External radiation of the parotid glands significantly reduces drooling in patients with motor neurone disease with bulbar paresis
Introduction
Drooling and subjective sialorrhea are common in amyotrophic lateral sclerosis (ALS) patients with facies–lingual–pharyngeal paresis, in particular in the later stages of the disease when severe paresis can develop. The symptoms can cause considerable social embarrassment and discomfort, difficulty in speaking, produce foul odors and increase the risk of aspiration pneumonia.
A wide range of different methods has been tried to alleviate drooling in patients with bulbar paresis. Commonly used medication is anticholinergic treatment often in the form of atropine tablets or mixture, nebulized scopolamine [1] or scopoderm transdermal patch [2]. A disadvantage of anticholinergic drugs is their side effects, which can become pronounced especially in elderly patients. Oral glycopyrrolate at 0.4 mg thrice daily has been suggested as an alternative, its chief advantage is that it does not cross the blood–brain barrier [3], [4]. Recently, injection of botulinum toxin into the parotid glands has been tried in five bulbar ALS patients with satisfactory results [5]. Several surgical procedures have been tried to reduce drooling including simple unilateral tympanic neurectomi, excision of one or more of the major salivary glands, laser intraductal photocoagulation of the parotid ducts [6], transposition and rerouting of the ducts [7], intraoral bilateral submandibular and parotid duct ligation [8], or a combination of these procedures [9].
We have tried several of these options in ALS patients with mixed results. Although the drug treatment is often successful initially, as bulbar paresis progresses, the increasing doses needed to relieve symptoms causes unacceptable side effects. The invasive surgical procedures are not without risk of complications including xerostomia necessitating the administration of artificial saliva. Borg and Hirst [10] reported beneficial effect of radiotherapy for treating sialorrhea in 31 patients with various diagnosis including two cases with motor neuron disease, some patients developing long-term side effects.
We have therefore investigated other options and now present the results of a prospective pilot study of lenient low-dosage radiation therapy in the management of drooling in ALS.
Section snippets
Patients and methods
Eighteen ALS patients with severe drooling and a life-expectancy of less than 2 years (based on previous disease history) were included. Ten were females and eight were males. Their mean age at the time of radiotherapy was 62.3 years, and the mean disease duration 22 months. At the time of inclusion, the patients were severely disabled with a score ranging from 14/40 to 6/40 on the ALS Functional Rating Scale [11] and fulfilled rigorous criteria for probable or definite ALS [12]. The patients
Results
The mean parotid salivary secretion rate was 0.29 ml/min (varied from 0.04 to 1.08 ml/min) before irradiation. The parotid salivary secretion diminished significantly 1 day (43%) and 2 weeks (57%) after irradiation (Table 1).
Subjectively, the patients reported diminished drooling beginning 12–24 h after radiation therapy. One patient reported no effect of the therapy.
For all patients, the caregivers noted diminished salivary secretions and no aspiration pneumonia. Anticholinergic treatment
Discussion
The oral–pharyngeal secretions are a combination of secretions from the salivary glands in the oral cavity, from the nasal glands, and from the mucosa in the pharynx, larynx and lower airways. The daily salivary secretion is estimated to be 500–600 ml with large individual variations [13]. Unstimulated saliva secretion rate in healthy individuals is reported to be 0.3 ml/min for whole saliva and 0.04 ml/min from each parotid gland [13].
The parotid glands normally contributes 20% of the total
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Prevalence of Sialorrhea Among Amyotrophic Lateral Sclerosis Patients: A Systematic Review and Meta-Analysis
2022, Journal of Pain and Symptom ManagementCitation Excerpt :At last, sialorrhea in ALS is currently treated as follows. According to the national ALS Patient CARE database, the ALS patients with mild-to-moderate drooling may be easier to benefit from atropine, glycopyrrolate, or amitriptyline39,40 than the ALS patients with severe drooling because of some side effects of using anticholinergic drugs, such as urinary retention, cutaneous reactions, blurred vision and other adverse reaction.34,39,41,42 Therefore, when anticholinergic drugs may not valid for the ALS patient with severe drooling, botulinum neurotoxin (BoNT) injected into the salivary glands, radiation therapy (RT),surgical options and neurectomy are also a option to choose.43
Symptom Management and End-of-Life Care in Amyotrophic Lateral Sclerosis
2015, Neurologic ClinicsManaging excessive saliva with salivary gland irradiation in patients with amyotrophic lateral sclerosis
2015, Journal of the Neurological SciencesRadiation therapy for hypersalivation: A prospective study in 50 amyotrophic lateral sclerosis patients
2014, International Journal of Radiation Oncology Biology PhysicsCitation Excerpt :In addition, we showed in the same study that patients with severe salivation (SSS score, ≥6) were poorly discriminated by other scales such as the Oral Secretion Scale or ALSFRS (12). Various schedules of RT have been reported, ranging from 4 to 48 Gy (4-8, 16, 17). A single low-dose (7-Gy) fraction was suggested to be as effective as higher total dosage in drooling reduction (5-7).
Salivary glands radiotherapy to reduce sialorrhea in amyotrophic lateral sclerosis: Dose and energy
2013, Cancer/RadiotherapieCitation Excerpt :However, amyotrophic lateral sclerosis is a rare disease, and radiotherapy for sialorrhea is typically proposed only after failure (and/or toxicity) of other treatments. Thus, this group of patients is one of the largest in the current literature [10,12–15]. Moreover, in a prospective series of nine patients, Harriman et al. described the difficulty of recruiting patients that presented the studied symptoms; however, they explained that a subjective evaluation of these patients was valid [14].