The Effect of Facial Burns on Long-Term Outcomes in Young Adults: A 5-Year Study.

Long-term functional outcomes in young adults with facial burns remain poorly studied. This 5-year (2003-2008) prospective multicenter study includes burn survivors (age 19-30 years) who completed the Young Adult Burn Outcome Questionnaire (YABOQ) from 0 to 36 months after baseline survey administration. A composite canonical score was developed from 15 YABOQ domains using discriminant analysis, maximizing the difference at the baseline between burn-injured patients with face involved and not involved. A generalized linear model with the generalized estimation equation technique was used to track the changing pattern of the composite score over time. Individual domain scores with high correlation to the canonical score were used to evaluate recovery patterns in facial burns. A total of 153 burned (31% with face burns) and 112 nonburned subjects completed 620 questionnaires. Canonical analysis showed that early postburn, facial burns were associated with a difference in outcome, but this overall difference diminished over time. Regression analysis showed that for survivors with facial injury, Emotion and Sexual Function scores were persistently lower (worse), while Religion scores were persistently higher. Satisfaction with Role was initially better than the nonface burned group, but over time got worse, while Perceived Appearance was initially worse in the face burned group but this difference diminished over time. Social Function Limited by Appearance was initially similar between the groups, but over time the group with face burns scored lower. The overall difference in recovery between survivors with and without facial burns diminished over time while the individual domains had various patterns of recovery.

Speech production gains following constraint-induced movement therapy in children with hemiparesis.

PURPOSE: The purpose of this study was to investigate changes in speech skills of children who have hemiparesis and speech impairment after participation in a constraint-induced movement therapy (CIMT) program. While case studies have reported collateral speech gains following CIMT, the effect of CIMT on speech production has not previously been directly investigated to the knowledge of these investigators. METHODS: Eighteen children with hemiparesis and co-occurring speech impairment participated in a 21-day clinical CIMT program. The Goldman-Fristoe Test of Articulation-2 (GFTA-2) was used to assess children's articulation of speech sounds before and after the intervention. Changes in percent of consonants correct (PCC) on the GFTA-2 were used as a measure of change in speech production. RESULTS: Children made significant gains in PCC following CIMT. Gains were similar in children with left and right-sided hemiparesis, and across age groups. CONCLUSION: This study reports significant collateral gains in speech production following CIMT and suggests benefits of CIMT may also spread to speech motor domains.

Quantifying Risk Factors for Long-Term Sleep Problems After Burn Injury in Young Adults.

Restorative sleep is an important component of quality of life. Disturbances in sleep after burn injury were reported but all based on uncontrolled or nonstandardized data. The occurrence and the effect of long-term sleep problems in young adult burn survivors have not been well defined. This 5-year (2003-2008) prospective multicenter longitudinal study included adults with burn injuries ages 19 to 30 years who completed the Young Adult Burn Outcome Questionnaire (YABOQ) up to 36 months after injury. The items measured 15 patient-reported outcomes including physical, psychological, and social statuses and symptoms such as itch and pain. Scores of these 15 YABOQ outcome domains were standardized to a mean of 50 and a SD of 10 based on an age-matched nonburned reference group of young adults. Sleep quality was assessed using the item 'How satisfied are you now with your sleep,' rated by a 5-point Likert scale. Patients responding with very and somewhat dissatisfied were classified as having sleep dissatisfaction and the remaining as less or not dissatisfied. The associations between sleep dissatisfaction (yes/no) and YABOQ outcome domains were analyzed longitudinally using mixed-effect generalized linear models, adjusted for %TBSA burned, age, gender, and race. Generalized estimating equations were used to take into account correlated error resulting from repeated surveys on each patient over time. One hundred and fifty-two burn survivors participated in the YABOQ survey at baseline and during the follow-up who had at least one survey with a response to the sleep item. Among them, sleep dissatisfaction was twice as prevalent (76/152, 50%) when compared with the nonburned reference group (29/112, 26%). The likelihood of a burn survivor being dissatisfied with sleep was reduced over time after the burn injury. Sleep dissatisfaction following burns was significantly associated, in a dose-dependent manner, with increasing burn size (P = .001). Better sleep was associated with better outcomes in all domains (P < .05) except Fine Motor Function, and this association was significantly more apparent in the longer term compared with the shorter term with the same domains (P < .05). Dissatisfaction with sleep is highly prevalent following burn injuries in young adults. Lower satisfaction with sleep is associated with poorer scores in nearly all quality of life measures. Satisfaction with sleep should be addressed during the long-term clinical follow-up of young adults with burn injuries. Further research should be undertaken to understand the components of sleep quality that are important to burn survivors and which ones might be modified and tested in future intervention studies.

