Rehabilitation interventions for foot drop in neuromuscular disease.

BACKGROUND: "Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many neuromuscular diseases. OBJECTIVES: To conduct a systematic review of randomised trials of treatment for footdrop resulting from neuromuscular disease. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group Trials Register (July 2005), MEDLINE (January 1966 to July 2005), EMBASE (January 1980 to July 2005), AMED (January 1985 to July 2005) and CINAHL databases (January 1982 to July 2005). SELECTION CRITERIA: Randomised and quasi-randomised trials of physical, orthotic and surgical treatments for footdrop resulting from lower motor neuron or muscle disease and related contractures were included. People with primary joint disease were excluded. Interventions included a 'wait and see' approach, physiotherapy, orthotics, surgery and pharmacological therapy. The primary outcome measure was ability to walk whilst secondary outcome measures included dorsiflexor torque and strength, measures of 'activity' and 'participation' and adverse effects. DATA COLLECTION AND ANALYSIS: Methodological quality was evaluated by two authors using the van Tulder criteria. Three studies with altogether 139 participants were included in the review. Heterogeneity of the studies precluded pooling the data. MAIN RESULTS: Early surgery did not significantly affect walking speed in a trial including 20 children with Duchenne muscular dystrophy. After one year, the mean difference (MD) of the 28 feet walking time was 0.00 seconds (95% confidence interval (CI) -0.83 to 0.83) and the MD of the 150 feet walking time was -2.88 seconds, (95% CI -8.18 to 2.42). In a trial with altogether 26 participants with Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathy), long-term strength training significantly increased walking speed on a 6 metre timed walk (MD -0.70 seconds, 95% CI -1.17 to -0.23) but not on a 50 metre timed walk (MD -1.9 seconds, 95% CI -4.09 to 0.29). In a trial of a 24-week strength training programme in 28 participants with myotonic dystrophy, there was no significant change in walking speed on either a 6 or 50 metre walk. AUTHORS' CONCLUSIONS: Using the primary outcome of ability to walk, only one study demonstrated a positive effect and that was an exercise programme for people with Charcot-Marie-Tooth disease. Surgery was not significantly effective in children with Duchenne Muscular Dystrophy. More evidence generated by methodologically sound trials is required.

Multi-disciplinary rehabilitation for acquired brain injury in adults of working age.

BACKGROUND: Evidence from systematic reviews demonstrates that multi-disciplinary rehabilitation is effective in the stroke population, where older adults predominate. However, the evidence base for the effectiveness of rehabilitation following acquired brain injury (ABI) in younger adults is not yet established, perhaps because there are different methodological challenges. OBJECTIVES: To assess the effects of multi-disciplinary rehabilitation following ABI in adults, 16 to 65 years. To explore approaches that are effective in different settings and the outcomes that are affected. SEARCH STRATEGY: We used a wide range of sources including: Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (1966-2004), EMBASE (1988-2004), CINAHL (1983-2004), PsycLIT (1967-2004), AMED, the National Research Register 2004 and ISI Science Citation Index (1981-2004). SELECTION CRITERIA: Randomised controlled trials (RCTs) comparing multi-disciplinary rehabilitation with either routinely available local services or lower levels of intervention; or trials comparing intervention in different settings or at different levels of intensity. Quasi-randomised and quasi-experimental designs were also included, providing they met pre-defined methodological criteria. DATA COLLECTION AND ANALYSIS: Trials were selected by two authors independently, and their methodological quality rated, again by two independent authors. A third reviewer arbitrated when disagreements could not be resolved by discussion. A 'best evidence' synthesis was performed by attributing levels of evidence, based on methodological quality. Trials were sub-divided in terms of severity of ABI and the setting and type of rehabilitation offered. MAIN RESULTS: Ten trials were identified of good methodological quality and four of lower quality. Within the subgroup of predominantly mild brain injury, 'strong evidence' suggested that most patients make a good recovery with provision of appropriate information, without additional specific intervention. For moderate to severe injury, there is 'strong evidence' of benefit from formal intervention. For patients with moderate to severe ABI already in rehabilitation, there is strong evidence that more intensive programmes are associated with earlier functional gains, and 'moderate evidence' that continued outpatient therapy can help to sustain gains made in early post-acute rehabilitation. There is 'limited evidence' that specialist in-patient rehabilitation and specialist multi-disciplinary community rehabilitation may provide additional functional gains, but the studies serve to highlight the particular practical and ethical restraints on randomisation of severely affected individuals for whom there are no realistic alternatives to specialist intervention. AUTHORS' CONCLUSIONS: Problems following ABI vary; different services are required to suit the needs of patients with different problems. Patients presenting acutely to hospital with moderate to severe brain injury should be routinely followed up to assess their need for rehabilitation. Intensive intervention appears to lead to earlier gains. The balance between intensity and cost-effectiveness has yet to be determined. Patients discharged from in-patient rehabilitation should have access to out-patient or community-based services appropriate to their needs. Those with milder brain injury benefit from follow-up, and appropriate information and advice. Not all questions in rehabilitation can be addressed by traditional research methodologies. There are important questions still to be answered and future research should employ the most appropriate methodology.

