Multidisciplinary Amyotrophic Lateral Sclerosis Clinic Research Articles

Advance Care Planning in a Multidisciplinary Amyotrophic Lateral Sclerosis Clinic

Advance Care Planning in a Multidisciplinary Amyotrophic Lateral Sclerosis Clinic

Qualitative Analysis of Initial Palliative Care Consultations in Amyotrophic Lateral Sclerosis

BackgroundPalliative care (PC) benefits patients with amyotrophic lateral sclerosis (ALS), however the needs of patients and caregivers and the optimal timing of PC discussions remains unclear. This study reports the analysis of PC consult notes from a larger feasibility trial. The specific aims of this analysis were to i) identify the PC needs of patients with ALS via qualitative analysis and ii) identify characteristics of patients and caregivers that could predict specific PC needs. MethodsThis study was nested within a nonrandomized, prospective study of patients with ALS (and their caregivers) being treated at a multidisciplinary ALS clinic. Exclusion criteria of the main study were age <18 years, inability to complete questionnaires, and prior receipt of PC. All patients were offered a PC consultation (PCC); those who accepted were included in this nested study. Consultation notes were reviewed and thematic and content analyses were conducted. The occurrence of themes across patient and caregiver contextual variables were examined. ResultsThirty-two PCCs were completed between October 2020 and April 2022. Six major themes were identified: PC roles (with subthemes encompassing the spectrum of specialist PC practice including symptom management and advance care planning), engagement with PC, patients’ concerns for their caregivers, caregiver-specific concerns, finances, and COVID-19. An average of 12 topics were discussed per PCC (range = 3–22). Discussion of advance care planning, care coordination, and symptom management was common, and these topics were not discussed more frequently in PCCs with patients with lower functional status, more bulbar symptoms, or lower quality of life. Time from diagnosis did not impact topics of discussion. Patients reporting more symptoms of depression more frequently required psychological support, particularly regarding loss of independence, employment, and leisure activities. DiscussionPatients with ALS and their caregivers have a wide range of PC needs. These needs vary irrespective of time from diagnosis, functional status, or quality of life, therefore PCC is recommended for all patients with ALS. PCC should be individualized based on patient and caregiver preferences. Trial registration informationThe study was registered with ClinicalTrials.gov (NCT04257760; https://clinicaltrials.gov/ct2/show/NCT04257760) on February 6, 2020. The first enrollment occurred on October 20, 2020.

Survival and multidisciplinary amyotrophic lateral sclerosis clinic care at a United States Veterans Affairs medical center

Introduction/Aims: The purpose of this work was to investigate survival outcomes in patients with amyotrophic lateral sclerosis (ALS) at our Veteran’s Affairs Medical Center multidisciplinary ALS clinic and compare this to relevant data from several European studies.&#x0D; Methods: Our sample consisted of 56 total Veterans (n=56; 54 males, 2 females) that had been seen between June 24, 2013 and February 1, 2021 at our multidisciplinary ALS clinic.&#x0D; Results: The median survival time of our Veterans from symptom onset was 40.96 months (95% CI of 32.17, 76.07), and the median survival time from diagnosis was 23.77 months (95% CI of 18.64, 38.58). This was consistent with the literature. Further consistent with the literature is that multidisciplinary clinics, including ours, have survival advantage over general neurology clinics. Analyzing factors that contributed to this survival, we found significant protective effect on survival from Edaravone use (HR = 0.32, p = 0.036). Otherwise, there was no significant effect on survival noted from use of percutaneous endoscopic gastrostomy (PEG), non-invasive ventilation (NIV), or Riluzole.&#x0D; Conclusion: We found no significant difference in survival rates between our U.S. Veterans in our multidisciplinary ALS clinic and European multidisciplinary ALS clinics, and both are better than general neurology clinics. We also found that Edaravone use may provide some benefit to survival in this patient population.

Racial disparities in ALS diagnostic delay: a single center's experience and review of potential contributing factors.

