Care Of Amyotrophic Lateral Sclerosis Patients Research Articles

Amyotrophic Lateral Sclerosis and Pain: A Narrative Review from Pain Assessment to Therapy.

Amyotrophic lateral sclerosis (ALS) is the most frequent neurodegenerative disease of the motor system that affects upper and lower motor neurons, leading to progressive muscle weakness, spasticity, atrophy, and respiratory failure, with a life expectancy of 2-5 years after symptom onset. In addition to motor symptoms, patients with ALS have a multitude of nonmotor symptoms; in fact, it is currently considered a multisystem disease. The purpose of our narrative review is to evaluate the different types of pain, the correlation between pain and the disease's stages, the pain assessment tools in ALS patients, and the available therapies focusing above all on the benefits of cannabis use. Pain is an underestimated and undertreated symptom that, in the last few years, has received more attention from research because it has a strong impact on the quality of life of these patients. The prevalence of pain is between 15% and 85% of ALS patients, and the studies on the type and intensity of pain are controversial. The absence of pain assessment tools validated in the ALS population and the dissimilar study designs influence the knowledge of ALS pain and consequently the pharmacological therapy. Several studies suggest that ALS is associated with changes in the endocannabinoid system, and the use of cannabis could slow the disease progression due to its neuroprotective action and act on pain, spasticity, cramps, sialorrhea, and depression. Our research has shown high patients' satisfaction with the use of cannabis for the treatment of spasticity and related pain. However, especially due to the ethical problems and the lack of interest of pharmaceutical companies, further studies are needed to ensure the most appropriate care for ALS patients.

The clinical profile of amyotrophic lateral sclerosis patients presenting to the National Hospital of Sri Lanka

OBJECTIVES Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease. Published data on ALS in Sri Lanka is scarce. The aim of this study was to describe the clinical profile of ALS patients presenting to the National Hospital of Sri Lanka(NHSL). METHODS A descriptive cross-sectional study was carried out in 33 consecutive patients with “clinically definite ALS” according to revised El-Escorial and Awaji criteria, who presented from May 2018-May 2020 to two neurology units at NHSL. RESULTS The mean age was 59.7 (±12.3) years while 57.6%(n=19) were males. The average duration for diagnosis was 15.48 (±12.4) months. The commonest first symptom was upper limb weakness 39.4%(n=13) followed by lower limb weakness 33.3%(n=11) and bulbar symptoms 27.3%(n=9). According to King’s Staging System, 18.2%(n=6) were in stage 1, 39.4%(n=13) were in stage 2, 36.4%(n=12) were in stage 3 and 3%(n=1) each were in stage 4A and 4B at the time of diagnosis. None had a family history of ALS. Four were treated with Riluzole. Out of all, 45%(n=15), 24.2%(n=8) and 6.1%(n=2) were referred for physiotherapy, occupational therapy and psychiatry assessment respectively. Seventy five percent (18/24) patients with bulbar symptoms, were referred to speech therapists and one had gastrostomy. Advance directives were not discussed with any of the patients. CONCLUSION The ALS patients presenting to the NHSL had a mean age of 59.7yrs with a slight male preponderance. The average duration taken for diagnosis was 15.5months while the commonest first presentation was upper limb weakness. Most present in King’s stage 2 or 3 and only a few were treated with disease modifying agents. Multidisciplinary care provided and management of disease related complications were suboptimal. Discussing about advance directives with ALS patients, is yet not part of Sri Lankan practice. This emphasizes the need to enhance standards of care for ALS patients to improve their quality of life.

Design Innovation Driven by Artificial Intelligence AI Multifunctional Wheelchair Design Based on the Needs of Patients with ALS

With the maturity of artificial intelligence technology, the integration of artificial intelligence and the medical and health fields has become more and more closely. The improvement of technology has played an important role in the development of the medical and health industry and has provided more possibilities for the treatment and rehabilitation of rare diseases. In mechanical design, integrated and innovative design methods can better discover the needs of patients, better reflect the advantages of technology and solve practical problems. Under the guidance of design thinking and inclusive design, this article will study the life needs of patients with amyotrophic lateral sclerosis (ALS) and the nursing needs of nursing staff, and integrate artificial intelligence technology to propose innovative solutions to users Solution-Design an AI multifunctional wheelchair dedicated to ALS patients to provide intelligent methods and paths for the rehabilitation and care of ALS patients.

