nine percent noted chronic pain, most commonly in the back and legs. The mean score on the SWLS was 26.0. Higher perception of health correlated with higher SWLS scores. There was also a trend (P .06) toward lower SWLS scores in those who reported pain. Sixty-four percent reported that the rhizotomy had improved their QOL, whereas 10% thought it decreased their QOL. Seventy-one percent would recommend the rhizotomy to others. A perception of improved QOL predicted a positive recommendation (P .01). Conclusions: Adults and adolescents were generally satisfied with childhood rhizotomy outcomes and would recommend it, especially if they had a perception of improved quality of life. These adults were generally healthy, although they had significant pain, common in adults with CP. The relationship between back pain and rhizotomy, which involves a laminectomy, needs more investigation.

Poster 213: Health and Function Data From an Adult Cerebral Palsy Clinic: Initial Report

Objective: To analyze long-term functional recovery and residual deficits in patients with Guillain-Barre Syndrome (GBS). Design: Prospective longitudinal follow-up study. Setting: Neurological rehabilitation unit of tertiary care hospital. Participants: Forty-one out of 330 patients (12.42%) admitted in the institute were transferred to the neurological rehabilitation unit for inpatient rehabilitation. Twenty-six patients (M:F,14:12) reporting at 1 year follow-up (63.41%) were included in the study (between Sept. 2005-Arpil 2008). Their functional recovery and residual deficits were recorded and analyzed. Interventions: Not applicable. Main Outcome Measures: Modified Barthel Index scores, Modified Rankin Scale and Hughes Disability Scale were used to assess functional disabilities at admission, discharge and follow-up. Results: Age ranged from 4-65 years (29.69 17.19). At admission, 21 patients had typical GBS and 5 had AMAN variant. Eight patients had bulbar weakness, 3 had autonomic dysfunction and 17 had facial palsy (uni/ bilateral). Nineteen patients (73.07%) had neuropathic pain needing medication with 6 requiring more than one drug. Five patients (19.2%) were prescribed wrist cock-up splint (WCS) for wrist drop and 15 patients (57.6%) prescribed ankle-foot orthoses-AFO (13 bilateral AFO) for foot drop. Twenty patients (77%) needed assistive devices also for locomotion at discharge. At 1 year follow-up, wrist drop and foot drop were still present in 1 and 4 patients, respectively, using orthoses. Modified Barthel Index scores, Modified Rankin Scale and Hughes Disability Scale were used to assess functional disabilities. Significant recovery was observed at the time of discharge and after 1 year when compared with discharge scores (P .001 each). Conclusions: GBS patients continue to show significant functional recovery for a long period. They have disability in the form of motor weakness even after 1 year and need to be followed up for longer duration.