Background Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder leading to weakness, spasticity, and incoordination of the muscles involved in speaking, swallowing, breathing, and ambulation. Currently, there is no cure for ALS and the disease progresses inexorably toward death. Despite the lack of cure, there are available treatments that can improve quality of life. This Fast Fact will discuss non-pharmacologic therapies for common challenges faced by ALS patients. See Fast Fact ### for pharmacologic therapies and Fast Fact ### for management of sialorrhea specifically.
Head Drop Axial muscle involvement, particularly neck extensor can lead to disabling head drop and kyphosis. A soft collar may be helpful early in the course of disease, but as the disease progresses, a semi-rigid collar is often needed to stabilize the neck and restrict motion. Individualized collars and adjustable head rests on wheelchairs may be needed considering the significant variability in neck anatomy (1,2,3).
Dysphagia Dysphagia is a common bulbar manifestation of ALS and should be assessed each visit (4). Poor nutritional status at diagnosis or disease progression has been associated with higher mortality (5).
- Early referrals to a dietitian and speech pathologist are recommended. Use of thickened fluids, high-protein/high-calorie supplements, and modified swallowing techniques can mitigate the malnutrition associated with dysphagia (4,6).
- Gastrostomy tubes do not prevent aspiration, but they have been shown to improve nutritional status and may prolong survival. It is highly recommended that if patients are agreeable to getting a gastronomy tube, it be done before vital capacity falls below 50% of predicted (4,7).
- Nasogastric tubes have been used as a short-term alternative, but they are uncomfortable and may worsen sialorrhea (4,8).
Ventilatory Compromise The most common cause of death in ALS is ventilatory failure (9). Symptoms of ventilatory compromise, such as poor nighttime sleep, daytime somnolence, anorexia, morning headache, and weak cough, often precede dyspnea.
- Noninvasive ventilation (NIV) with bilevel positive airway pressure has been shown to prolong survival and improve quality of life in patients with ALS who can maintain their airway (10). Nasal masks/pillows and sip/puff devices may improve tolerability.
- Mechanical in-exufflators alternate positive and negative pressures to improve airflow and clearance of secretions. These devices may reduce pulmonary morbidity and associated hospitalizations in muscular dystrophy (11), but there are no similar studies specific to ALS.
- Diaphragmatic pacemakers can be surgically implanted to stimulate more forceful muscle contractions in patients with some degree of residual diaphragm function. Their effectiveness has yet to be confirmed in randomized controlled trials.
- When NIV is no longer adequate or tolerated (i.e. inability to clear one’s own secretions), a small percentage of patients pursue long-term mechanical ventilation. See Fast Fact #73.
Impairment of Mobility Physical therapy and use of equipment such as canes, walkers, and ankle-foot orthoses can minimize foot-drop, improve gait, and help prevent falls (12-14). Occupational therapy with assistive devices such as modified cutlery, Velcro fasteners for dressing, and bathroom modifications such as grab bars and higher toilet seats help maintain function (12-14). In patients with prominent distal weakness, wrist braces at 30 to 35 degrees can improve grip efficiency while a universal cuff can assist with eating and typing (14). Early intermittent use of a wheelchair is recommended for energy conservation (14). Modifiable controls, such as a joystick that requires minimal arm/hand strength (12), make power wheelchairs a better long-term option than power scooters for maintaining mobility. Modified remote controls and security systems may allow patients to maintain employment (12).
Impairment of Communication Communication boards can be useful even after hand motor function is lost. Computer, tablet, or smartphone applications can be used to generate electronic speech from typed language. Patients can preemptively record their speech using voice banking systems to preserve the personalization, inflection, and accent of the electronic speech (15). Eye-tracking software can be used to generate typewritten language and electronic speech.
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2. Jenkins TM, Hollinger H, McDermott CJ. The evidence for symptomatic treatments in amyotrophic lateral sclerosis. Curr Opin Neurol. Oct 2014;27(5):524-531.
3. Dal Bello-Haas V, Kloos AD, Mitsumoto H. Physical therapy for a patient through six stages of amyotrophic lateral sclerosis. Phys Ther. Dec 1998;78(12):1312-1324.
4. Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)–revised report of an EFNS task force. Eur J Neurol. Mar 2012;19(3):360-375.
5. Marin B, Desport JC, Kajeu P, et al. Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry. Jun 2011;82(6):628-634.
6. Desport JC, Preux PM, Truong CT, Courat L, Vallat JM, Couratier P. Nutritional assessment and survival in ALS patients. Amyotroph Lateral Scler Other Motor Neuron Disord. Mar 2000;1(2):91-96.
7. Heffernan C, Jenkinson C, Holmes T, et al. Nutritional management in MND/ALS patients: an evidence based review. Amyotroph Lateral Scler Other Motor Neuron Disord. Jun 2004;5(2):72-83.
8. Scott AG, Austin HE. Nasogastric feeding in the management of severe dysphagia in motor neurone disease. Palliat Med. 1994;8(1):45-49.
9. Mitchell JD, Borasio GD. Amyotrophic lateral scerosis. Lancet. June 2007;369(9578):2031-2041.
10. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. Feb 2006;5(2):140-147.
11. Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest. 1997;112(4):1024-1028.
12. Ng L, Khan F. Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis. In: Mauer MH, ed. Amyotrophic Lateral Sclerosis. Rijeka, Croatia: InTech; 2012:557-578.
13. Blatzheim K. Interdisciplinary palliative care, including massage, in treatment of amyotrophic lateral sclerosis. J Bodyw Mov Ther. Oct 2009;13(4):328-335.
14. Francis K, Bach JR, DeLisa JA. Evaluation and rehabilitation of patients with adult motor neuron disease. Arch Phys Med Rehabil. Aug 1999;80(8):951-963.
15. Yamagishi J, Veaux C, King S, Renals S. Speech synthesis technologies for individuals with vocal disabilities: Voice banking and reconstruction. Acoust Sci Technol. 2012;33(1):1-5.
Author’s Affiliations: Mayo Clinic, Jacksonville, FL
Conflict of Interest: The authors have disclosed no relevant conflicts of interest.
Version History: First electronically published June 2015
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