Functional outcomes in Rett syndrome.

AIM: To relate functional outcomes to mutation type and age at evaluation in patients with Rett syndrome (RTT). METHOD: We identified 96 RTT patients with mutations in the MECP2 (methyl-CpG-binding protein 2) gene. Chart analysis, clinical evaluation, and functional measures were completed. RESULTS: Among 11 mutation groups, a statistically significant group effect of mutation type was observed for self-care, upper extremity function, and mobility, on standardized measures administered by occupational and physical therapists. Patients with R133C and uncommon mutations tended to perform best on upper extremity and self-care items, whereas patients with R133C, R306C and R294X had the highest scores on the mobility items. The worst performers on upper extremity and self-care items were patients with large deletions, R255X, R168X, and T158M mutations. The lowest scores for mobility were found in patients with T158M, R255X, R168X, and R270X mutations. On categorical variables as reported by parents at the time of initial evaluation, patients with R133C and R294X were most likely to have hand use, those with R133C, R294X, R306C and small deletions were most likely to be ambulatory, and those with R133C were most likely to be verbal. INTERPRETATION: Functional performance in RTT patients may relate to the type of mutation. Knowledge of these relationships is useful for developing appropriate rehabilitation strategies and prognosis.

Recovery trajectories after burn injury in young adults: does burn size matter?

The impact of burn size on mortality is well known, but the association of burn size with the trajectories of long-term functional outcomes remains poorly studied. This prospective multi-center study included burned adults ages 19 to 30 years who completed the Young Adult Burn Outcome Questionnaire at initial baseline contact, 2 weeks, and at 6 and 12 months after initial questionnaire administration. Non-burned adults of comparable ages also completed the questionnaire as a reference group. The association between functional recovery and TBSA burned was analyzed longitudinally using generalized linear models with the generalized estimation equation technique. Functional status was characterized in 15 domains: physical function, fine motor function, pain, itch, social function limited by physical function, perceived appearance, social function limited by appearance, sexual function, emotion, family function, family concern, satisfaction with symptom relief, satisfaction with role, work reintegration, and religion. Scores were standardized to a mean of 50 and a SD of 10 based on non-burned controls. There were 153 burned and 112 non-burned subjects with a total of 620 questionnaires. TBSA burned was 11 ± 14% (mean ± SD); 31% had face involvement and 57% had hand involvement. The lag time from burn injury to questionnaire administration was on average 7 ± 7.7 months, with a maximum of 36 months. Lower recovery levels were associated with increasing burn size for physical function, pain, itch, work reintegration, emotion, satisfaction with symptom relief, satisfaction with role, family function, and family concern (P value ranged from .04-<.0001). No significant differences in recovery levels were found with increasing burn size for fine motor function, social function limited by physical function, sexual function, and religion; these areas tracked toward the age-matched non-burned group regardless of burn size. Perceived appearance and social function limited by appearance remained below the non-burn levels throughout the 3-year period regardless of burn size. Three-year recovery trajectories of survivors with larger burn size showed improvements in most areas, but these improvements lagged behind those with smaller burns. Poor perceived appearance was persistent and prevalent regardless of burn size and was found to limit social function in these young adult burn survivors. Expectations for multidimensional recovery from burns in young adults can be benchmarked based on burn size with important implications for patient monitoring and intervening in clinical care.