Outcomes for amyotrophic lateral sclerosis (ALS) patients are improved with prompt diagnosis, earlier initiation of disease-modifying treatments, and participation in a multidisciplinary clinic. We studied diagnostic delay and disease severity at time of clinic presentation between Black and non-Hispanic Caucasian ALS patients. We performed a retrospective analysis of non-Hispanic Caucasian and Black ALS patients seen in the Virginia Commonwealth University Health System multidisciplinary ALS clinic between 2017 and 2023. Diagnostic delay, ALS Functional Rating Scale-Revised (ALSFRS-R) and upright forced vital capacity (FVC) scores at baseline appointment were collected. Patient's distance from clinic and affluency of residential neighborhood were evaluated. We analyzed 172 non-Hispanic Caucasian and 33 Black ALS patients. Black patients had a 64% increase in diagnostic delay compared to non-Hispanic Caucasian patients. Black patients had a lower performance on ALSFRS-R (5.3 points, p < 0.001) and FVC (17.9 percentage points p < 0.001) at time of first clinic visit. Black patients lived closer to clinic, with higher proportion living in the city of Richmond, but in less affluent areas with lower median house income ($55,300 ± 22,600 vs $69,900 ± 23,700). Our findings demonstrate a large racial difference in ALS diagnostic delay, and greater disease severity and lower respiratory function at time of diagnosis for Black ALS patients. Delay in diagnosis prolongs access to disease-modifying therapies, multidisciplinary care, durable medical equipment, and respiratory and nutritional support. Potential sources of these racial disparities include providers' implicit bias and structural racism.

Accessing and Receiving Speech-Language Pathology Services at the Multidisciplinary Amyotrophic Lateral Sclerosis Clinic: An Exploratory Qualitative Study of Patient Experiences and Needs.

This study sought to explore how patients with amyotrophic lateral sclerosis (ALS) presenting with coexisting bulbar and cognitive impairments and their caregivers experienced the speech-language pathologist (SLP) services provided in multidisciplinary ALS clinics in Canada and identified their perceived needs for bulbar symptom management. This qualitative study was informed by interpretive description. Seven interviews were conducted with patients with severe bulbar dysfunction or severe bulbar and cognitive dysfunction due to ALS or ALS-frontotemporal dementia, respectively, and/or their caregivers. Purposive sampling was used to recruit individuals with severe bulbar or bulbar and cognitive disease. Thematic analysis was used to analyze interview data. Patients and caregivers reported difficulties with accessing and receiving SLP services at the multidisciplinary ALS clinic. These difficulties were further exacerbated in those with severe cognitive disease. Participants expressed a need for more specific (i.e., disease and service-related) information and personalized care to address their changing needs and preferences. Engaging caregivers earlier in SLP appointments was perceived as vital to support care planning and provide in-time caregiver education. This study highlighted the challenges experienced by patients and caregivers in accessing and receiving SLP services. There is a pressing need for a more person-centered approach to ALS care and a continuing need for education of SLPs on care provision in cases of complex multisymptom diseases within a multidisciplinary ALS clinic. https://doi.org/10.23641/asha.24069222.

Customized 3D-Printed Assistive Technology in a Multidisciplinary Amyotrophic Lateral Sclerosis Clinic

Abstract Date Presented 04/22/2023 Providing customized, 3D-printed assistive technology (AT) in an amyotrophic lateral sclerosis (ALS) clinic proved feasible and satisfied clients’ needs. This session will explore clients’ feedback and propose next steps for establishing efficacy of this innovative approach to AT. Primary Author and Speaker: Kial-Ann M. Rasmussen Additional Authors and Speakers: Breanna C. Stewart Contributing Authors: William Janes

Rapid Conversion to a Completely Virtual Multidisciplinary ALS Clinic in Response to the COVID-19 Pandemic: Implications for Future Care Delivery.

