scholarly journals The Prevalence and Management of Saliva Problems in Motor Neuron Disease: A 4-Year Analysis of the Scottish Motor Neuron Disease Register

2021 ◽  
pp. 1-6
Author(s):  
Iona Pearson ◽  
Stella A. Glasmacher ◽  
Judith Newton ◽  
Emily Beswick ◽  
Arpan R. Mehta ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Health Care Professionals ◽  
Management Practice ◽  
Treatment Decision ◽  
Future Research ◽  
Clinical Factors ◽  
Pharmacological Management ◽  
Research Attention ◽  
Bulbar Onset

<b><i>Introduction:</i></b> Saliva problems are common and distressing for people with motor neuron disease (pwMND). Despite clinical guidelines for assessment and treatment, management of saliva problems has received little research attention. <b><i>Objective:</i></b> We aimed to investigate the prevalence of saliva problems in pwMND, their association with clinical factors, and their management practice using a highly curated population-based register for motor neuron disease (MND) with 99% case ascertainment. <b><i>Methods:</i></b> We conducted an analysis of pwMND diagnosed between January 2015 and October 2019 using the Scottish MND Register (CARE-MND [Clinical, Audit, Research, and Evaluation of MND]). The association between clinical factors and saliva problems was investigated using univariate and multivariable logistic regression; results are reported as odds ratio (OR) and 95% confidence intervals. A survey of health-care professionals involved in the care of pwMND was performed to contextualize the findings. <b><i>Results:</i></b> 939 pwMND were included. Prevalence of saliva problems was 31.3% (294). Bulbar onset (OR 9.46 [4.7, 19.2]; <i>p</i> &#x3c; 0.001) but not age, sex, time to diagnosis, or MND subtype were independently associated with the presence of saliva problems in multivariable regression, and 52.7% (155) of those with saliva problems received pharmacological management. The most commonly used medications were hyoscine, amitriptyline, carbocisteine, glycopyrrolate, and atropine. Evidence base (8, 72.7%) and local guidelines (10, 90.9%) were cited as the most important factors influencing treatment decision by survey respondents (<i>n</i> = 11). <b><i>Conclusion:</i></b> Saliva problems are common and associated with bulbar onset MND. A substantial proportion of pwMND with saliva problems did not receive recommended treatments. Future research is required to determine the relative efficacy of individual pharmacological treatments.

scholarly journals Use of non-invasive ventilation in motor neuron disease – a retrospective cohort analysis

2021 ◽  
Vol 18 ◽  
pp. 147997312110638
Author(s):  
Laura J Walsh ◽  
Kevin F Deasy ◽  
Fernando Gomez ◽  
Elizabeth O’Sullivan ◽  
Joseph Eustace ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Neurodegenerative Disorder ◽  
Cohort Analysis ◽  
Invasive Ventilation ◽  
Non Invasive ◽  
Progressive Muscle Weakness ◽  
Non Invasive Ventilation ◽  
Retrospective Cohort Analysis ◽  
Bulbar Onset

Motor neuron disease (MND) is a neurodegenerative disorder which leads to progressive muscle weakness including respiratory muscle decline. The introduction of non-invasive ventilation (NIV) has been shown to improve quality of life, survival and slow the rate of pulmonary function decline. A retrospective chart analysis of patients who attended the MND clinic from 2014 to 2019 at a tertiary-referral, academic, teaching hospital was carried out to evaluate if NIV and greater compliance with NIV was associated with improved survival. 111 patients were included. The mean age at diagnosis was 63.8 years and 61.3% were males. 66.7% of our cohort used NIV and of this 66.7%, 44.1% were compliant. There was a significantly longer survival in those who used NIV ( p = 0.002) and in those who used NIV optimally ( p = 0.02) when both groups were compared to those who did not use NIV. In the bulbar MND group those who were compliant with NIV survived longer than who those who did not use NIV ( p = 0.001). We found a significantly longer survival with the use of NIV, the use of NIV optimally and with use of NIV in those with bulbar onset MND compared to those who did not use NIV.

