A Conversation Between Past and Present Chairs of the American Board of Physical Medicine and Rehabilitation: Four Decades of History and the Future of Board Certification.

ABSTRACT: On the 75th anniversary of the founding of the American Board of Physical Medicine and Rehabilitation, 11 of the surviving chairs of the board convened virtually to reflect on the past 40 years of major trends for the accrediting body of physiatrists. The field rapidly expanded in the 1980s, driven by changes in the reimbursement environment. This rapid expansion drove an improvement in the caliber of residents choosing the field and in the quality of training programs. As physical medicine and rehabilitation evolved from a small- to medium-sized specialty, the board addressed many challenges: securing a credible position within the American Board of Medical Specialties; addressing a rising demand for subspecialty certification; improving training and exposure to physiatry; enhancing the quality of the accreditation process; and reducing the burden of accreditation on diplomates. The future development of physiatry includes improving diversity, equity, and inclusion, while restoring provider morale, well-being, and meaningfulness in work. Although challenges remain, physiatry as a field has grown to be well established through the board's efforts and respected within the larger medical community.

Long-term effect of botulinum toxin A on the hip and spine in cerebral palsy: A national retrospective cohort study in Taiwan.

OBJECTIVES: To investigate the effect of botulinum toxin A (BTA) on the development of hip dislocation and scoliosis, surgical rates for hip and spine, and mortality in cerebral palsy (CP). STUDY DESIGN: A cohort study was conducted using CP data from a Taiwan National Insurance Health Research Database. Diagnoses were defined using the International Classification of Diseases codes, 9th revision. Adjusted hazard ratios for outcomes were calculated using Cox regression analysis and adjusted for the following variables: BTA injection, sex, age, severities of CP, comorbidities, location, urbanization level, and level of care. RESULTS: A total of 1,405 CP children (670 female vs. 735 male), 281 in the BTA group and 1,124 in the controls, were followed-up for a mean of 5 years 4 months. There were no significant differences in the outcomes in both groups, in the incidence rates of hip dislocation and scoliosis, nor in the surgical rates for hip and spine surgery. Mortality rate in the BTA group was 0.49 times lower than that in the controls (p = 0.001). Moderate to severe types of CP had higher incidence rates of hip dislocation, scoliosis, hip surgery, spine surgery, and mortality. CONCLUSION: Moderate to severe types of CP had poorer outcomes in all aspects, including a higher risk of hip dislocation, scoliosis, surgical rate for hip and spine, and mortality. Although BTA injection in children with CP proved to not significantly reduce hip dislocation and scoliosis, it is considered safe as an anti-spasticity treatment and may be beneficial for survival.

Basics of bone metabolism and osteoporosis in common pediatric neuromuscular disabilities.

Bone modeling is a process that starts with fetal life and continues during adolescence. Complex factors such as hormones, nutritional and environmental factors affect this process. In addition to these factors, physical conditioning and medications that have toxic effects on bony tissue should be carefully considered in patient follow-up. Osteoporosis is a significant problem in pediatric population because of ongoing growth and development of skeletal system. Two types of osteoporosis are primary and secondary types and children with neuromuscular disabilities constitute a major group with secondary osteoporosis. Low bone mass in patients with cerebral palsy, spina bifida, and Duchenne muscular dystrophy cause increased bone fragility in even slight traumas. Maximizing peak bone mass and prevention of bone loss are very important to reduce the fracture risk in neuromuscular diseases. This article aims to review the determinants of bone physiology and bone loss in children with cerebral palsy, spina bifida, and Duchenne muscular dystrophy.

Rehabilitation issues in Charcot-Marie-Tooth disease.

