Neurobehavioral and family functioning following traumatic brain injury in children.
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Brain Injuries↗
Biomedical subjects
C M McDonald
Publications and source records attributed to C M McDonald.
Modifications to the traditional description of neurosegmental innervation in myelomeningocele.
The consistency between patterns of lower-limb muscle strengths in children with myelomeningocele and Sharrard's classic description of segmental innervation was examined in 291 patients. Although it has been assumed that medial hamstring innervation was from a similar neurosegmental level as gluteus medius, and gluteus maximus was from a similar level as gastrocnemius-soleus, the authors found that medial hamstring strength more frequently correlated with iliopsoas and quadriceps, and glutei with anterior tibialis. It is proposed that children with myelomeningocele be grouped according to specific muscle strength rather than by neurosegmental level.
Adolescent↗
Ambulatory outcome of children with myelomeningocele: effect of lower-extremity muscle strength.
The relationship between patterns of strength and mobility was studied in 291 children with myelomeningocele, graded as community ambulators, partial (household) ambulators and nonambulators. Iliopsoas strength was found to be the best predictor of ambulation, with the quadriceps, anterior tibialis and glutei also contributing significantly. Grade 0 to 3 iliopsoas strength was always associated with partial or complete reliance on a wheelchair. No patient with grade 4 to 5 iliopsoas and quadriceps function relied completely on wheelchairs and the majority were community ambulators. Grade 4 to 5 gluteal and anterior tibialis function was associated with community ambulation, without aids or braces. Deterioration in mobility was most common in those with strong iliopsoas/quadriceps and grade 0 to 3 gluteus medius, and was not age-related.
Adolescent↗
Symptoms of upper gastrointestinal dysfunction in Duchenne muscular dystrophy: case-control study.
A case-control study was undertaken to see if symptoms of upper gastrointestinal (UGI) (oropharyngeal, esophageal, and gastric) dysfunction occurred more frequently in males afflicted with Duchenne muscular dystrophy (DMD) than healthy controls. Subjects included 55 children with confirmed DMD and 55 age-matched controls without neuromuscular disease. All subjects and/or their parents responded to a standard set of questions concerning the frequency of symptoms of UGI dysfunction. Responses of the DMD and control groups were compared using the Wilcoxon signed rank test. A significantly higher percentage of DMD patients experienced nasal quality to the voice, dysphagia, choking while eating, the need to clear the throat during or after eating, heartburn, and vomiting during or after meals, than did controls. Only one symptom--heartburn--was found significantly more frequently in the 33 nonambulatory than the 22 ambulatory DMD subjects. These findings document that feeding difficulty and symptoms consistent with oropharyngeal, esophageal, and gastric dysfunction are more frequent in the DMD population than healthy, age-matched controls.
Adolescent↗
Assessment of muscle strength in children with meningomyelocele: accuracy and stability of measurements over time.
The strength of lower limb musculature, as determined by clinical muscle testing, influences assigned lesion levels, prognosis, and the subsequent orthopedic and rehabilitative management of children with meningomyelocele. The purpose of this study was to determine the accuracy of muscle testing at various ages based on clinical impressions of therapists; and to determine how the stability of measurements over time varies with age and muscle tested. A total of 3,084 examinations were performed on 825 children with meningomyelocele. Therapists coded the degree of accuracy of the last 1,282 examinations. Of these patients 177 with six or more serial examinations were studied by generating Spearman rank order correlation coefficients for comparison of strength measurements at given ages with median strengths during five-year follow-up periods. The percentages of patients who had examinations at a given age equal to, and within one grade of individual best estimates of ultimate muscle strength were also calculated. It was concluded that: therapists are markedly more confident about assessments in children five years and older; stability of muscle testing measurements over time generally increases from birth to age five or six when peak stability is approached; the probability that a single muscle test precisely predicts future muscle strength varies with age and muscle tested; and the probability that a muscle test will predict future strength to within one grade is not strongly influenced by age or muscle tested.
Child↗
Profiles of neuromuscular diseases. Duchenne muscular dystrophy.