Chronic granulocytic leukemia in a patient with a renal allograft.

A man received a cadaver renal allograft for end-stage renal failure. After 35 months of immunosuppressive therapy with azathioprine and prednisone, he developed septicemia and a high leukocyte count. In spite of successful treatment of the infection, the leukocyte count continued to rise and a diagnosis of Philadelphia chromosome positive chronic granulocytic leukemia was made. An increased incidence of malignant disease, especially lymphoreticular malignancy, is well described in immunosuppressed patients with allografts. However, the association of chronic granulocytic leukemia and immunosuppressive therapy previously has not been reported. An additional etiological factor in this patient may have been the extensive diagnostic radiological investigations undertaken in childhood. The recent addition of allopurinol to the immunosuppressive therapy has normalized the platelet and leukocyte counts, probably by potentiating mercaptopurine.

The role of diet in the pathogenesis and control of hyperlipidemia after renal transplantation.

Twenty-one Caucasian renal transplant recipients with hyperlipidemia and normal renal function were assessed with regard to their diet. Their average daily intake of energy and cholesterol was lower and the ratio of polyunsaturated to saturated fat higher in their diet than in that of a comparable urban Caucasian South African population. Nevertheless modification of their diets in one year produced a significant lowering of their body weight and a statistically significant drop in the mean plasma cholesterol (9.2 +/- 0.5 mmoles/l to 7.6 +/- 0.5 mmoles/l, P less than 0.05). Although the fall in plasma triglyceride in the group as a whole was not statistically significant (3.8 +/- 0.6 mmoles/l to 3.2 +/- 0.5 mmoles/l) the level did decrease substantially in 13 of the 21 patients (4.2 +/- 0.9 mmoles/l to 2.5 +/- 0.5 mmoles/l, P less than 0.01). It is suggested that diet has a definite role in the control of post-transplant hyperlipidemia but should probably be introduced immediately after immunosuppression is started and be coupled with a suitable exercise program for optimal effect.

Reliability of the American Medical Association guides' model for measuring spinal range of motion. Its implication for whole-person impairment rating.

STUDY DESIGN: Repeated measures design for intra- and interrater reliability. OBJECTIVES: To determine the intra- and interrater reliability of the lumbar spine range of motion measured with a dual inclinometer, and the thoracolumbar spine range of motion measured with a long-arm goniometer, as recommended in the American Medical Association Guides. SUMMARY OF BACKGROUND DATA: The American Medical Association Guides (2nd and 4th editions) recommend using measurements of thoracolumbar and lumbar range of movement, respectively, to estimate the percentage of permanent impairment in patients with chronic low back pain. However, the reliability of this method of estimating impairment has not been determined. METHODS: In all, 34 subjects participated in the study, 21 women with a mean age of 40.1 years (SD, +/- 11.1) and 13 men with a mean age of 47.7 years (SD, +/- 12.1). Measures of thoracolumbar flexion, extension, lateral flexion, and rotation were obtained with a long-arm goniometer. Lumbar flexion, extension, and lateral flexion were measured with a dual inclinometer. Measurements were taken by two examiners on one occasion and by one examiner on two occasions approximately 1 week apart. RESULTS: The results showed poor intra- and interrater reliability for all measurements taken with both instruments. Measurement error expressed in degrees showed that measurements taken by different raters exhibited systematic as well as random differences. As a result, subjects measured by two different examiners on the same day, with either instrument, could give impairment ratings ranging between 0% and 18% of the whole person (excluding rotation), in which percentage impairment is calculated using the average range of motion and the average systematic and random error in degrees for the group for each movement (flexion, extension, and lateral flexion). CONCLUSIONS: The poor reliability of the American Medical Association Guides' spinal range of motion model can result in marked variation in the percentage of whole-body impairment. These findings have implications for compensation bodies in Australia and other countries that use the American Medical Association Guides' procedure to estimate impairment in chronic low back pain patients.