Charcot Marie Tooth (CMT) disease is the most common hereditary sensorimotor neuropathy that has a slow onset. It presents usually in childhood, starting distally and from the lower limbs progressing to more proximal muscles. Due to the lack of curative medical treatments and the problematic outcomes of surgical intervention, rehabilitation continues to play a major role in treatment. This paper aims to summarize the rehabilitation approaches like aerobic, stretching and strengthening exercises. Orthotics is another important part of treatment that complete rehabilitative approaches. Orthotic devices that are currently being used and investigated in patients with CMT are also reviewed. The evidence shows that exercise is effective in improving strength and general fitness. Stretching is somewhat effective in maintaining range of motion. Orthotic devices are the mainstay of maintaining mobility and ambulation and upper extremity function.

Prevalence of Duchenne and Becker muscular dystrophies in the United States.

OBJECTIVE: To estimate prevalence of childhood-onset Duchenne and Becker muscular dystrophies (DBMD) in 6 sites in the United States by race/ethnicity and phenotype (Duchenne muscular dystrophy [DMD] or Becker muscular dystrophy [BMD]). METHODS: In 2002, the Centers for Disease Control and Prevention established the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) to conduct longitudinal, population-based surveillance and research of DBMD in the United States. Six sites conducted active, multiple-source case finding and record abstraction to identify MD STARnet cases born January 1982 to December 2011. We used cross-sectional analyses to estimate prevalence of DBMD per 10 000 boys, ages 5 to 9 years, for 4 quinquennia (1991-1995, 1996-2000, 2001-2005, and 2006-2010) and prevalence per 10 000 male individuals, ages 5 to 24 years, in 2010. Prevalence was also estimated by race/ethnicity and phenotype. RESULTS: Overall, 649 cases resided in an MD STARnet site during ≥1 quinquennia. Prevalence estimates per 10 000 boys, ages 5 to 9 years, were 1.93, 2.05, 2.04, and 1.51, respectively, for 1991-1995, 1996-2000, 2001-2005, and 2006-2010. Prevalence tended to be higher for Hispanic individuals than non-Hispanic white or black individuals, and higher for DMD than BMD. In 2010, prevalence of DBMD was 1.38 per 10 000 male individuals, ages 5 to 24 years. CONCLUSIONS: We present population-based prevalence estimates for DBMD in 6 US sites. Prevalence differed by race/ethnicity, suggesting potential cultural and socioeconomic influences in the diagnosis of DBMD. Prevalence also was higher for DMD than BMD. Continued longitudinal surveillance will permit us to examine racial/ethnic and socioeconomic differences in treatment and outcomes for MD STARnet cases.

Corticosteroid Treatments in Males With Duchenne Muscular Dystrophy: Treatment Duration and Time to Loss of Ambulation.

This population-based study examines the association between corticosteroid treatment and time to loss of ambulation, stratifying by treatment duration (short: 0.25-3 years, long: >3 years), among 477 Duchenne muscular dystrophy cases identified by the Muscular Dystrophy Surveillance Tracking and Research Network (MDSTARnet). Those cases who received short-term corticosteroid treatment had a time to loss of ambulation that was 0.8 years shorter (t test) and an annual risk of losing ambulation 77% higher than the untreated (Cox regression). Conversely, cases who received long-term corticosteroid treatment had a time to loss of ambulation that was 2 years longer and an annual risk of losing ambulation 82% lower than the untreated, up to age 11 years; after which the risks were not statistically different. The relationship of corticosteroids and time to loss of ambulation is more complex than depicted by previous studies limited to treatment responders or subjects who lost ambulation during study follow-up.

Complementary and alternative medicine for Duchenne and Becker muscular dystrophies: characteristics of users and caregivers.