Multidisciplinary care in Amyotrophic Lateral Sclerosis: a systematic review and meta-analysis.

Multidisciplinary care (MDC) has been the most recommended approach for symptom management in amyotrophic lateral sclerosis (ALS) but there is conflicting evidence about its effectiveness on survival and quality of life (QoL) of ALS patients. We conducted a systematic review to determine the effects of multidisciplinary care compared to general neurological care in survival and quality of life of ALS patients. A comprehensive literature search using Scopus, MEDLINE-PubMed, Cochrane, Web of Science, PEDro, and Science Direct was undertaken. Studies related to multidisciplinary care or general neurological care in ALS patients that assessed survival and quality of life and were published in the period up to and including January 2020 were included. A total of 1192 studies were initially identified, but only 6 were included. All studies that investigated survival showed and advantage of MDC over NC, and this benefit was even greater for bulbar onset patients. A meta-analysis was performed and showed a mean difference of 141.67 (CI 95%, 61.48 to 221.86), indicating that patients who received MDC had longer survival than those who underwent NC (p = 0.0005). Concerning QoL, only one study found better mental health scores related to QoL for patients under MDC. Multidisciplinary care is more effective than general neurology care at improving survival of patients with ALS, but only improves mental health outcomes related to quality of life of these patients.

Amyotrophic lateral sclerosis care in Saudi Arabia: A survey of providers' perceptions.

ObjectiveProvision of care for patients with amyotrophic lateral sclerosis (ALS) is complex and requires the contribution of multiple healthcare professionals. Several international ALS care measures were developed to ensure optimal care for ALS patients. We looked at the rate of inconsistency in providing standard ALS care measures in Saudi Arabia (SA).MethodsA 5‐point response survey was distributed to practicing neurologists in SA. They were asked to grade their perceived consistency of accessibility for 19 items of ALS care measures at their center. The list of ALS care measures items was derived from international ALS guidelines.ResultsThe response rate from neurologists was 47.3% (62/131), and the responses of 39 neurologists who follow ALS cases were included. Most of the selected ALS care measure items, 63.1% (12/19), were perceived by 50% or more of the ALS care providers to be not consistently accessible to their patients. The perception of ALS care providers of the inconsistent accessibility for ALS patients to ALS care measures was high for communication devices (92.3%), supportive equipment such as motorized wheelchairs (76.9%), end‐of‐life discussion (74.4%), and respiratory monitoring (66.7%).ConclusionOur data show that ALS patients in SA do not have consistent access to the recommended ALS care measures.

Respiratory Assessment of ALS Patients: A Nationwide Survey of Current Dutch Practice.

Non-invasive ventilation (NIV) is an established treatment for respiratory failure in patients with amyotrophic lateral sclerosis (ALS). Several studies have shown room for improvement with regard to respiratory care for ALS patients, including latency of referral. These studies focused on the time period starting at the moment of referral to a home ventilation service (HVS) onwards. In the current study we performed a nationwide survey to gain insight in the trajectory before referral. We questioned the assessment of respiratory impairment by ALS physicians/care teams, including criteria for referral to an HVS. We requested 40 ALS care teams in the Netherlands to fill in an online questionnaire on respiratory management in ALS patients. Thirty-two ALS care teams (80%) responded. Forced vital capacity was the most frequently used test at each outpatient visit (72%) and often served as a criterion (78%) for referral to an HVS. Other respiratory function measurements that were performed less often included peak cough flow (50%), maximum inspiratory/expiratory pressure (31% /28%) and sniff nasal inspiratory pressure (13%). Morning headache was the most frequently questioned complaint (94%), followed by daytime sleepiness (91%). Dyspnoea and orthopnoea were reported by 38% and 59% as important complaints. Out of all patients under the care of the ALS care teams, the mean estimated proportion of patients that was referred to an HVS was 69% (range 20-100%). When physicians refrained from referral, the most often cited reasons were patient's decision to withhold NIV (94%) and cognitive impairment (50%). Sixteen percent of the respondents stated bulbar impairment as a reason to refrain from referral. Despite findings in previous studies on the superiority of SNIP and PCF as compared to FVC, our study shows that a majority of ALS care teams still prefers to use FVC for the assessment of respiratory dysfunction and for the timing of referral to an HVS. Another finding is that bulbar impairment is not an obstacle for referral for NIV.

