Reliability and validity of the Wisconsin HSS Quality Of Life inventory in traumatic brain injury.

The present investigation examined the psychometric properties of the Wisconsin HSS Quality of Life Inventory (WI HSS QOL) when used in the study of traumatic brain injury (TBI). The WI HSS QOL is a theoretically based measure that assesses QOL via level of need satisfaction as conceptualized in Maslow's theory of human needs. Both internal consistency and test-retest reliability were demonstrated in a group of individuals with moderate to severe TBI. The demonstrated consistency of the participants' self-reports indicates that QOL can be reliably assessed from the survivor's perspective. Cluster analytic results generally supported the construct validity of the measure's use in TBI. The cluster solution reflected four of the five theoretical need categories; however, the fifth category, "self-actualization" needs, did not emerge as a single cluster as expected. This deviation from the theoretical model may relate to participants' difficulties in understanding the relatively abstract items designed to assess the concept of self-actualization. Overall, the WI HSS QOL holds significant promise as a measure of QOL in TBI.

Cognitive, psychosocial, and educational issues in neuromuscular disease.

There is a broad range of cognitive function in persons with neuromuscular disease, both between and within the various types. Those groups exhibiting little or no cognitive impairment included FSHD, LGS, BMD, SMA, and HMSN. DMD and MD--particularly C-MD-groups did show deficits. The data on HSCA are too minimal to reach any firm conclusion at this time. Specific patterns of deficits in DMD have been proposed, but need further research. No specific correlates with diminished intellectual function have yet been found, and the search for a cause of the intellectual deficit has as yet been unsuccessful. Nevertheless, the impact of NMD on psychosocial and educational function of both children and adults is significant. It is recommended that supportive services be provided by medical professionals specifically trained in such helping professions as psychology to lessen the impact of these chronic progressive diseases and thus to improve the quality of life of persons with NMD.

Profiles of neuromuscular diseases. Myotonic dystrophy.

Ninety-two individuals with myotonic dystrophy (MD) were evaluated prospectively over a 10-yr period and separated into two types, 75 noncongenital (NC-MD) and 17 congenital (C-MD) MD. Muscle weakness was relatively mild and similar in both types, 4.0 +/- 0.7 manual muscle test (MMT) scores for NC-MD and 3.8 +/- 0.7 in C-MD. However, weakness was progressive in the former, -0.36 MMT units per decade, and nonprogressive in C-MD. Weakness was usually generalized in both types, with no significant differences between upper and lower extremities or the proximal and distal muscles. Flexor and extensor differences were variable. Quantitative strength measurements showed a similar pattern but were more sensitive showing marked strength losses of 40-50% in muscle groups with MMT scores of four or more. There was a high frequency (47%) of relatively mild, nonprogressive scoliosis in C-MD, whereas spine deformity was unusual in NC-MD. Contractures, usually at the ankles, were also more common in C-MD. In NC-MD and C-MD, respectively, there was a low frequency of severe restrictive lung disease (14 and 20%) but a high percentage of significant electrocardiographic (ECG) abnormalities (75 and 81%), including conduction defects. There was a marked difference between the two types of MD in intellectual and cognitive function. Seventy-five percent of C-MD subjects showed impairment, frequently severe, compared with 35% impairment, usually mild, for NC-MD individuals. Functional evaluation was not markedly affected, but timed motor performance showed significant disability especially for individuals with C-MD.

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)