Developing normalized strength scores for neuromuscular research.

INTRODUCTION: The Accurate Test of Limb Isometric Strength (ATLIS) device can reliably measure the strength of 12 muscle groups using a fixed load cell. The purpose of this study was to analyze ATLIS data from healthy adults to calculate an individual's predicted strength scores. METHODS: ATLIS data were collected from 432 healthy adults. Linear regression models were developed to predict each muscle group's strength. The R-squared statistic assessed variability accounted for by the models. RESULTS: Simple main effects models stratified by gender were used to establish regression equations for each muscle using factors of age, weight, and height. CONCLUSIONS: Normalizing raw strength scores controls for biometric factors, thus enabling meaningful comparisons between subjects and allowing each muscle to contribute equally to a summary score. Normalized scores are easily interpreted for broad clinical uses, and derived summary scores establish individuals' disease progression rates using a common scale, allowing for more efficient clinical trials.

Validation of a new strength measurement device for amyotrophic lateral sclerosis clinical trials.

INTRODUCTION: Strength measures with reduced variability and higher sensitivity could improve efficiency in clinical trials of amyotrophic lateral sclerosis (ALS). The Accurate Test of Limb Isometric Strength (ATLIS) was developed to precisely and conveniently measure force in 12 muscle groups. In this study we evaluate the reliability and validity of the ATLIS testing protocol. METHODS: Twenty healthy adults and 10 patients with ALS were tested twice by the same or by different evaluators to determine test-retest and interrater reliability. Twenty healthy adults were examined using ATLIS and a well-validated strength testing protocol (TQNE) to assess criterion-based validity. RESULTS: Mean absolute variation between tests was 8.6%, and intraclass correlation coefficients for each muscle group were high (range 0.82-0.99). The Pearson correlation coefficient of mean ATLIS and TQNE scores was 0.90. A subject survey demonstrated high user acceptance of ATLIS. CONCLUSIONS: ATLIS is convenient for patients and evaluators, produces precise strength measurements, and is easily moved between examining rooms.

Neurology education in Latin America and the World Federation of Neurology.

The World Federation of Neurology (WFN) is the international body representing the specialty of neurology in more than 100 countries/regions of the globe. The WFN was organized as a federation of national neurologic societies in 1957. A primary goal of the WFN is to assist low-resource countries in providing meaningful education for its neurologic health care providers and thus improve the neurologic health of its citizens. During the last decade the WFN has promoted the Neurology Education in Latin America establishing different strategies.

Developing a neurology training program in Honduras: a joint project of neurologists in Honduras and the World Federation of Neurology.

One of the major barriers to the provision of quality care for patients with neurological disorders in developing countries is a low ratio of neurologists per inhabitants, the World Health Organization recommends one neurologist per 100,000. In 1998 Honduras had one neurologist per 325,000 inhabitants and all the neurologists were trained outside the country. The Education Committee of the World Federation of Neurology (WFN), in collaboration with the Postgraduate Direction of the National Autonomous University of Honduras, the Honduran Neurological Association, and the Honduran Secretary of Health helped establish the country's first Neurology Training Program in 1998. This program was established using a problem- and epidemiological-oriented methodology with oversight by an external WFN review board. By 2006 the program has resulted in a 31% increase in the national neurologist ratio per inhabitant, significantly improved the quality of patient care and promoted research in the neurosciences. The Honduras Neurology Training Program has provided a valuable model for other developing countries with similar needs for neurological care. Based on this Honduras experience, members of the Education Committee of the WFN have established guidelines for neurology training programs in developing countries.

Promoting excellence in end-of-life care in ALS.

The type and quality of end-of-life care varies greatly in ALS; the time to initiate end-of-life care is not defined, and decision making is hampered by logistical and financial barriers. There has been no systematic review of these issues in ALS. The goals of this initiative are to: 1) improve end-of-life care for patients with ALS and families based on what limited evidence is available; 2) increase awareness, interest, and debate on the end-of-life care in ALS; and 3) identify areas needed for new prospective clinical research. The ALS Peer Workgroup reviewed the literature and 1) identified the current state of knowledge, 2) analysed the gaps in care, and 3) provided recommendations for standard of care and future research. It was shown that areas of investigation are needed on the incorporation of an interdisciplinary approach to care in ALS that includes: psychosocial evaluation and spiritual care; the use of validated instruments to assess patient and caregiver quality of life; and the establishment of proactive caregiver programs. Several public policy changes that will improve coverage for medical care, hospice, and caregiver costs are also reviewed. More clinical evidence is needed on how to provide optimal end-of-life care specifically in ALS.