Respiratory Assessment of ALS Patients: A Nationwide Survey of Current Dutch Practice.

Non-invasive ventilation (NIV) is an established treatment for respiratory failure in patients with amyotrophic lateral sclerosis (ALS). Several studies have shown room for improvement with regard to respiratory care for ALS patients, including latency of referral. These studies focused on the time period starting at the moment of referral to a home ventilation service (HVS) onwards. In the current study we performed a nationwide survey to gain insight in the trajectory before referral. We questioned the assessment of respiratory impairment by ALS physicians/care teams, including criteria for referral to an HVS. We requested 40 ALS care teams in the Netherlands to fill in an online questionnaire on respiratory management in ALS patients. Thirty-two ALS care teams (80%) responded. Forced vital capacity was the most frequently used test at each outpatient visit (72%) and often served as a criterion (78%) for referral to an HVS. Other respiratory function measurements that were performed less often included peak cough flow (50%), maximum inspiratory/expiratory pressure (31% /28%) and sniff nasal inspiratory pressure (13%). Morning headache was the most frequently questioned complaint (94%), followed by daytime sleepiness (91%). Dyspnoea and orthopnoea were reported by 38% and 59% as important complaints. Out of all patients under the care of the ALS care teams, the mean estimated proportion of patients that was referred to an HVS was 69% (range 20-100%). When physicians refrained from referral, the most often cited reasons were patient's decision to withhold NIV (94%) and cognitive impairment (50%). Sixteen percent of the respondents stated bulbar impairment as a reason to refrain from referral. Despite findings in previous studies on the superiority of SNIP and PCF as compared to FVC, our study shows that a majority of ALS care teams still prefers to use FVC for the assessment of respiratory dysfunction and for the timing of referral to an HVS. Another finding is that bulbar impairment is not an obstacle for referral for NIV.

Multidisciplinary amyotrophic lateral sclerosis telemedicine care: The store and forward method.

Amyotrophic lateral sclerosis (ALS) patients benefit from multidisciplinary care in an ALS clinic. We studied whether multidisciplinary care of ALS patients using the store and forward method of telemedicine was feasible and acceptable to patients and providers. ALS patients seen in the University of Florida (UF) Jacksonville ALS clinic were eligible for our study. A trained telemedicine nurse performed and recorded a multidisciplinary assessment of the patient in their home. Clinic team members reviewed the assessments and provided recommendations, and the clinic director discussed the plan with the patient via videoconference. Patient and provider satisfaction was evaluated using surveys. Eighteen patients completed a total of 27 telemedicine visits. Patient satisfaction was excellent and provider satisfaction was very good. The store and forward method of telemedicine is an acceptable alternative to live telemedicine for the multidisciplinary care of ALS patients. This method of care may improve access to multidisciplinary care for this patient population. Muscle Nerve 59:34-39, 2019.

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS): a Russian version.

To translate into Russian and adapt the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) for patients with amyotrophic lateral sclerosis (ALS). Medical care for ALS patients requires participation of a multidisciplinary team. More than one third of patients with ALS suffer from cognitive and behavioral disturbances, and it influences decisions of the team, patients and family. The scale for measuring of these disturbances should be rapid and understandable for evaluation and take into account the specificity of cognitive and behavioral impairments in ALS. ECAS met these criteria and allows screening examination of patients with ALS at different stages of movement impairment.