The goals of this study were to assess the feasibility of maintaining multidisciplinary remote care, patient preferences, and outcomes of this transition because of COVID-19. From March 18, 2020 to June 3, 2020, 127 patients with amyotrophic lateral sclerosis (ALS) who were scheduled to be seen in our ALS clinic were contacted and scheduled according their preference for a telemedicine visit, telephone visit, or postponement until the next available in-person visit. Age, time from disease onset, ALS Functional Rating Scale-Revised, patient choices, and outcomes were recorded. Patient visit preferences were 69% telemedicine, 21% telephone, and 10% postpone for a later in-clinic visit. Patients with higher ALS Functional Rating Scale-Revised were more likely to choose the next in-person opening (P = 0.04). Age and time from disease onset were not related to visit type preference. There were 118 virtual encounters, of which 91 (77%) began as telemedicine and 27 (23%) as telephone visits. Most telemedicine visits were conducted successfully, but 10 were converted to a telephone visit. The clinic maintained 88.6% of patient volume compared with the prior year, during which most visits were in-person. Telemedicine care using synchronous videoconferencing is preferable and feasible for most patients on short notice, with telephone as back-up. Clinic volumes can be maintained. These findings support the conversion of a multidisciplinary ALS clinic to 1 with exclusively virtual visits when future events again disrupt in-person care.

D.1 Feasibility and impact of palliative care at any stage of amyotrophic lateral sclerosis

Background: Although palliative care (PC) is recommended for patients with amyotrophic lateral sclerosis (ALS), many patients receive PC very late or not at all. Our study goals included 1) determing the feasibility of early PC 2) describing patient/caregiver satisfaction with early PC and 3) measuring the impact of early PC on quality of life (QOL) and mood. Methods: Patients followed at the multidisciplinary ALS clinic in Ottawa, Canada and their caregivers were eligible for the study irrespective of duration or severity of disease. All participants completed questionnaires tracking QOL and mood and all were offered a palliative care consultation. Participants completed a satisfaction survey post-PC consultation. Results: 32 patients and 20 caregivers received a PC consultation, conducted virtually. All of them found the consult beneficial and none of the patients reported preferring the consultation later in their illness. The PC consultations were most highly rated by patients with high levels of anxiety and worse bulbar function, and by caregivers of patients with low function. There was no statistically significant change in mood or QOL compared to the 7 participants who declined PC consultation. Conclusions: PC consultations are feasible and beneficial at all stages of illness. Patients with anxiety and bulbar dysfunction may benefit most.

Pulmonary care for ALS: Progress, gaps, and paths forward.

Adults with amyotrophic lateral sclerosis (ALS) have been using home mechanical ventilation for over 50 years. More recently, home respiratory care has evolved to include portable home ventilators, airway clearance devices, and physiological assessments with telemonitoring capability. National organizations currently offer incentives for providing a pulmonary care specialist within a multidisciplinary ALS clinic; however, several critical gaps exist between the available technology and employing a clinician with the necessary expertise. Lack of formal training and poor financial incentives have led to a paucity of both clinicians and active clinical research engaging in the home respiratory care of ALS. Criteria for noninvasive ventilation (NIV) initiation are controversial, and few guidelines exist on the ideal subsequent adjustments of NIV with evolving disease. Consequently, many patients with ALS tolerate NIV poorly and must face the harrowing decision of hospice vs tracheostomy. Advancement of respiratory care in ALS has been hindered by critical gaps in pulmonologist availability, training in chronic respiratory failure, financial support, clinical research, and clarity on ventilation management beyond initiation. Only a multifaceted approach will suffice for addressing the voids in ALS respiratory care, including various education initiatives, financial incentives, clinical research programs, and elevating the standard of respiratory care.

Comparative Performance of Different Respiratory Test Parameters for Detection of Early Respiratory Insufficiency in Patients With ALS.