Benchmarks for multidimensional recovery after burn injury in young adults: the development, validation, and testing of the American Burn Association/Shriners Hospitals for Children young adult burn outcome questionnaire.

Although data exist on burn survival, there are little data on long-term burn recovery. Patient-centered health outcomes are useful in monitoring and predicting recovery and evaluating treatments. An outcome questionnaire for young adult burn survivors was developed and tested. This 5-year (2003-2008) prospective, controlled, multicenter study included burned and nonburned adults ages 19 to 30 years. The Young Adult Burn Outcome Questionnaires were completed at initial contact, 10 days, and 6 and 12 months. Factor analysis established construct validity. Reliability assessments used Cronbach α and test-retest. Recovery patterns were investigated using generalized linear models, with generalized estimating equations using mixed models and random effects. Burned (n = 153) and nonburned subjects (n = 112) completed 620 questionnaires (47 items). Time from injury to first questionnaire administration was 157 ± 36 days (mean ± SEM). Factor analysis included 15 factors: Physical Function, Fine Motor Function, Pain, Itch, Social Function Limited by Physical Function, Perceived Appearance, Social Function Limited by Appearance, Sexual Function, Emotion, Family Function, Family Concern, Satisfaction With Symptom Relief, Satisfaction With Role, Work Reintegration, and Religion. Cronbach α ranged from 0.72 to 0.92, with 11 scales >0.8. Test-retest reliability ranged from 0.29 to 0.94, suggesting changes in underlying health status after burns. Recovery curves in five domains, Itch, Perceived Appearance, Social Function Limited by Appearance, Family Concern, and Satisfaction with Symptom Relief, remained below the reference group at 24 months. The Young Adult Burn Outcome Questionnaire is a reliable and valid instrument for multidimensional functional outcomes assessment. Recovery in some domains was incomplete.

Treatment of cerebellar cognitive affective syndrome with aripiprazole.

This report describes a patient who developed agitation, disorientation, visual hallucinations, inappropriate verbal outbursts, and impaired memory following resection of a choroid plexus papilloma. No medical, neurologic, or metabolic disorders unrelated to the surgery were identified. Five weeks following surgery, treatment with aripiprazole, a partial dopamine agonist, was started to address the delirious state. Improvements in agitation, orientation, memory, and executive functions, as well as a decrease in emotional lability, began within twenty-four hours and continued over the remainder of the inpatient hospitalization. Five months after initial resection, aripiprazole was discontinued without worsening of cognitive or emotional functions. Persistent difficulties with working memory, planning, judgment, and visuospatial skills were noted on neuropsychological examination six months following tumor removal. This case illustrated the therapeutic benefit of aripiprazole for treatment of mental status changes associated with resection of a posterior fossa tumor.

Pediatric constraint-induced movement therapy: a promising intervention for childhood hemiparesis.

Experimental and clinical evidence is accumulating that supports the assertion that the damaged human brain is capable of responding to sensory stimulation in a sufficient manner to result in sustainable and useful benefits. The intensity and duration of therapeutic maneuvers that elicit improvement are under active investigation. Recent studies in animals, adults, and children with hemiparesis have shown that constraint of the less involved upper limb coupled with a behavioral program that repetitively encourages graded unilateral movements can result in long-term "new" functional activities. Constraint-induced movement therapy (CIMT) is a promising approach for treatment of children with stroke-related hemiparesis from either prenatal or postnatal causes due to the enhanced neuroplasticity of the brain during early life.

Botulinum toxin type A treatment in neurogenetic syndromes.