scholarly journals Do pain, anxiety and depression influence quality of life for people with amyotrophic lateral sclerosis/motor neuron disease? A national study reconciling previous conflicting literature

2019 ◽  
Vol 267 (3) ◽  
pp. 607-615 ◽  
Author(s):  
Rhiannon Edge ◽  
◽  
Roger Mills ◽  
Alan Tennant ◽  
Peter J. Diggle ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
National Study ◽  
Future Research ◽  
Depression And Anxiety ◽  
Patient Reported ◽  
Lateral Sclerosis

Abstract Introduction The importance of elucidating the relationships between pain, mood and quality of life (QoL) amongst people with amyotrophic lateral sclerosis/motor neuron disease is evident to clinicians, yet the literature is limited and inconsistent. We explored the relationships between pain, depression, anxiety and QoL to reconcile the previous contrasting findings and inform future research and clinical practice. Methods Patient-reported outcomes were obtained as part of the Trajectories of Outcomes in Neurological Conditions study. Mood and QoL scales underwent Rasch analysis. Correlation coefficients examined the strength of association between variables of interest. A bivariate regression model was developed to examine the effects of pain, depression and anxiety on joint psychological and physical QoL domains. Results Of 636 people with ALS, 69% reported pain, of these most had mild pain. Seven percent (7%) of participants exceeded published cutoffs for probable depression and 14% had probable anxiety. Pain, depression and anxiety all influence quality of life; depression has a significant effect on both physical and psychological domains of QoL, whereas pain affects physical QoL and anxiety psychological QoL. Conclusions These results show the importance of expressing quality of life in a conceptually appropriate way, as failing to take account of the multidimensional nature of QoL can result in important nuances being overlooked. Clinicians must be aware that pain, depression and anxiety all worsen QoL across their ranges, and not just when pain is severe or when anxiety or depression reach case level.

Apolipoprotein E ɛ4 in bulbar-onset motor neuron disease

The Lancet ◽  
1996 ◽  
Vol 348 (9023) ◽  
pp. 334-335 ◽  
Author(s):  
R Glenn Smith ◽  
Lanny J Haverkamp ◽  
Susan Case ◽  
Vicki Appel ◽  
Stanley H Appel
Keyword(s):  
Apolipoprotein E ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Bulbar Onset

021 Clinical implications of cortical dysfunction in motor neuron disease: a combined structural and functional approach

2018 ◽  
Vol 89 (6) ◽  
pp. A9.3-A10
Author(s):  
Thanuja Dharmadasa ◽  
Neil G Simon ◽  
Chenyu Wang ◽  
Steve Vucic ◽  
Matthew C Kiernan
Keyword(s):  
Motor Cortex ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Disease Progression ◽  
Diffusion Tensor ◽  
Statistical Parametric Mapping ◽  
Functional Approach ◽  
Cerebral White Matter ◽  
Bulbar Onset ◽  
Cortical Dysfunction