One hundred and sixty-two patients with Duchenne muscular dystrophy (DMD) were followed over a 10-yr period to provide a profile of impairment and disability. The median height and weight of DMD boys were normally distributed before ages 9-10, but during the second decade height was markedly reduced, and weight was no longer normally distributed. Younger boys gained more weight than normals, whereas older individuals actually showed weight loss. Manual muscle test (MMT) measurements showed loss of strength in a fairly linear fashion from ages 5-13 yr, -0.25 MMT units per year. Upper extremity muscles were stronger than lower extremity muscles, proximal muscle groups were weaker than distal muscle groups, and extensor muscles were weaker than flexor muscles. There was no side dominance. There was a change in the rate of strength loss at 14-15 yr, and the decline slowed to only -0.06 MMT units per year. Although MMT and quantitative strength measurement profiles were similar, the latter were far more sensitive. In general, by the time strength declined to MMT grade 4, isometrically measured strength was 40-50% of normal control values. Joint contractures were rare before age 9, increased in frequency and severity with age, and were present in most individuals older than 13. Lower extremity contractures were strongly related to onset of wheelchair reliance, but there was no association between muscle imbalance around a joint. The prevalence of scoliosis increased between ages 11 and 16, with about 50% of the boys acquiring scoliosis between ages 12 and 15, corresponding to the onset of the adolescent growth spurt. Wheelchair reliance and scoliosis were both age-related. Percent predicted forced vital capacity declined at different yearly rates: ages 7-10, -0.3%; ages 10-20, -8.5%; after age 20, -6.2%. There was a direct relationship between percent predicted FVC and MMT scores. Decreased airway pressures, especially maximal expiratory pressure, appeared earlier than reductions in FVC but followed the same pattern. Thirty percent of the DMD boys had a history of respiratory complications, and the frequency increased with age. Spine deformity did not have a significant additive effect on the age-related decrement in pulmonary function. There was a high occurrence (79%) of abnormal electrocardiograms with age-related progression of some abnormalities, but only 30% of the patients had a history of cardiovascular complications. Functional level grades and timed motor performance measurements had a nonlinear relationship with strength and age.(ABSTRACT TRUNCATED AT 400 WORDS)
Adolescent↗
Profiles of neuromuscular diseases. Becker's muscular dystrophy.
Fifteen subjects with Becker's muscular dystrophy (BMD) were studied prospectively over a 10-yr period to provide a profile of impairment and disability. Proximal lower extremity musculature (particularly the hip and knee extensors), ankle dorsiflexors, and neck flexors showed significantly early weakness. Extensor muscle groups were weaker than flexor muscles at the elbow and knee. The mean manual muscle test (MMT) strength grade for all muscle groups combined was 3.7 +/- 0.8 MMT units. There was a slowly progressive decline in strength, only -0.31 MMT units per decade, and the decline was relatively equal in all muscle groups. There was not side dominance. Severe contractures did not appear to be a problem until after transition to a wheelchair, and scoliosis was rare. Restrictive lung disease occurred as a late complication in a small percentage of cases; however, maximal expiratory pressure was significantly reduced early in the disease. Only two individuals (19%) had severe restrictive lung disease and a history of significant respiratory complications. There was a slight but significant decline in forced vital capacity and maximal expiratory pressure with age and disease duration. The cardiomyopathy in BMD appeared to be disproportionately severe in some cases. Eleven individuals (73%) had abnormal electrocardiograms, and five (25%) had a history of significant cardiovascular complication, with no age or disease duration effect. Functional evaluations and timed motor performance tests showed only mild disability in most individuals. Mean intellectual and neuropsychologic function was within normal limits, but with a large variability in intelligence quotient scores. This report and others suggest a tremendous heterogeneity of severity among males with BMD.
Adolescent↗
Profiles of neuromuscular diseases. Limb-girdle syndromes.
Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.
Adolescent↗
Profiles of neuromuscular diseases. Facioscapulohumeral muscular dystrophy.
Data were collected prospectively over a 10-yr period from 53 subjects with facioscapulohumeral muscular dystrophy (FSHD) to provide a profile of impairment and disability. Manual muscle testing (MMT) indicated greater involvement of proximal musculature, although a subgroup demonstrated early weakness of the ankle dorsiflexors. Asymmetry of upper extremity musculature was noted, with greater weakness of selected dominant limb muscle groups. Weakness, in general, was relatively mild, with an overall mean MMT score of 3.7 units. The rate of strength loss was quite slowly progressive, a decline of only -0.22 MMT units per decade of age. An early age of onset was associated with greater likelihood of more severe and progressive weakness. Isometric and isokinetic quantitative strength testing revealed that all muscle groups were 36-68% weaker than a control population. Although nearly 50% of the subjects had vital capacity evidence of restrictive lung disease, only 13% had severe involvement, and only 22% had a history of pulmonary complications. There was no age or disease duration effect on pulmonary function measurements or complications. As with the other neuromuscular diseases, maximal expiratory pressure measurements were more sensitive than other pulmonary function tests. Abnormal electrocardiogram findings were rare and minor and not related to overt cardiac disease. Contractures were rare and mild. Thirty-five percent of the patients had spine deformity; however, most had hyperlordosis. Intellectual function was normal, and there were few abnormalities on personality tests. Functional testing demonstrated wide variation in disability with FSHD, but motor weakness uniformly translated into impaired motor performance skills. This profile demonstrates the clinical heterogeneity of FSHD.
Adult↗
Profiles of neuromuscular diseases. Hereditary motor and sensory neuropathy, types I and II.