To compare the performance of different respiratory function testing parameters in a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic. Demographics, clinical data, and respiratory testing parameters were abstracted from the medical records of patients who attended a multidisciplinary ALS clinic from 2008 to 2016. We compared the performance of the 3 primary respiratory test parameters used by Medicare for the initiation of noninvasive ventilation (NIV) (forced vital capacity [FVC] < 50% predicted, maximum inspiratory pressure [MIP] < 60 cm H2O, and abnormal overnight pulse oximetry [OvOx]) on how they related to several clinically relevant attributes. Four hundred seventy-six patients were identified who underwent at least 1 respiratory test. Abnormalities of OvOx, MIP, and FVC occurred at a median of 1.6, 1.5, and 3.8 years from disease onset, respectively (p < 0.00001). Patients with bulbar-onset ALS exhibited earlier abnormalities in MIP and FVC than in spinal-onset ALS (p < 0.005). The median survival after an abnormal OvOx, MIP, or FVC test was 1.4, 1.4, and 0.9 years, respectively (p < 0.0001). Using the ALS Functional Rating Score respiratory subscales, at the time of reported respiratory symptoms there were abnormalities in OvOx (60%), MIP (69%), and FVC (19%). Conversely, when respiratory parameter abnormalities preceded reported respiratory symptoms, this occurred with frequencies in OvOx (79%), MIP (42%), or FVC (24%). Four hundred forty-three patients (93.1%) developed at least 1 abnormal respiratory measure meeting Medicare criteria for NIV consideration, but fewer than 50% in our cohort demonstrated NIV use. Improved survival in subjects using NIV was statistically significant in patients with bulbar-onset ALS. Abnormalities in OvOx and MIP perform better than FVC at early detection of neuromuscular respiratory weakness in ALS. Initiation of NIV in patients with respiratory insufficiency may improve the overall survival in ALS. In the setting of the COVID-19 pandemic, FVC and MIP have not been routinely performed because of infectious aerosol generation. OvOx, which we now routinely mail to patients' homes, has been used exclusively during the COVID-19 pandemic and allows for continued remote monitoring of the respiratory status of patients with ALS. This cohort study provides Class III evidence that in people with ALS, OvOx and MIP are valuable respiratory parameters for the detection of early respiratory insufficiency.

Protocol for psychometric evaluation of the Amyotrophic Lateral Sclerosis - Bulbar Dysfunction Index (ALS-BDI): a prospective longitudinal study

IntroductionEarly detection and tracking of bulbar dysfunction in amyotrophic lateral sclerosis (ALS) are critical for directing management of the disease. Current clinical bulbar assessment tools are lacking, while existing physiological...

Feasibility of customized 3D-printed assistive technology within an existing multidisciplinary amyotrophic lateral sclerosis clinic

Purpose Three-dimensional (3 D) printing of assistive technology (AT) is an emerging intervention in rehabilitation sciences. Existing research primarily considers narrow applications of this technology with most studies focussing on a single type of assistive device. Individuals with amyotrophic lateral scleroses (ALS) have high abandonment rates of prefabricated AT. Therefore, the goal of this project was to determine the implementation and acceptability feasibility of meeting AT needs through the creation of customized, 3D-printed devices within an existing multidisciplinary ALS clinic. Methods Implementation feasibility was measured using descriptive statistics about the research team’s ability to fulfil device requests. Acceptability was evaluated using semi-structured patient satisfaction interviews after AT device provision. The study utilized thematic analysis to identify patterns in the qualitative data collected from participant interviews. Results Nine participants identified at least one need for 3D-printed assistive devices customized to their unique performance abilities and needs. Seven participants received 3D-printed devices, four of whom completed the follow-up interview. The research team was able to fulfil 20 of 34 device requests, supporting implementation feasibility. Thematic analysis revealed three themes from the interviews: Satisfaction with Devices and Services, Value of Training as a Service, and Increased Participation and Choice. Conclusions These findings demonstrate implementation feasibility and participant satisfaction with the process of receiving devices, supporting acceptability feasibility. Future research into the efficacy of customized 3D-printed AT is recommended. Implications for rehabilitation Individuals with ALS have unmet needs in assistive technology. Custom AT development using 3D printing can be implemented within an existing, multidisciplinary ALS clinic. Future 3D printing research should rigorously investigate methods to meet the unique needs and challenges of individuals in this setting.

Exploring the association between outcome measures to guide clinical management in patients with amyotrophic lateral sclerosis.