Amyotrophic lateral sclerosis, lead, and genetic susceptibility: polymorphisms in the delta-aminolevulinic acid dehydratase and vitamin D receptor genes.

Previous studies have suggested that lead exposure may be associated with increased risk of amyotrophic lateral sclerosis (ALS). Polymorphisms in the genes for delta-aminolevulinic acid dehydratase (ALAD) and the vitamin D receptor (VDR) may affect susceptibility to lead exposure. We used data from a case-control study conducted in New England from 1993 to 1996 to evaluate the relationship of ALS to polymorphisms in ALAD and VDR and the effect of these polymorphisms on the association of ALS with lead exposure. The ALAD 2 allele (177G to C; K59N) was associated with decreased lead levels in both patella and tibia, although not in blood, and with an imprecise increase in ALS risk [odds ratio (OR) = 1.9; 95% confidence interval (95% CI), 0.60-6.3]. We found a previously unreported polymorphism in ALAD at an Msp1 site in intron 2 (IVS2+299G>A) that was associated with decreased bone lead levels and with an imprecise decrease in ALS risk (OR = 0.35; 95% CI, 0.10-1.2). The VDR B allele was not associated with lead levels or ALS risk. Our ability to observe effects of genotype on associations of ALS with occupational exposure to lead or with blood or bone lead levels was limited. These findings suggest that genetic susceptibility conferred by polymorphisms in ALAD may affect ALS risk, possibly through a mechanism related to internal lead exposure.

Neurologic services in sub-Saharan Africa: a case study among Zambian primary healthcare workers.

INTRODUCTION: In many parts of the developing world, access to physician consultation and neurologic expertise is limited or nonexistent. We conducted a survey among non-physician, primary healthcare workers (PHCWs) to determine the neurological needs and services in rural Zambia. METHODS: Semi-structured written questionnaire utilizing fill-in-the-blank, multiple-choice likert-scaled questions, and open-ended questions. RESULTS: Seizures were reported as the most common neurologic disorder by 66% of the PHCWs. Only 1/3 of PHCWs reported feeling adequately trained to care for seizures and seizure disorders. PHCWs reported even less expertise for other neurologic conditions. Over 40% of PHCWs surveyed work in primary care clinics without a physician available for consultation. Their patients must travel a median of 50 km to access a physician and geographic barriers are a frequent problem. In addition to difficulty physically accessing care, PHCWs reported that financial barriers to physician referral are substantial. Expenses cited include additional user fees for physician-level care, transportation costs, and the cost of maintaining the patient and/or family at a site distant from the home village. Traditional beliefs, social stigma, and discriminatory healthcare policies associated with neurologic conditions were also noted to deter and defer care and care seeking. CONCLUSIONS: PHCWs lack sufficient training and experience to care for the neurologic disorders in their patient populations, although such disorders are relatively common. Geographic, financial and cultural barriers substantially limit physician referrals. To assure at least a minimal quality of care for people with nervous system disorders in Zambia, PHCWs' neurologic education must be increased and barriers to physician referral decreased.

Lead exposure and amyotrophic lateral sclerosis.

BACKGROUND: Previous interview-based studies have suggested that exposure to neurotoxicants including metals might be related to ALS. METHODS: We evaluated the relation of lead exposure to ALS, using both biological measures and interviews, in a case-control study conducted in New England from 1993 to 1996. Cases (N = 109) were recruited at two hospitals in Boston, MA. Population controls (N = 256) identified by random-digit dialing were frequency-matched to cases by age, sex, and region of residence within New England. RESULTS: Risk of ALS was associated with self-reported occupational exposure to lead (odds ratio [OR] = 1.9; 95% confidence interval [CI] = 1.1-3.3), with a dose response for lifetime days of lead exposure. Blood and bone lead levels were measured in most cases (N = 107) and in a subset of controls (N = 41). Risk of ALS was associated with elevations in both blood and bone lead levels. ORs were 1.9 (95% CI = 1.4-2.6) for each microg/dl increase in blood lead, 3.6 (95% CI = 0.6-20.6) for each unit increase in log-transformed patella lead, and 2.3 (95% CI = 0.4-14.5) for each unit increase in log-transformed tibia lead. CONCLUSIONS: These results are consistent with previous reports and suggest a potential role for lead exposure in the etiology of ALS.