Data were collected prospectively for an impairment and disability profile for 86 hereditary motor and sensory neuropathy, types I and II (HMSN) subjects over a 10-yr period. Our data confirm that HMSN is a slowly progressive disorder that has a very heterogeneous phenotypical expression. The disorder was characterized primarily by diffuse muscle weakness with prominent distal atrophy. The mean manual muscle test (MMT) strength grade for all muscle groups combined was 3.9 +/- 0.7 MMT units. There was a slowly progressive decline in strength, only -0.15 MMT units per decade. Distal muscle groups were weaker than proximal muscles, and the decline in strength of the ankle muscles was greater than for the proximal muscles. There was no side dominance. Anthropometric data revealed that distal atrophy may be masked by subcutaneous fat in female subjects. On average, HMSN subjects produced 20-40% less force than normal controls, using quantitative isometric and isokinetic strength measures, even when MMT scores were normal. Pulmonary and cardiac abnormalities were uncommon, as were spine deformity and joint contractures. Only 1 individual had severe restrictive lung disease, and 12 (14%) had a history of significant respiratory complications with no age or disease duration effect. As with the other neuromuscular diseases, maximum expiratory pressure was more affected than forced vital capacity. Fourteen individuals (30%) had abnormal electrocardiograms, and six (7%) had a history of significant cardiovascular complications with no age or disease duration effect. Kyphosis was the major spine deformity. Cardiopulmonary responses to exercise testing were markedly abnormal, showing reduced aerobic capacity. Functional evaluations and timed motor performance tests showed only mild disability in most individuals. With timed motor performance testing muscle weakness translated to impaired motor performance skills. Overall, mean scores on intellectual function and neuropsychologic profiles were normal.
Adult↗
Profiles of neuromuscular diseases. Spinal muscular atrophy.
Forty-five individuals with spinal muscular atrophy (SMA) types II and III were evaluated prospectively over a 10-yr period to develop an impairment and disability profile. SMA II subjects showed marked weakness and progressive decline of strength. Mean manual muscle test (MMT) score for all muscles combined was 2.3 +/- 0.6, with a decline in strength of -0.24 MMT units per decade. SMA III individuals had a relatively static or very slowly progressive course and were far stronger. Mean MMT score for all muscles combined was 3.8 +/- 0.7, and the decline in strength per decade was not significant. In both types proximal weakness was greater than distal, but there was greater involvement of the lower extremities and the extensor muscle groups only in SMA II. Contractures, progressive scoliosis, and restrictive lung disease (RLD) were present in most of the SMA II individuals, but these complications were rare in SMA III. Maximal expiratory pressures were affected earlier and to a greater degree than vital capacity. Seventy-eight percent of those with SMA II had scoliosis with a mean Cobb angle of the primary curve of 62 +/- 37 degrees. Forty-one percent had severe RLD, and 17% had moderate RLD. In both types, 63% had abnormal electrocardiograms although most had minor findings. Timed motor performance and functional evaluations indicated that muscle weakness translated to substantial disability in both SMA II and III, with more severe impairment noted in SMA II. Neither type was associated with abnormal means scores on intellectual and neuropsychologic test batteries.
Adolescent↗
Profiles of functional recovery in fifty traumatically brain-injured patients after acute rehabilitation.
Research to demonstrate the efficacy of head injury rehabilitation is important at a time when cost-containment efforts are intensifying. A useful tool that would predict the functional improvement during hospitalization and length of stay (LOS) of persons with traumatic brain injury would be of benefit to patients and their families, insurance carriers, and rehabilitation specialists. This study examines functional improvements made by 50 traumatic brain-injured patients admitted to the rehabilitation unit at the University of California, Davis, Medical Center (UCDMC) as measured by the UCDMC Davis Functional Status Measure (DFSM), which was adapted from the Functional Independence Measure (FIM). The DFSM incorporates additional items to provide a more thorough measure of skills to be rehabilitated. The purpose of this study was to compare scores and profiles on the DFSM items obtained by patients with LOS greater than and less than and equal to the median rehabilitation LOS (23 days). Relationships were explored among admission DFSM scores, LOS for rehabilitation, discharge destination, and functional outcome. Results indicate that patients admitted to the rehabilitation unit attained a similar profile or level of function by discharge, regardless of admission Glasgow Coma Scale scores or admission DFSM scores. There were no significant differences in admission Glasgow Coma Scale score, age, acute LOS, or discharge disposition between the LOS groups. There was a significant difference in median admission DFSM score in 26 of 31 categories between the LOS groups. There was a significant difference in median DFSM change (admission to discharge) in 24 of 31 categories between the LOS groups. The admission DFSM total score was inversely proportional to the length of stay, with a correlation coefficient of 0.78. DFSM change and admission to discharge was linearly correlated with LOS (R = 0.66). The DFSM documents functional outcome and measures gains during inpatient rehabilitation. The DFSM profile is helpful in predicting the LOS needed to achieve those gains.
Activities of Daily Living↗
Preserving function in Duchenne dystrophy with long-term pulse prednisone therapy.
We report a case of a patient with Duchenne muscular dystrophy who was treated with intermittent pulse prednisone for severe asthma from age 3 to 17 yr and had remarkable preservation of skeletal muscle function. He had a maternal uncle with Duchenne muscular dystrophy who had the identical familial deletion mutation and died at age 19 of respiratory failure. Compared with his untreated uncle, our patient remains partially ambulatory at age 20. This case provides interesting, albeit anecdotal, evidence of considerable clinical benefit from pulse prednisone used on a much longer term basis than has been previously studied and promotes the need for further investigation on this type of therapy in Duchenne muscular dystrophy.
Activities of Daily Living↗