Abstract
Background
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This will be the first of three Fast Facts addressing the management of common ALS symptoms. The intent of these Fast Facts is to inform readers of common symptoms clinicians may encounter when caring for ALS patients and to provide a brief overview on how to manage these symptoms. For more complex presentations, involvement of a neurologist, physical medicine and rehabilitation (PMR) clinician, or ALS specialist is advised.
Sialorrhea
Bulbar weakness, spasticity, and loss of muscle control in ALS can lead to dysphagia, which causes difficulty swallowing one's saliva. In ALS, this condition is referred to as sialorrhea, although it is caused by difficulty clearing secretions rather than by an increase in saliva production as is the case in other conditions associated with the term sialorrhea. Sialorrhea in ALS often leads to drooling, which can be socially disabling, as some patients are reluctant to engage in social interactions as a result of embarrassment. Difficulty with secretion management also increases the risk of perioral skin irritation and aspiration. There are various pharmacologic and nonpharmacologic management strategies for the treatment of sialorrhea in ALS:
• Experts often use medications that inhibit saliva production, such as atropine, glycopyrrolate, tricyclic antidepressants, and scopolamine patches, although there are no randomized trials specific to ALS that evaluate the effectiveness of these medications. These medications capitalize on the anticholinergic side effect of xerostomia; therefore, other less desirable anticholinergic side effects such as constipation, urinary retention, and cognitive dysfunction are a risk. • The effectiveness of botulinum toxin injections into salivary glands has been demonstrated in randomized trials.2,3 The toxin reduces the production of saliva by inhibiting the release of acetylcholine at neurosecretory junctions within the salivary glands. Botulinum toxin A and B appear to have similar efficacy.
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The injections are typically performed by a neurologist in the office setting and generally must be repeated every three months. Inadvertent infiltration into adjacent muscles is a small risk. Rarely, xerostomia, dysphagia, or thickened secretions occur, but the effects are most often temporary. Advantages of botulinum toxin treatment for sialorrhea are reduced systemic side effects and lack of drug-drug interactions. • To minimize drooling, portable suction devices can be used to clear excess secretions. • In refractory cases, unilateral salivary gland irradiation delivered over one to five fractions may improve sialorrhea within 24 hours.
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Maximum benefit usually occurs within a week. Although xerostomia is a risk, salivary function often returns after three months; hence retreatment may be indicated. • In rare cases, laryngectomy is used for secretion management and prevention of aspiration in patients whose speech is already severely compromised (as it completely eliminates a patient's ability to speak). This approach can be used regardless of whether a patient chooses long-term mechanical ventilation for ventilatory failure.
Summary
Although there is currently no cure for ALS, there are numerous meaningful interventions for the symptoms of the disease. Please see Fast Fact #301 for pharmacologic therapies and Fast Fact #300 for nonpharmacologic therapies used in other common challenges faced by patients with ALS.