[Purpose] The usefulness and limitations of outcome measures changes throughout functional decline of patients with Amyotrophic Lateral Sclerosis (ALS). This study aims to describe associations between outcome measures in patients with ALS over time. [Participants and Methods] Participant data was collected at a multidisciplinary ALS clinic during regular clinic visits, including gait velocity, Timed Up and Go, the ALS Functional Rating Scale-Revised, and it’s Gross Motor Subscale. [Results] All gait velocity measures were <1.2 m/sec; average Timed Up and Go was >13.5 sec. There was strong internal consistency between ALS Functional Rating Scale-Revised and its functional mobility components and a strong, significant correlation between the Timed Up and Go and the Gross Motor Subscale. [Conclusion] Patients with ALS are not community ambulators and demonstrate risk for falls. We found concurrent validity between objective and self-reported measures. The strong association between the Gross Motor Subscale and the Timed Up and Go may allow PTs to utilize the self-reported Gross Motor Subscale to predict fall risk. Clinically, when the Timed Up and Go and gait velocity are no longer appropriate due to disease progression, the Gross Motor Subscale can provide insight into functional decline.

Embedded Psychiatric Services in a Multidisciplinary Amyotrophic Lateral Sclerosis Clinic: An Assessment of Patient Needs and Perceptions.

Amyotrophic lateral sclerosis (ALS) is often associated with a range of difficult neuropsychiatric symptoms and conditions, including depression, apathy, pseudobulbar affect, and frontotemporal dementia (FTD). Despite the potential role for psychiatrists in the treatment of ALS, they are not typically involved in the ALS clinical team. The investigators describe a quality improvement intervention providing embedded psychiatric services within a multidisciplinary clinic (MDC). A psychiatrist working within an ALS MDC evaluated patients (N=116) over a 1-year period. The clinic assessed the prevalence of neuropsychiatric symptoms and conditions in patients with ALS (depression, anxiety, pseudobulbar affect, and cognitive impairment, including FTD) using standardized screening methods. Fifty-five patients and 47 family members completed surveys about perceptions of their need for psychiatric care, their experience of meeting with a psychiatrist, and their desire for future access to psychiatric care. Prevalence rates for neuropsychiatric symptoms were 14.9% for depression, 11.3% for anxiety, 19% for cognitive impairment (including FTD, 8.6%), and 36.2% for pseudobulbar affect; 62.0% of patients were being prescribed at least one psychotropic medication. Both patients and family members reported that meeting with a psychiatrist was helpful, that the treatment provided was helpful, and that they would prefer continued availability of psychiatric services in the future. The presence of cognitive impairment and use of antidepressants increased the likelihood of patients reporting a benefit from psychiatric care. Patients with ALS report a benefit from increased access to psychiatric services. The inclusion of a psychiatrist within the ALS MDC model should be considered to improve quality of care for this patient population.

Bowel, bladder, and sudomotor symptoms in ALS patients

ObjectivesTo describe prevalence rates of bowel, bladder, and sudomotor symptoms in patients with amyotrophic lateral sclerosis (ALS) in relation to disease onset and progression. Treatment strategies and efficacies were also assessed. MethodsA pilot patient cohort revealed increased incidences of bowel/bladder and sudomotor symptoms. Questionnaires derived from formal bowel and bladder survey instruments were administered to a second cohort of patients during multidisciplinary ALS clinic visits. ResultsThe pilot cohort of 30 patients reported an increase in bowel symptoms from 17% prior to 70% after the diagnosis of ALS, and an increase in urinary symptoms from 24% to 76%. In the second cohort of 66 patients an increase in constipation from 33% prior to 64.7% after the diagnosis of ALS was reported. 25.4% of patients reported bowel urgency initially, which increased to 33.3% over time. Constipation was most commonly treated with docusate, dietary fiber supplementation, fluid/exercise, and polyethylene glycol. In the second cohort the prevalence of overactive bladder symptoms increased from 3.1% prior to 25.0% after the diagnosis of ALS. Urinary symptoms are most commonly treated with catheters and oxybutynin. A sudomotor survey found stinging eyes in 17.2% of patients, oily/greasy skin in 14.1% of patients, and flaking of the skin in 29.7% of patients. ConclusionsBowel and bladder symptoms are common in the ALS population and respond to treatment. Sudomotor symptoms are also common. Inquiring about these symptoms at clinic visits and initiating treatment can significantly improve the patients' quality of life.