IntroductionMotor neuron disease (MND) is characterised by progressive failure of upper (UMN) and lower motor neurons (LMN). UMN dysfunction remains difficult to detect clinically, and the influence of cortical change on disease progression and prognosis remains unclarified. This study quantitatively assessed cortical dysfunction in vivo using a novel combined structural and functional approach.MethodsForty-one newly diagnosed MND patients and 30 controls prospectively underwent 3T diffusion tensor magnetic resonance imaging (DTI) to assess cerebral white matter structural integrity. DTI measures including apparent diffusion coefficient (ADC) and functional anisotropy (FA) were analysed using statistical parametric mapping. Threshold tracking transcranial magnetic stimulation (TT-TMS) studies were additionally performed across all four limbs to concurrently assess the functional integrity of the motor cortex. All patients underwent detailed clinical and cognitive assessment.ResultsCortical abnormalities were prominent, with FA significantly reduced (p<0.01) and ADC enhanced (p<0.03) along the primary motor tracts in all MND patients, suggestive of primary axonal degeneration. These changes correlated with clinical function and disease duration. TT-TMS revealed evidence of cortical hyper-excitability over the motor cortex corresponding with clinical site-of-onset (p<0.05). Structural (FA) and functional (cortical hyper-excitability) changes correlated across all extremities. Patients without clinical UMN signs also showed evidence of UMN dysfunction, identifying subclinical change. Subgroup analysis identified the greatest changes in bulbar-onset patients (p<0.03).ConclusionThis multimodal technique identified UMN dysfunction in patients prior to clinically detectable change, which may contribute to earlier diagnosis. More significant cortical involvement in bulbar-onset patients aligns with their poorer survival, highlighting the cortically driven determination of clinical progression and prognosis. Further clinical translation of such patterns may therefore lend insight into pathophysiological disease mechanisms and may influence stratification for clinical trials. Overall, this can serve as a robust biomarker for earlier diagnosis, disease progression and prognosis.

Association of apolipoprotein E &isin;4 allele with bulbar-onset motor neuron disease

The Lancet ◽  
1996 ◽  
Vol 347 (8995) ◽  
pp. 159-160 ◽  
Author(s):  
A Al-Chalabi ◽  
M.C Bakker ◽  
C.E Shaw ◽  
C.M Lloyd ◽  
Z.E Enayat ◽  
...  
Keyword(s):  
Apolipoprotein E ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Bulbar Onset

Lack of association of apolipoprotein E ϵ4 allele with bulbar-onset motor neuron disease

1997 ◽  
Vol 41 (3) ◽  
pp. 417-417 ◽  
Author(s):  
Rainer Bachus ◽  
Sebastian Bader ◽  
Reinhard Geßner ◽  
Albert C. Ludolph
Keyword(s):  
Apolipoprotein E ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Bulbar Onset

scholarly journals Use of Non-invasive Ventilation in Motor Neuron Disease – A Retrospective Cohort Analysis

2021 ◽  
Author(s):  
Laura J Walsh ◽  
Kevin F Deasy ◽  
Fernando Gomez ◽  
Elizabeth O’Sullivan ◽  
Joseph Eustace ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Neurodegenerative Disorder ◽  
Pulmonary Function Tests ◽  
Cohort Analysis ◽  
Invasive Ventilation ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Bulbar Onset

Abstract Background Motor neuron disease (MND) is a neurodegenerative disorder which leads to progressive muscle weakness including respiratory muscle function decline. The introduction of non-invasive ventilation (NIV) has been shown to improve quality of life, survival and slow the rate of pulmonary function decline. Better compliance with the machine (using it for greater than 4 hours per day) has been shown to have the greatest long-term benefit. Methods A retrospective chart analysis of patients who attended the MND clinic from 2014-2019 at a tertiary-referral, academic, teaching hospital was carried out to evaluate if NIV and greater compliance with NIV was associated with improved survival. Information regarding NIV use, compliance, pulmonary function tests (PFTs) and basic patient demographics was collected. In total 111 patients were included in the study. Results The mean age at diagnosis was 63.8 years and 61.3% were males. 66.7% of our patient cohort used NIV and of this 44.1% were compliant. There was a statistically significant longer survival in those who used NIV (P=0.002) and in those who used NIV optimally (P=0.02) when both groups were compared to those who did not use NIV. In the bulbar MND group those who were compliant with NIV survived longer than who those who did not use NIV (P=0.001). Patients with bulbar onset MND had worse SNIP and peak cough flow values at diagnosis.Conclusion We found a significantly longer survival with the use of NIV, the use of NIV optimally and with use of NIV in those with bulbar onset MND compared to those who did not use NIV.

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