The evaluation of bulbar dysfunction in amyotrophic lateral sclerosis: survey of clinical practice patterns in the United States

Objective: Speech and swallowing impairments are highly prevalent in individuals with amyotrophic lateral sclerosis (ALS) and contribute to reduced quality of life, malnutrition, aspiration, pneumonia and death. Established practice parameters for bulbar dysfunction in ALS do not currently exist. The aim of this study was to identify current practice patterns for the evaluation of speech and swallowing function within participating Northeast ALS clinics in the United States. Methods and results: A 15-item survey was emailed to all registered NEALS centres. Thirty-eight sites completed the survey. The majority (92%) offered Speech-Language Pathology, augmentative and alternative communication (71%), and dietician (92%) health care services. The ALS Functional Rating Scale-Revised and body weight represented the only parameters routinely collected in greater then 90% of responding sites. Referral for modified barium swallow study was routinely utilised in only 27% of sites and the use of percutaneous gastrostomy tubes in ALS patient care was found to vary considerably. Conclusions: This survey reveals significant variability and inconsistency in the management of bulbar dysfunction in ALS across NEALS sites. We conclude that a great need exists for the development of bulbar practice guidelines in ALS clinical care to accurately detect and monitor bulbar dysfunction.

Respite Care Services for Patients With Amyotrophic Lateral Sclerosis and Their Families From the Perspective of Home Care Nurses

The objective of this study was to use the perspective of home care nurses to elucidate the reactions of in-home patients with amyotrophic lateral sclerosis (ALS) and their family members regarding use of respite care, and involvement of home care nurses with patients and family before and after respite care. Six home care nurses participated in semistructured interviews. Data of seven ALS patients regarding respite care use were collected. The findings showed that ALS patients had both positive and negative reactions to using respite care. The family members provided highly patient-centered care on a daily basis and they wanted the care staff to provide the same quality of care in the respite care setting. The home care nurses provided various services relevant to respite care for ALS patients and their family members.

Health Care Management in ALS Patients

An optimized management of amyotrophic lateral sclerosis (ALS) is an added value of a follow-up by an interdisciplinary team. The aim of this study was to identify which professionals are involved in care of ALS patients at Portugal and their impact on quality of life (QoL). We divided the sample in Group 1 (G1; Southern) and Group 2 (G2; Northern) and used a semi-structured questionnaire, functional status scale (Amyotrophic Lateral Sclerosis Functional Rating Scale–Revised [ALSFRS-R]), and QoL evaluation to perform the study. There were observed differences at admission in ALSFRS-R, higher in G1, presence of specialized professionals higher in G2; non-invasive ventilation equipment had higher use and compliance in G1. Multiple regression analysis shows gender, education level, and technical aids as predictors of QoL. The study showed that, despite regional differences, there were external factors common to both groups that influenced QoL.

The impact of physical impairment on emotional well-being in ALS

There has been evidence that subjective quality of life in patients with amyotrophic lateral sclerosis (ALS) is comparatively good, unrelated to the state of physical functioning, so called ‘disability paradox’. Other studies show weak to moderate correlations between disease severity and emotional well-being. Our aim was to analyse the impact of physical impairment on emotional well-being when assessed disease-specifically and seen through the patient's eyes with additional clinical evaluation. In 121 patients emotional functioning was evaluated by the ALS Assessment Questionnaire (ALSAQ-40). Physical status was assessed by the ALS Functional Rating Scale-Extension (ALSFRS-EX) and Borg dyspnoea scales and by clinical examination (muscle strength and pulmonary function). Multiple regression and correlation analyses were performed. Results showed that physical impairment and progression rate of physical deterioration had a significant impact and explained some variance in emotional well-being (adjusted R2 = 0.22). Pulmonary function and the sense of dyspnoea correlated significantly on a weak to moderate level with emotional well-being. In conclusion, disease-specific patient- reported outcome measurement instruments revealed a moderate but distinct impact of physical impairment on emotional well-being. This study challenges the ‘disability paradox’ and has relevant findings that can support the timely delivery of care for ALS patients.

