ALS is a progressive neurological disease. Symptoms begin to manifest slowly, and progress to complete inability to move.
Symptoms that occur early in the disease may include difficulty with fine motor functions. As the disease progresses some signs include:
- Difficulty grasping an ink pen or holding the handle of a cup.
- Difficulty walking.
- Difficulty performing normal activities of daily life.
- Increased number of falls.
- Weakness in the legs, feet, ankles and/or hands.
- Slurred speech.
- Difficulty swallowing.
- Muscle spasms in the upper extremities or lower extremities.
- Inability or difficulty in holding one’s posture appropriately.
- It may become increasingly difficult for the patient to hold his/her head up and keep good posture, as a result of weakening muscles.
As the condition gradually moves into the later stages of the disease, patients will experience severe weaknesses of muscles and paralysis. The patient’s lungs will become affected and the result will be an inability to breathe without ventilatory support. Ventilator support is required in many patients with late stage ALS.
ALS only affects motor neurons, the afferent (sensory) neurons are spared. Therefore, the patient will not lose sensation, touch, hearing, taste and smell. For some patients, the muscles of the eyes may be spared. Each case is unique and the development of symptoms will depend on which areas are affected.
Complications can be significant and life-threatening. Complications that are commonly associated with late stages of ALS include: breathing difficulty, speaking difficulty and eating difficulty.
As discussed above, in late stages of ALS, the patient’s lungs begin to be affected. Over time, the lung muscles become paralyzed, which can be life-threatening. However, there are interventions for this complication.
The first type of intervention is CPAP or BiPAP. These are machines that will help the patient breathe better in the night. Other interventions for more severe cases include tracheostomy and respirator, that will function to mimic inspiration and expiration. Respiratory failure is the most common cause of death in patients with advanced ALS.
The inability to speak is another complication associated with ALS. Speech difficulty can be present in early stages as well as late stages. As ALS is a progressive disease, speech difficulties may start with mild or occasional slurring of words, but then over time can become so severe that the patient cannot be understood by others. At this point in time, verbal communication is no longer an option, and other forms of communication must be utilized.
As with all other functions in the body, eating requires muscle strength. The muscles that are required for swallowing become weak, and later paralyzed, making it difficult, and eventually impossible, to swallow food and water. In later stages of ALS, the patient can become malnourished and dehydrated. If these patients try to consume food and/or liquid orally, they have a significantly increased chance of aspiration. With aspiration comes complications of severe infections that can be life-threatening. To address this a feeding tube, either a PEG tube or an NG tube, can be utilized to provide hydration and nutrition.