Applications of functional assessment to musculoskeletal disability evaluation.

A review of the history of the disability determination for LBP suggests that the early focus upon disability as a direct "effect" of impairment has given way to an updated approach acknowledging the multitude of factors, many of which are subjective, that contribute to an individual's future return to work and disability status. In addition to the evaluation of the underlying impairment and pathology, physicians performing disability evaluations for LBP will be best served when they can assess the individual's functional status, and incorporate that information into their decision regarding the individual's current limitations and prognosis. As discussed above, many of the factors contributing to the evaluation of functional status, including pain, physical functioning, and affective status, are necessarily subjective. Nonetheless, the rehabilitation literature suggests that inclusion of this information into the disability determination using a patient-centered approach may provide the factors that are most potent with respect to patient prognosis and eventual return to work.

The acute attack of porphyria.

The acute neurological crisis is the most significant complication of variegate and acute intermittent porphyria and hereditary coproporphyria. If it is managed correctly, the mortality rate should be negligible. An outline is given of the major symptoms and signs encountered in the acute attack, and the therapy which should be used for their relief is discussed. Mention is made of forms of treatment which may decrease the activity of the haem biosynthetic pathway and thus specifically influence the clinical problems.

The UCT class of '32--50 years on.

In 1982 several members of the 1932 final-year M.B. Ch.B. class of the University of Cape Town met for a golden anniversary dinner. Apart from its nostalgic significance, it provided an opportunity to compare the career choices of that era with those of today. Unlike present graduates, 50 years ago graduating doctors tended not to specialize and tended to enter rural areas to practise. The significance of these changes for local medical manpower needs is discussed.

Breast-feeding in a socio-economically disadvantaged area of Cape Town. Part I. Analysis of breast-feeding patterns among clinic attenders.

A survey of infant-feeding practices in a socio-economically disadvantaged community in the Cape Peninsula was undertaken. By the age of 6 weeks 47,3% of the infants were receiving bottle feeds, and this had increased to 76,3% by the age of 7-12 weeks. Analysis of factors which might have influenced this pattern indicated that subjective dissatisfaction with the quantity and quality of milk dominated the mothers' reasons for stopping breast-feeding. Since contact between the local authority and mothers often took place only after breast-feeding had been discontinued, it is suggested that concentrating on education and support during the perinatal period may have a positive influence on this disturbing trend.

Breast-feeding in a socio-economically disadvantaged area of Cape Town. Part II. The introduction of an educational and support programme.

A study of the infant-feeding practices of a socio-economically disadvantaged community in the Cape Peninsula suggested the need for improved support and education of mothers during the perinatal period. Such a programme was introduced and the study was repeated 2 years later. It was found that a significant increase in the rate of breast-feeding had occurred. It is suggested that the programme might have made an important contribution to this improvement.

A programme to promote breast feeding in a socio-economically disadvantaged area of Cape Town, Republic of South Africa.