Anesthetic management of the patient with amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS), with an incidence of 1.5-2.5 for 100 000 per year, is a rare but rapid progression neuromuscular degeneration disorder that poses unique perioperatively challenges to clinical anesthesiologists. The progressive degeneration of motor neurons causes a constellation of symptoms, including muscular weakness, atrophy, fasciculations, spasticity, and hyperreflexia. Therapeutic and experimental treatments, including riluzole, beta lactams, methylcobalamin, dexpramipexole, antiepileptics, antioxidant agents, neutrophin, antiinflammatory agents, and antiapoptosis drugs, are described. Newer therapies, such as neural stem cells and diaphragmatic pacing, are presented. Because of the inherent muscle weakness and associated respiratory insufficiency, certain precautions must be utilized during anesthetic care of ALS patients. In particular, certain neuromuscular agents are contraindicated and anesthetics that leave the body more rapidly present logical and attractive options in this population. A solid understanding of the disease process, therapeutic interventions, and anesthesia considerations are all paramount for the successful management of a patient with ALS in the perioperative setting.

Guest Editorial: Early and innovative symptomatic care to improve quality of life of ALS patients at Cleveland VA ALS Center

INTRODUCTION Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of unknown etiology. However, there is good evidence to validate the theory that military service predisposes the individual to the future development of the disease [1]. As a direct result of these data, in 2008 ALS became a presumptively compensable illness for all veterans with 90 days or more of continuously active service in the military. This decision led to an influx of ALS patients into the Department of Veterans Affairs (VA) healthcare system and the development of a nationwide plan of care [2]. The implementation of this plan at the Cleveland VA Spinal Cord Injury (SCI) Division has been challenging. However, it has offered the opportunity to review current practice parameters that dictate the care of ALS patients in a new context. Using these parameters and considering the strengths and weakness of the VA healthcare system, we have made significant improvements to current practice and applied novel technology to care for ALS patients. Herein we describe these innovations and the foundation we have established to provide high quality ALS care. BACKGROUND ALS is a terminal neurodegenerative disorder. The management of patients with ALS centers almost entirely around symptomatic care. Although many studies have examined the role of particular interventions in prolonging survival after diagnosis or decreasing the rate of decline in physical ability, perhaps the most important studies guiding intervention have investigated quality of life (QoL) over the course of the disease. In 1999, the American Academy of Neurology (AAN) published the first set of formal practice parameters based on a review of the literature [3]. These evidence- based practice parameters were revised in 2009 [4] and included important updates on how care is administered, as well as recommendations regarding the use of the first disease-modifying agent available. The European Federation of Neurological Societies (EFNS) published parallel recommendations in 2005 [5], with revised and updated practice guidelines published in 2012 [6]. A third publication in 2007 also summarized current evidence-based recommendations for care of ALS patients [7]. These three sets of recommendations are nearly identical and in many instances lack precise details in terms of when and how to implement care. This reveals a need for further studies to answer basic questions regarding interventions, such as when a percutaneous endoscopic gastrostomy (PEG) tube should be placed in a patient or how early respiratory support should be provided. This information would help standardize practice parameters and allow for a higher level of symptomatic care in patients with ALS. INNOVATIVE CARE IN VA SYSTEM Early Noninvasive Positive Pressure Ventilation for ALS Patients The majority of patients with ALS will die from respiratory failure [8]. Furthermore, during the course of the disease, respiratory function correlates with QoL and patient survival [9-11]. The survival benefits from noninvasive positive pressure ventilation (NIPPV) supersede those obtained from riluzole or PEG tube insertion [12]. It follows, logically, that aggressive management of respiratory compromise should be part of a comprehensive plan not simply to prolong life (because some patients prefer not to have their life prolonged) but also as a mechanism to improve QoL. The latter goal, which is more consistent with our mission as an ALS center, is supported by several well-designed studies [12-13]. These studies suggest that NIPPV improves QoL by improving a patient's energy, concentration, and sleep quality, decreasing daytime somnolence and physical fatigue. NIPPV also improves lung compliance [14] and decreases the rate of decline in vital capacity [15-16]. Despite this evidence, the ALS Clinical Assessment Research and Education database has documented underutilization of NIPPV by patients with ALS [17]. …

Guest Editorial: Developing a system of care for ALS patients at the Louis Stokes Cleveland VA Medical Center, Spinal Cord Injury Division

