Abstract
Background
A
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 semirigid 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.1–3
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 30–35 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
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 Communication boards can be useful even after hand motor function is lost. Eye-tracking software can be used to generate typewritten language and electronic speech.