PURPOSE: To review the use of therapeutic botulinum toxin type A (BoNT-A) treatments in uncommon neurogenetic syndromes. METHOD: A retrospective questionnaire and interview study of a selected case series to assess the efficacy and safety following initial BoNT-A treatment (50-400 units per subject) was conducted to determine the response of families to treatment. Twelve male and six female subjects with ages from 2-19 years were included. The reasons for treatments were based on both patient-related and caregiver-related objectives. Satisfaction with achievement of stated goals was assessed by follow-up interviews. RESULTS: Beneficial effects were reported in 56%, some effects in 22% and no to minimal effects in 22%. The duration of effect ranged from 10 days to 12 months with an average of 3.16 months. Ten families would repeat the injections as needed, four would not and four were not sure. Unanticipated effects of BoNT-A treatments were reported by some families. Adverse effects did not occur with the doses that were used. CONCLUSIONS: The results suggest that obtaining family input may be useful when treating spasticity in unusual circumstances. The use of BTX-A in uncommon neurogenetic syndromes was supported by the majority of families interviewed.

Hip migration percentage in children with cerebral palsy treated with botulinum toxin type A.

OBJECTIVE: To determine hip radiographic findings in children with cerebral palsy (CP) treated with botulinum toxin type A (BTX-A). DESIGN: Retrospective chart review with correlation to radiographic findings. SETTING: Academic center. PARTICIPANTS: Sixteen subjects with CP. INTERVENTION: BTX-A treatment to adductor muscles. MAIN OUTCOME MEASURE: The Reimers hip migration percentage before and after BTX-A. RESULTS: Thirty-two hips in 16 children with CP were treated. We examined the effect of initial migration percentage and initial migration percentage age on the change in migration percentage after BTX-A injection. A significant effect for initial migration percentage (<30% or >/=30%; F=19.05, P <.001) and a significant interaction between initial migration percentage and initial migration percentage age (F=7.5, P <.01) was noted. Initial migration percentage age (24 mo) was not significant (F=.95, P =.34). Patients who had an initial migration percentage of 30% or more and were less than 24 months old were more likely to have a decrease in migration percentage after BTX-A injection compared with patients who were older than 24 months and who had an initial migration percentage of 30% or more. CONCLUSIONS: Improvement in hip migration percentage after BTX-A injection is a function of age and the initial migration percentage. BTX-A injections to adductor muscles may be beneficial for some children with CP.

The emerging role of therapeutic botulinum toxin in the treatment of cerebral palsy.

Therapeutic injections of botulinum toxin have been used for the past decade to decrease muscle tone in children with cerebral palsy. Although this unique intervention has been shown to provide safe localized spasticity reduction, functional benefits have been more difficult to demonstrate. Appropriate selection of patients and clearly defined goals are key factors in the success of a treatment program. The therapeutic approach to treating children with cerebral palsy should include a variety of complementary interventions that address the effect of abnormal muscle tone on the abilities of these children as they grow and develop.

Motor outcome differences between two groups of children with spastic diplegia who received different intensities of early onset physiotherapy followed for 5 years.

The objective of this study is to determine the clinical effectiveness of early onset long-term intensive physiotherapy on motor development in children with spastic diplegic cerebral palsy (CP). The study was a non-randomized cohort study with 62 months (mean) follow-up. The participants were ten infants who were first examined before 3 months of age corrected for prematurity. All had a gestational age of less than 33 weeks and a birth weight of less than 2000 g. Brain magnetic resonance imaging revealed periventricular white matter injury in nine subjects and moderate grade bilateral porencephaly in one. Five completed a full course of training of 52 months (mean), two did not receive therapy, and three received an insufficient course of therapy. The study was conducted at the Regional Center for Children with Disabilities including outpatient clinics and a school for children with special needs. The Vojta Method was used, which is an extensive family oriented physiotherapy program which uses isometric strengthening of muscles with tactile stimulation. Subjects were evaluated for the highest motor developmental level at the outcome evaluation 59 months (mean) after initiation of therapy. Four of the five who completed training could either stand still for 5 s or walk at the time of the outcome evaluation 52 months after the beginning of the therapy program. None of the five subjects with no training or insufficient training could accomplish this task when evaluated 64 months following therapy initiation. This was a statistically significant difference (P = 0.0278). A consistently applied physiotherapy program resulted in better motor outcomes in this group of children at risk for developing spastic diplegic CP.