BACKGROUND: Complementary and alternative medicine is frequently used in the management of chronic pediatric diseases, but little is known about its use by those with Duchenne or Becker muscular dystrophy. METHODS: Complementary and alternative medicine use by male patients with Duchenne or Becker muscular dystrophy and associations with characteristics of male patients and their caregivers were examined through interviews with 362 primary caregivers identified from the Muscular Dystrophy Surveillance, Tracking, and Research Network. RESULTS: Overall, 272 of the 362 (75.1%) primary caregivers reported that they had used any complementary and alternative medicine for the oldest Muscular Dystrophy Surveillance, Tracking, and Research Network male in their family. The most commonly reported therapies were from the mind-body medicine domain (61.0%) followed by those from the biologically based practice (39.2%), manipulative and body-based practice (29.3%), and whole medical system (6.9%) domains. Aquatherapy, prayer and/or blessing, special diet, and massage were the most frequently used therapies. Compared with nonusers, male patients who used any therapy were more likely to have an early onset of symptoms and use a wheel chair; their caregivers were more likely to be non-Hispanic white. Among domains, associations were observed with caregiver education and family income (mind-body medicines [excluding prayer and/or blessing only] and whole medical systems) and Muscular Dystrophy Surveillance, Tracking, and Research Network site (biologically based practices and mind-body medicines [excluding prayer and/or blessing only]). CONCLUSIONS: Complementary and alternative medicine use was common in the management of Duchenne and Becker muscular dystrophies among Muscular Dystrophy Surveillance, Tracking, and Research Network males. This widespread use suggests further study to evaluate the efficacy of integrating complementary and alternative medicine into treatment regimens for Duchenne and Becker muscular dystrophies.

Botulinum toxin a treatment in children with cerebral palsy: its effects on walking and energy expenditure.

OBJECTIVE: This study aimed to evaluate the effects of botulinum toxin A (BTX-A) treatment on energy expenditure and ambulation in children with cerebral palsy (CP) and to determine if energy expenditure measurement could be considered a useful tool for evaluating the effectiveness of BTX-A treatment for CP. DESIGN: The study included 16 pediatric CP patients with equinus deformity who were capable of walking without assistance (group with CP). The children with CP were administered BTX-A injections into the gastrocnemius muscle. All the children underwent three-dimensional gait analysis and energy expenditure measurement before injection and 8 wks after injection, the results of which were compared with those of children without CP (control group). RESULTS: After BTX-A treatment, oxygen consumption in the group with CP was significantly lower than at pretreatment and was similar to that in the control group (P > 0.05). In the group with CP, post-BTX-A treatment ankle joint dorsiflexion angle at initial contact, mid-stance, and mid-swing improved significantly (P < 0.05). CONCLUSION: BTX-A injected into the gastrocnemius muscle in children with CP not only reduced spasticity and improved ankle range of motion and walking pattern but also reduced energy consumption, resulting in functional improvement. Energy expenditure measurement can be considered an objective quantitative tool for clinical evaluation of the functional outcome of therapeutic interventions such as BTX-A.

Use of complementary and alternative medicine by males with Duchenne or Becker muscular dystrophy.

Use of complementary and alternative medicine by males with Duchenne or Becker muscular dystrophy was examined using interview reports from caregivers enrolled in the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Of the 200 caregivers interviewed, 160 (80%) reported "ever" using complementary and alternative medicine for their affected children. Mind-body medicine (61.5%) was most frequently used, followed by biologically based practices (48.0%), manipulative and body-based practices (29.0%), and whole medical systems (8.5%). Caregivers reporting use of whole medical systems had higher education and income levels compared with nonusers; affected males had shorter disease duration. Caregivers reporting use of mind-body medicine, excluding aquatherapy, had higher education level compared with nonusers. Overall, complementary and alternative medicine use was high; disease duration, education, and income levels influenced use. These findings have implications for developing clinical care protocols and monitoring possible interactions between complementary and alternative medicine and conventional medical therapies.

Use of corticosteroids in a population-based cohort of boys with duchenne and becker muscular dystrophy.