Behavioural changes predict poorer survival in amyotrophic lateral sclerosis

ObjectiveThe Motor Neuron Disease Behavioural Scale (MiND-B) is a clinically validated tool that was developed to detect behavioural dysfunction in patients with amyotrophic lateral sclerosis (ALS). The current study aimed to evaluate behavioural impairment using MiND-B, as well as cognitive dysfunction in ALS patients, and to determine their prognostic implications. MethodPatients with a clinical diagnosis of ALS were prospectively recruited from a specialised multidisciplinary ALS clinic. Patients underwent behavioural assessment with the Motor Neuron Disease Behavioural Scale (MiND-B) and cognitive evaluation using the Addenbrooke’s Cognitive Examination (ACE). Primary outcome measure was selected as survival time, defined by time from assessment to time of death or censor date. Univariate assessment of survival effect was carried out using Kaplan-Meier survival analysis followed by cox regression analysis to assess the effect of MiND-B and ACE scores on survival time. ResultsA total of 134 patients were included in the study. MiND-B testing determined that 59% were classified as having behavioural dysfunction, with deficits associated with a significantly shorter survival time (HR 2.53, p = 0.003, 95% CI 1.3–4.6). Furthermore, regression analysis demonstrated that for every 1-point reduction in the MiND-B score, risk of death increased by 3%. ACE testing established that 33% of the cohort had evidence of cognitive dysfunction. Patients with cognitive dysfunction on ACE testing had a significantly shorter survival time than patients without cognitive impairment (HR 2.0, p = 0.042, 95% CI 1.04–3.3). ConclusionThe presence of behavioural and cognitive impairments in ALS patients was associated with poor survival. The MiND-B and ACE inventories are simple and efficient clinical tools that can be administered in the multidisciplinary ALS clinic, that aid in the prognostication of this patient population.

Assessment of feasibility and utility of universal referral to specialty palliative care in a multidisciplinary amyotrophic lateral sclerosis clinic: A cohort study.

Although the value of palliative care integration in multidisciplinary amyotrophic lateral sclerosis (ALS) clinics has been increasingly recognized, limited data are available demonstrating the feasibility and utility of a palliative care specialist embedded in an ALS clinic. The purpose of this study is to describe the feasibility and utility of a universal outpatient referral model for specialty palliative care for patients with amyotrophic lateral sclerosis. This is a retrospective cohort study of initial patient visits with a palliative care physician in a multidisciplinary ALS clinic at an academic institution. All patients were referred to an embedded palliative care physician from January to December 2019. Within the study period, 52 of 69 (75%) patients with a confirmed diagnosis of ALS were seen by the palliative care physician. The most common reason patients were not seen by palliative care was a lack of insurance authorization (n=5). At the visit, 94% of patients discussed at least one advance care planning or goals of care topic. The most common advance care planning topic discussed was code status (40%). The most common goals of care topic discussed was meaning and values (57%). Symptom management was discussed with 40 patients (77%). The most common symptom addressed was pain and/or muscle spasms (33%). These data support the feasibility and utility of universal, outpatient palliative care referral for patients with ALS. Further research is necessary to determine the long-term effect on outcomes for this population.

Transitions in Amyotrophic Lateral Sclerosis: Patient and Caregiver Experiences.

Amyotrophic lateral sclerosis (ALS) presents many transitions for persons/people with ALS (PwALS) and their caregivers. Transitions are passages from one life phase, condition, or status to another. We used qualitative methods to understand how PwALS and caregivers experience transitions throughout their ALS journey. PwALS and their caregivers were recruited from a multidisciplinary ALS clinic in Edmonton, Canada. We recruited patients at the stage of ALS that home mechanical ventilation, a feeding tube, and/or assistive communication technology had been offered. Semi-structured interviews were audio-recorded, transcribed, and analyzed using qualitative thematic analysis. We interviewed 14 PwALS and 15 caregivers and identified 5 recurring themes. The importance of community was identified by many PwALS and caregivers who expressed feelings of loneliness and isolation. Most caregivers were spouses and couples navigated a change in their relationship roles as one spouse transitioned to becoming a caregiver while the other transitioned to dependency. The caregiver spouses reported a sense of "total responsibility" that encompassed continual vigilance for the PwALS's well-being, managing their household and finances. PwALS and caregivers reported transitioning to reliance on life-sustaining medical devices; early adoption and information on these devices increased their quality of life. Participants also wanted more and earlier information on advanced care planning. PwALS and caregivers identified adapting to new forms of communication as a necessity. ALS presents many transitions for PwALS and caregivers. Understanding these transitions is important for ALS healthcare professionals who seek to implement best care practices.

