A multicentre evaluation of oropharyngeal secretion management practices in amyotrophic lateral sclerosis

McGeachan, Alexander; Hobson, Esther; Al‐Chalabi, Ammar; Stephenson, Jodie; Chandran, Siddharthan; Crawley, Francesca; Dick, D J; Donaghy, Colette; Ellis, Cathy; Gorrie, George; Hanemann, C. Oliver; Harrower, Timothy; Jung, Agam; Malaspina, Andrea; Morrison, Karen; Orrell, Richard W.; Talbot, Kevin; Turner, Martin R; Williams, Tim; Young, Carolyn; Shaw, Pamela J.; McDermott, Christopher J.

doi:10.1080/21678421.2016.1221433

Cited by 22 publications

(20 citation statements)

References 27 publications

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“…Sialorrhea (hypersalivation) due to impaired swallowing can be managed with anticholinergic medications, salivary gland botulinum toxin injections, or radiotherapy. 86,87 Suction machines can be used to remove oral and pharyngeal secretions. Thick secretions can be liquefied with hydration, oral mucolytics, or nebulized acetylcysteine.…”

Section: Clinical Carementioning

confidence: 99%

Amyotrophic Lateral Sclerosis: An Update for 2018

Oskarsson

Gendron

Staff

2018

Mayo Clinic Proceedings

262

178

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons and other neuronal cells, leading to severe disability and eventually death from ventilatory failure. It has a prevalence of 5 in 100,000, with an incidence of 1.7 per 100,000, reflecting short average survival. The pathogenesis is incompletely understood, but defects of RNA processing and protein clearance may be fundamental. Repeat expansions in the chromosome 9 open reading frame 72 gene (C9orf72) are the most common known genetic cause of ALS and are seen in approximately 40% of patients with a family history and approximately 10% of those without. No environmental risk factors are proved to be causative, but many have been proposed, including military service. The diagnosis of ALS rests on a history of painless progressive weakness coupled with examination findings of upper and lower motor dysfunction. No diagnostic test is yet available, but electromyography and genetic tests can support the diagnosis. Care for patients is best provided by a multidisciplinary team, and most interventions are directed at managing symptoms. Two medications with modest benefits have Food and Drug Administration approval for the treatment of ALS: riluzole, a glutamate receptor antagonist, and, new in 2017, edaravone, a free radical scavenger. Many other encouraging treatment strategies are being explored in clinical trials for ALS; herein we review stem cell and antisense oligonucleotide gene therapies.

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Section: Clinical Carementioning

confidence: 99%

Amyotrophic Lateral Sclerosis: An Update for 2018

Oskarsson

Gendron

Staff

2018

Mayo Clinic Proceedings

262

178

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“…Other treatment options for sialorrhea in neuromuscular patients include anticholinergic drugs, radiation therapy and surgical manipulation of salivary glands (15). Anticholinergic drugs are limited by side effects (16). Radiation therapy lacks consensus on the type of radiation and optimal dose (17).…”

Section: Discussionmentioning

confidence: 99%

Efficacy of Botulinum Toxin for Treating Sialorrhea in Neuromuscular Conditions

et al. 2020

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Background: Drooling related to bulbar weakness and dysfunction is a common concern in patients with neuromuscular disease. While there are numerous medications to manage sialorrhea, they are often limited by side effects and lack of efficacy. Botulinum toxin has shown to benefit ALS patients in a few studies, but there is scant data on the benefit in other neuromuscular conditions. Objective: To assess the effectiveness of Botulinum toxin in reducing sialorrhea in patients with various neuromuscular disease. Design/Methods: 25 patients (19M, 6F; 54.36 ± 17.09 yr) with documented neuromuscular illness and concern for drooling was followed for 6 weeks after Botulinum toxin injection. These patients had one of the following diagnoses: Duchenne muscular dystrophy (3), myotonic dystrophy (3), oculopharyngeal muscular dystrophy (1), inclusion body myositis (2), primary lateral sclerosis (1), amyotrophic lateral sclerosis (9), spinal muscular atrophy type 2 and 3 (2), spinal-bulbar muscular atrophy (2), and Becker's muscular dystrophy (2). A subjective drooling scale (1: markedly worse, 5: markedly better) and drooling thickness score (0=normal, 100=thick) was calculated on these patients prior to the injection and 4 and 6 weeks after the injection. Botulinum toxin 20-30 units were injected into bilateral parotid gland (70% of the dose) and submandibular gland (30% of the dose). Results: The drooling thickness score at before the injection was 75.2 ± 10.46. At 4 and 6 weeks, average scores reduced to 47.2 ± 6.14 and 18.8 ± 5.26, respectively (p < 0.05). The average pre injection perception about drooling was 3.0 (p < 0.05). The average change in perception was +0.84 and +1.28 at 4 and 6 weeks, respectively, (p < 0.05) implying significant improvement. There were no reported adverse effects. Conclusion: This study provides preliminary evidence for the use of botulinum toxin for refractory sialorrhea for a variety of neuromuscular conditions.

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“…However, there is increasing evidence that first‐line anticholinergic therapy should be glycopyrronium. In a number of studies, including the current one, glycopyrronium is the most effective anticholinergic treatment and has a much‐reduced side effect profile, in large part because it does not cross the blood–brain barrier …”

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confidence: 99%

“…In a number of studies, including the current one, glycopyrronium is the most effective anticholinergic treatment and has a much-reduced side effect profile, in large part because it does not cross the bloodbrain barrier. 1,3 There have been few attempts to collect systematically prospective outcome data from patients receiving anticholinergic therapy. The choice of a patient reported outcome rather than an objective measure of saliva volume is welcome, as the latter has been shown to not correlate with the burden and impact of saliva related symptoms.…”

mentioning

confidence: 99%