PIP: 169 mothers with infants under 6 weeks who attended the Local Authority Child Health Clinic in a socioeconomically disadvantaged area of Cape Town, South Africa, were interviewed in a study of infant feeding practices. Data was obtained on ages of infant of mother, place of birth, i.e., home, hospital, or midwife obstetrics unit, number of previous pregnancies and live children, last birth interval, method used for feeding the infant, reasons for introducing bottle or discontinuing breast, and age of the infant when contact was first made with Local Authority. On the basis of the interview, infants were classified as either breastfed, i.e., exclusively except for water given in a bottle or bottle fed, i.e., receiving no breast milk at all, or mixed fed, i.e., part breast and part bottle. Of the 89 mothers who fulfilled the criteria for breastfeeding, 80 were interviewed 6 weeks later. At the 1st interview, 89 of the 169 babies (52.7%) were being breastfed, 28 (16.6%) were receiving bottle feeds only, and 52 (30.7%) a mixture of both. When 80 of the breastfeeding mother were interviewed for the 2nd time 6 weeks later, 40 were still giving breast milk alone and 5 were not breastfeeding at all. Further analysis showed that the major decrease in the practice of breastfeeding occurred in the first 2 weeks of life, after which there was a slow but steady decline. The reasons given for introducing bottle feeding both before and after 6 weeks were "insufficient quantity" or "poor quality" milk. Other significant problems reported included local breast complications and return to work. The maternal age, parity, and interval between pregnancies did not appear to influence the choice of feeding practice. Approximately 2 years after a program was introduced to give increased support and education to mothers from health personnel a survey similar to the 1st study was undertaken in the same area. Socioeconomic, age range, and other demographic characteristics were similar and the questionnaire constant. At the 1st interview, 94 (75.8%) of the 124 mothers were still breastfeeding, a significant increase from the 1st survey. This trend continued when the 94 mothers who were originally breastfeeding were interviewed for the 2nd time, 6 weeks later. 48, 38.7% of the original number interviewed, were still breastfeeding in comparison with the previous study. The study showed a significant increase in breastfeeding rate.^ieng

Can we achieve accountability for long-term outcomes?

OBJECTIVE: To explore options for the development of a set of indicators to assess the long-term outcomes achieved by all people with a given disabling condition in a given population. DATA SOURCES: The review draws on empirical studies of predictive indicators, theoretical literature on long-term recovery processes, and literature from administrative science on the use of indicators in accountability systems. STUDY SELECTION: Studies were selected that explicitly sought to relate short-term and long-term outcomes or that explored the mediating factors in the relationship between impairment, disability, and handicap. DATA EXTRACTION: The focus of the review is on (1) empirical evidence of the relationship between short- and long-term outcomes, particularly in causal claims, and (2) theoretical analyses of the factors that mediate this relationship. DATA SYNTHESIS: Evidence is presented that certain outcome states can be considered thresholds that make the outcome usable and, hence, sustainable or that create the opportunity for further improvement. Such thresholds could meet the construct validity criteria necessary for measures that are to be used as indicators in an accountability system. The interaction between psychological and physical factors in setting thresholds means that both objective and subjective indicators are required in an indicator system. CONCLUSIONS: It may well be possible to develop a parsimonious set of population-based outcome indicators for people with disabilities. The key safeguards required are construct validity and the involvement of people with disabilities in both the development and use of the indicators.

Predicting hours of care needed.

This study investigated whether the number of hours of care needed by a person with disability could be predicted by his or her score on the Functional Independence Measure (FIM) or the Edinburgh Rehabilitation Status Scale (ERSS). Seventy-five subjects (age range, 19 to 65), from a variety of residential services, with neurological disabilities, were visited by an experienced observer to estimate the number of hours of care per week required, from whatever source, for the subject to manage adequately. A second observer, blind to the observations of the first, assessed the subjects' FIM and ERSS scores by interview with the subjects and their carers. Wide ranges of scores on both scales suggested that subjects with many different dependency levels were surveyed, confirmed by "required care hours" varying between 0 and 168 per week (median 18). Pearson's correlation coefficients were 0.36 for ERSS (p < 0.002) and -0.39 for FIM (p < 0.001). Analysis of the scattergrams identified three aberrant cases. Investigation of these showed they each had a high level of dependency due to supervision rather than physical care; exclusion of these cases resulted in much stronger correlations for the remaining 72 cases (ERSS, 0.61; FIM, -0.76). Both ERSS and FIM correlate well with hours of care required, but their association with hours of supervision is poor.

Serum ferritin levels in patients with porphyria cutanea tarda.

Serum ferritin was measured in 9 patients with overt porphyria cutanea tarda. In 6, the level was higher than normal. We confirm that moderate, but not gross iron overload is a feature of such patients.