Natural history and clinical features of ALS in Malaysia

Objective Studies from multiethnic populations are rarely reported but do indicate differences in phenotypic presentation and survival in amyotrophic lateral sclerosis (ALS). In this study, we aimed to investigate the natural history of a cohort of ALS patients from a multiethnic population. Methods: Data from ALS patients presenting to our multidisciplinary ALS clinic were prospectively collected from January 2015 to June 2020 as part of an ongoing hospital-based patient registry. Kaplan–Meier and Cox regression model were performed to identify potential prognostic factors. Results: A total of 144 ALS patients were recruited. We estimated the crude ALS incidence as 0.53 per 100,000 for 2019 but rises to 2 per 100,000 in patients aged 60–74 years. The majority of patients were of Chinese ethnicity (59.7%), followed by Malay (24.3%), Indian (11.1%), and others (4.9%). Malaysian Indians had a significantly steeper ALSFRS-R slope at diagnosis (p = 0.040). We found a worse prognosis in patients with bulbar-onset (HR = 1.915, p = 0.019), older age (HR = 1.052, p = 0.000), and who were fast-progressors (HR = 1.274, p = 0.000). In contrast, a higher body mass index (HR = 0.921, p = 0.007) and a longer time to diagnosis (HR = 0.967, p = 0.006), noninvasive ventilation (HR = 0.820, p = 0.000) and percutaneous endoscopic gastrostomy insertion (HR = 0.823, p = 0.000) were associated with better survival. On multivariate analysis, diagnostic delay and slow disease progression were associated with better survival. Conclusions: In our cohort, diagnostic delay and a slow disease progression were significantly associated with better survival in ALS. We also found ethnic variation with Chinese preponderance and more rapid disease progression in patients of Indian descent.

Respiratory function and cognitive profile in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is increasingly recognized as a multisystem disorder with 30%-50% of patients exhibiting cognitive impairment. The pathophysiological mechanisms of cognitive dysfunction are probably multifactorial although hypoventilation secondary to respiratory dysfunction may contribute to cognitive decline. The current study aimed to identify the relationship between respiratory function in ALS patients and the presence and degree of cognitive impairment. Amyotrophic lateral sclerosis patients were prospectively recruited from a multidisciplinary ALS clinic. Baseline clinical assessments including respiratory function as assessed by spirometry were recorded with forced vital capacity (FVC) ≤ 75% considered to be reduced respiratory function. Cognitive testing was performed utilizing the Addenbrooke's Cognitive Examination (ACE) and the Mini-Mental State Examination (MMSE). From a cohort of 100 ALS patients, 48% were categorized as having impaired respiratory function (FVC=58.24%±2.15%) whilst 52% had normal function (88.65%±1.27%). Compared to the group with normal respiratory function (ACE 90.68 ± 0.89, MMSE 28.22 ± 0.21), patients with respiratory dysfunction had significantly reduced cognitive function (ACE 86.83± 1.5, P=0.025; MMSE 26.29± 0.45, P=0.029). Furthermore, subscores demonstrated significant differences between the groups with respect to domains in memory (P=0.003) and attention (P=0.05) with a trend observed in fluency (P=0.082). There was a significant correlation between patient baseline FVC and ACE scores as well as between FVC and memory and fluency subscores (P<0.01). Amyotrophic lateral sclerosis patients with respiratory compromise were more likely to develop reduced cognitive function. In addition to improving physical function, it remains plausible that non-invasive ventilation may alter the progression of cognitive impairment in ALS patients and potentially improve overall quality of life and carer burden.