The benefit of evolving multidisciplinary care in ALS: a diagnostic cohort survival comparison

Martin, Sarah; Trevor-Jones, Emma; Khan, Sabyha; Shaw, Keelan; Marchment, Deepti; Kulka, Anna; Ellis, Catherine; Burman, Rachel; Turner, Martin R; Carroll, Liam; Mursaleen, Leah; Leigh, P. Nigel; Pearce, Neil; Ståhl, Daniel; Al‐Chalabi, Ammar

doi:10.1080/21678421.2017.1349151

Cited by 43 publications

(27 citation statements)

References 19 publications

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“…Motor neuron disease (MND) causes progressive muscle paralysis, severe disability and eventually death within on average 2 to 4 years 1. Attendance at a specialist multidisciplinary clinic (MDC) is associated with improved survival and use of assistive aids, devices and proven therapies 2–7. Patients face many barriers accessing specialist services, meaning many patients fail to gain the full benefit from these services 8.…”

Section: Introductionmentioning

confidence: 99%

Process evaluation and exploration of telehealth in motor neuron disease in a UK specialist centre

et al. 2019

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ObjectivesTo evaluate the processes involved in using a novel digitally enabled healthcare system (telehealth in motor neuron disease (TiM)) in people living with motor neuron disease (MND) and their informal carers. We examined TiM implementation, potential mechanisms of impact and contextual factors that might influence TiM implementation or impact.DesignAn 18-month, single-centre process evaluation within a randomised, pilot and feasibility study.InterventionTiM plus usual care versus usual care alone.SettingA specialist UK MND care centre.Participants40 patients with MND and 37 primary informal carers.Primary and secondary outcome measuresPatient, carer and staff outcomes and experiences using semistructured interviews. Descriptive data on implementation and use of TiM.ResultsThe TiM was acceptable and accessible to patients, carers and staff. Intervention uptake and adherence were good: 14 (70%) patients completed a TiM session at least fortnightly. Barriers to TiM use (such as technology experience and disability) were overcome with well-designed technology and face-to-face training. Reported potential benefits of TiM included improved communication and care coordination, reassurance, identification of complications and the potential for TiM to be an alternative or addition to clinic. Benefits depended on patients’ current level of needs or disability. The main challenges were the large number of alerts that were generated by TiM, how the clinicians responded to these alerts and the mismatch between patient/carer expectations and nurses actions. This could be improved by better communication systems and adjusting the alerts algorithm.ConclusionTiM has the potential to facilitate access to specialist care, but further iterative developments to the intervention and process evaluations of the TiM in different services are required.Trial identifier number ISRCTN26675465.

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Section: Introductionmentioning

confidence: 99%

Process evaluation and exploration of telehealth in motor neuron disease in a UK specialist centre

et al. 2019

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“…We found that those cases reported as definite, probable, and probable (lab supported) on the El Escorial criteria had a slightly higher median survival time compared with those in the possible and not classifiable diagnosis group. Studies have shown that individuals who regularly are seen at an ALS specialty clinic have longer survival (33,34). Although we do not know who regularly attended a specialty clinic, we can hypothesize that those with a less definitive diagnosis are less likely to attend such a clinic which could contribute to their decreased survival time.…”

Section: Discussionmentioning

confidence: 90%

Atlanta metropolitan area amyotrophic lateral sclerosis (ALS) surveillance: incidence and prevalence 2009–2011 and survival characteristics through 2015

Punjani

Wagner

Horton

et al. 2019

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Amyotrophic lateral sclerosis (ALS) is a fatal, rare, and hard to diagnose neurological disease with unknown etiology. Objective: To understand the incidence, prevalence, and survival characteristics of ALS cases in the Atlanta metropolitan area. Methods: Neurologists in Clayton, Cobb, DeKalb, Fulton, and Gwinnett counties provided case reports for ALS patients under their care from 1 January 2009 to 31 December 2011. Incidence and prevalence rates were calculated for 2009, 2010, and 2011 by sex, race, and ethnicity. Using data from the National Death Index, survival time was calculated for age, sex, race, ethnicity, and El Escorial criteria. Results: There were 281 unique ALS cases reported, which is approximately 104% of the expected cases. The majority of the 281 cases were white, non-Hispanic, male, and in the 50-59 age category. The overall average incidence rate for 2009 to 2011 was 1.54 per 100,000 person-years, with higher annual incidence rates for whites, males, and non-Hispanics. The prevalence rates for 2009, 2010, and 2011 were 5.05, 5.44, and 5.56 per 100,000, respectively. Median survival time was highest for the 18-39 age group, Asians, non-Hispanics, and males. Additionally, the log-rank tests for homogeneity across strata indicate a statistical significance between strata for the age category for survival time. Conclusion: The findings for Atlanta are similar to other populationbased studies in the United States. Although the Atlanta metropolitan area was selected to over-represent the minority population, the strongest predictor of survival time was age at diagnosis.

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“…Although there is evidence that PLS has some histopathological features in common with ALS, and similar changes in cortical inhibitory function,17 the very modest survival benefit of riluzole shown in ALS trials is far less certain for PLS. The mainstay of management in PLS is currently symptomatic and optimally delivered through in a multidisciplinary setting, which has been shown to have an independent survival benefit in ALS 42. An annual follow-up is often adequate for those living with PLS, with flexibility to respond to unexpected interim deterioration.…”

Section: Managementmentioning

confidence: 99%

Primary lateral sclerosis: diagnosis and management

Turner

Talbot

2020

Pract Neurol

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Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder at the upper motor neurone extreme of the spectrum of motor neurone disease. The diagnosis is clinical and based on the characteristic features of slowly progressive spasticity beginning in the lower limbs, or more rarely with spastic dysarthria, typically presenting around 50 years of age. The absence of lower motor neurone involvement is considered to be a defining feature, but confident distinction of PLS from upper motor neurone-predominant forms of amyotrophic lateral sclerosis may be difficult in the first few years. Corticobulbar involvement in PLS is frequently accompanied by emotionality. While there may be dysphagia, gastrostomy is rarely required to maintain nutrition. Cognitive dysfunction is recognised, though dementia is rarely a prominent management issue. PLS is not necessarily life shortening. Specialised multidisciplinary care is recommended. Increasing international research cooperation is required if the aspiration of dedicated therapeutic trials for PLS is to be achieved.

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The benefit of evolving multidisciplinary care in ALS: a diagnostic cohort survival comparison

Cited by 43 publications

References 19 publications

Process evaluation and exploration of telehealth in motor neuron disease in a UK specialist centre

Process evaluation and exploration of telehealth in motor neuron disease in a UK specialist centre

Atlanta metropolitan area amyotrophic lateral sclerosis (ALS) surveillance: incidence and prevalence 2009–2011 and survival characteristics through 2015

Primary lateral sclerosis: diagnosis and management

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