INTRODUCTION Amyotrophic lateral sclerosis (ALS), also referred to as Lou Gehrig's disease, is a fatal motor neuron disease characterized by rapidly progressive weakness, dysarthria, dysphagia, and respiratory failure. Fifty percent of patients will die within 3 years of diagnosis and 95 percent within 5 years-- mainly from respiratory failure [1]. The only available treatment, riluzole, has limited efficacy, prolonging survival by 3 months [2-3]. Although familial cases have led to the identification of associated genetic loci, the etiology of sporadic cases is unknown. There is, however, an association between military service and the development of ALS [4]. Hence, in 2008, ALS became a presumptively compensable illness for all Veterans with 90 days or more of continuously active service in the military. This provides the Veteran full access to medical care, medication management, durable medical equipment (DME), and monthly financial compensation. Since this decision in 2008, there has been a growing appreciation for the level of Department of Veterans Affairs (VA) benefits associated with the diagnosis of ALS, and subsequently more Veterans have entered the VA medical system with a diagnosis of ALS, both at the Cleveland VA Medical Center and in Veterans Integrated Service Network (VISN) 10 in Ohio. Between 2005 and 2010, the spinal cord injury (SCI) outpatient clinic treated 19 patients with ALS, compared with 60 patients in years 2011 to 2012 alone. This increased awareness of VA benefits at the outset resulted in Veterans presenting at their first evaluation with advanced stages of ALS, characterized by speech, swallowing, and breathing problems. We found many of the Veterans and their family members psychologically unprepared to cope with the disease progression, often times expressing anger or fear during the initial evaluation. Most patients lacked a clearly defined plan for their future care. The rapid progression of this disease resulted in both the Veteran and the staff being unprepared to cope with the need to make imminent medical decisions regarding percutaneous nutrition, noninvasive versus invasive ventilation, and other complex end-of-life decisions. The processes that were in place to provide care for patients with SCI, although interdisciplinary in nature, lacked the speed and complexity required to care for patients with a rapidly progressive neurodegenerative disorder. The realization that our current paradigm of care needed to be modified to provide excellent medical services to patients with ALS led to the development of a joint workgroup between the SCI and the Neurology Services. The overarching goal of this workgroup was to address care needs and improve processes for Veterans with ALS. This team recognized the complexity of ALS care and looked for solutions to address the unique needs of Veterans with ALS, which included rapid medical management of respiratory issues, nutritional needs, psychological support, education, and providing end-of-life care while expanding the interdisciplinary approach already in place on the SCI service. BACKGROUND ALS and Veterans ALS occurs in all major countries worldwide, with higher rates in the United States and England [5]. Approximately 5,600 new cases are diagnosed in the United States each year. The estimated prevalence in the United States is 5 per 100,000 people [6]. About 5 percent of cases are attributed to the inheritance of genetic defects, many of which have been identified; most of these follow an autosomal dominant inheritance pattern. The remaining 95 percent of cases are considered sporadic (nonfamilial) and of unknown etiology. Interestingly, cohorts have been identified suggesting possible causative relationships between the disease and various factors, including pesticide exposure [7], head trauma [8], strenuous physical activity [9], and heavy metal exposure [10]. …

Current Status of the Diagnosis and Management of Amyotrophic Lateral Sclerosis in Korea: A Multi-Center Cross-Sectional Study

Background and PurposeRecently published, evidence-based guidelines should alter the management of amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). However, the newest recommendations for ALS/MND therapy are not reflected in actual clinical practice. We sought to evaluate the current status of the diagnosis and management of ALS in Korea.MethodsThe Korean ALS/MND research group was organized in 2010, involving more than 50 neurologists from neuromuscular centers in Korea. Participating centers collected data from April to September 2010 on the diagnosis and management of patients with ALS. Data forms from the ALS patient care database, which is a component of the ALS clinical assessment, research, and education program (http://www.outcomes-umassmed.org/ALS/), were modified and used for data collection.ResultsIn total, 373 sporadic ALS cases from 35 centers were enrolled. The demographic features and clinical findings were similar to those in previous reports from other countries. The mean age at onset was 50-60 years, and a slight male predominance was observed. The enrolled patients predominantly showed focal onset of cervical or lumbosacral symptoms. Only about one-half of the indicated patients (31.4%) received a physician's recommendation for a parenteral gastrostomy, and 18.1% underwent the procedure. Noninvasive ventilation was recommended in 23% of patients, but applied in only 9.5% of them. Tracheostomy was performed in 12.7% of patients.ConclusionsThe demographic and clinical features of the diagnosis and management of ALS in Korea are similar to those reported in other countries; however, supportive management, as recommended in evidence-based guidelines, are not yet widely recommended or performed for patients with ALS in Korea.