The use of corticosteroids for treatment of Duchenne and Becker muscular dystrophy in clinical practice from 1991 through 2005 was reviewed in a large population-based cohort (MD STARnet) of boys in 4 regional sites and 6 clinics of the United States. Corticosteroid use increased from 20% (11 of 56 individuals) in 1991 to 44% (93 of 218 individuals) in 2005. Average use varied by site and ranged from 15% to 49%. The median age of corticosteroid initiation was 6.9 years (range, 3.7-17.4 years). Dosage and growth information was available for 102 participants and showed a median dose as 0.729 mg/kg for prednisone and 0.831 mg/kg for deflazacort. T. The most common reasons that corticosteroids were discontinued included weight gain, behavioral side effects, and loss of ambulation, resulting in full-time wheelchair use. Substantial variations in clinical practice were identified among study sites.

[Management of spasticity in children with cerebral palsy]. / Beyin felçli çocuklarda spastisitenin tedavisi.

Management of spasticity is a major challenge to the rehabilitation team. The initial management has centered on the elimination of externally exacerbating causes, physical therapy, splinting and casting. Medical management has centered on anti-spasticity medication use, but more recently focal treatment methods including phenol blocks and botulinum toxin have been utilized. There has been an increased use of intrathecal baclofen in the management of refractory tone. Dorsal rhizotomy has been advocated for a selective population of children with spasticity. There is no standardized approach to spasticity management and this paper will discuss the importance of evidence-based treatment of spasticity that is adapted for the individual child.

Delayed diagnosis in duchenne muscular dystrophy: data from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet).

OBJECTIVE: To identify key factors for the delay in diagnosis of Duchenne muscular dystrophy (DMD) without known family history. STUDY DESIGN: The cohort comes from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet), a multistate, multiple-source, population-based surveillance system that identifies and gathers information on all cases of Duchenne and Becker muscular dystrophy born since 1982. We analyzed medical records of 453 Duchenne and Becker muscular dystrophy boys to document the time course and steps taken to reach a definitive diagnosis. RESULTS: Among 156 boys without known family history of DMD prior to birth, first signs or symptoms were noted at a mean age of 2.5 years. Concerns resulted in primary care provider evaluation of the child at a mean age of 3.6 years. Mean age at time of initial creatine kinase was 4.7 years. Mean age at definitive diagnosis of DMD was 4.9 years. CONCLUSIONS: There is a delay of about 2.5 years between onset of DMD symptoms and the time of definitive diagnosis, unchanged over the previous 2 decades. This delay results in lost opportunities for timely genetic counseling and initiation of corticosteroid treatment. We recommend checking creatine kinase early in the evaluation of boys with unexplained developmental delay.

Corticosteroid treatment and functional improvement in Duchenne muscular dystrophy: long-term effect.

OBJECTIVE: To determine and compare the long-term effects of prednisone and deflazacort on the functional status of children with Duchenne muscular dystrophy. DESIGN: A total of 49 boys with Duchenne muscular dystrophy, between the age of 12 and 15 yrs, who were observed over a 7-yr period were reviewed retrospectively. Eighteen had been treated with prednisone, 12 with deflazacort, and 19 had no drug treatment. All boys treated with steroids received medication for >2 yrs before losing their ambulation. Lower and upper limb motor functions, pulmonary function, prevalence of surgery for scoliosis, and side effects were compared. RESULTS: Boys in the steroid groups were significantly more functional and performed better on all tests than boys not treated (P < 0.05). There was no significant difference between the deflazacort- and prednisone-treated groups (P > 0.05). The number of boys having scoliosis surgery in treated groups was significantly less than nontreated boys (P < 0.05). The control group's pulmonary capacity was decreasing and significantly less than both prednisone- and deflazacort-treated boys. Both deflazacort and prednisone had beneficial effect on pulmonary function and scoliosis. Cataracts, hypertension, behavioral changes, excessive weight gain, and vertebral fracture were noted as serious side effects. CONCLUSIONS: Prednisone and deflazacort have a significant beneficial effect on slowing the disease progress. Their usage in Duchenne muscular dystrophy may prolong ambulation and upper limb function with similar potency. Both steroids also improve pulmonary function, in addition to delaying the need for spinal interventions, with similar therapeutic profiles.