Causes and places of death of patients with amyotrophic lateral sclerosis in south-west China

Understanding the causes and places of death of amyotrophic lateral sclerosis (ALS) patients leads to the development of better treatment modalities. The present study aimed to identify the causes and places of death of ALS patients in south-west China. A total of 139 ALS patients (89 males and 50 females) were regularly followed up in the Department of Neurology, West China Hospital of Sichuan University from 2004 to 2010 until their deaths. Information on the causes and places of death was provided by family members, caregivers, or family physicians. Overall, 91 patients (65.5%) died of respiratory failure, 36 patients (25.9%) died of nutritional causes due to dysphagia, five patients (3.6%) met sudden deaths, three patients (2.2%) died of heart related causes, and four patients (2.9%) died of unknown reasons. Of the 139 patients, 114 (82%) died at home. Consistent with reports in the literature, respiratory failure is the leading cause of death in ALS patients. However, unlike in other studies, the second leading cause is nutrition related. Most patients die at home. The inconsistencies of these results with foreign studies may be attributable to economic and cultural differences. The findings have significant implications on the treatment modalities and palliative care for ALS patients in China.

Clinical attention and assistance profile of patients with amyotrophic lateral sclerosis

To evaluate the functional status of amyotrophic lateral sclerosis (ALS) patients diagnosed at this institution; to analyze hospital and palliative care; to identify patients' knowledge about home care and supportive resources. Twenty-nine patients were evaluated on the ALSFRS-R scale and two semi-structured questionnaires, at the start of the study and every four months thereafter for 1 year. ALSFRS-R score was 30.1±11.5 initially and 24.4±10.5 at 1 year. There was an increase in use of physiotherapeutic care and adaptive aids. The primary caregivers were spouses (55.2%), parents/children/cousins (20.7%), friends (10.3%) and private nurses (3.5%); 10.3% of patients had no caregivers. Basic ALS patient care was provided by the public health system. ALS patients' multidisciplinary care was provided by UNICAMP hospital and its outpatient clinics and, in some patients, complemented by a private health plan or personal expenditure. Few ALS patients were aware of the possibility of home nursing. It is necessary to implement national and regional public home nursing in addition to multidisciplinary specialized care of ALS patients.

Recent advances in motor neuron disease

Amyotrophic lateral sclerosis (ALS) is the most common and most aggressive form of adult onset motor neuron degeneration. Despite intensive research, riluzole remains the only drug with proven efficacy. The cause and pathogenesis of the motor neuron degeneration in ALS appears to be a complex and multifactorial process. In this study, we review recent advances in our understanding of the molecular basis of sporadic and familial forms of ALS and improvements in the treatment of ALS patients. The discovery of mutations in the DNA/RNA-binding proteins transactive response DNA-binding protein 43 (TDP-43) and fused in sarcoma in familial ALS and the cytosolic sequestration of TDP-43 occurring in sporadic ALS opens new avenues in ALS research and suggests that altered RNA processing may be involved in the disease pathogenesis. The survival of ALS patients continues to increase most likely due to noninvasive respiratory ventilation, early percutaneous endoscopic gastrostomy and multidisciplinary care in ALS clinics. Recent insights in the pathology and genetics of ALS have improved our understanding of motor neuron degeneration and will hopefully lead to novel treatment strategies in ALS. Awaiting these, the beneficial effect of multidisciplinary care of ALS patients on survival and quality of life has become clear.