ALS—amyotrophic lateral sclerosis or Lou Gehrig’s Disease—is a degenerative disease that affects the motor neurons in the brain and spine responsible for voluntary movement.
While there is no cure, new drugs are available to slow the onset of more severe symptoms. This not only extends life but also increases the quality of it. A prompt diagnosis is key to beginning effective treatment, and recognizing that something is wrong is key to a prompt diagnosis. Here’s a look at the signs of ALS, how symptoms tend to progress, and the survival rate for the disease.
Symptom Onset
ALS is categorized by two types of onset: limb onset ALS and bulbar onset ALS. Three-quarters of those diagnosed with ALS first notice a weakness in their limbs. The other 25% experience changes in a different area of the brain as the disease presents with slowed speech in the affected individual. Onset symptoms might also entail a change in the pitch of your voice, difficulty picking up a pen, muscle tremors, cramping, or twitching. Other common signs include frequent tripping, difficulty balancing, clumsiness, fatigue, and even trouble maintaining posture or keeping your head up.
Symptomatic Progression
In many cases, the disease progresses to severe symptoms before a diagnosis is made. As motor neurons begin to degenerate further, not all parts of the body are affected in the same way. Some muscles may start to become weaker, while other muscles begin to paralyze. Joints may start to become sore or misshapen. Swallowing can become difficult, and it may eventually become necessary to use a feeding tube to prevent malnutrition. As diaphragm muscles grow weak, artificial respiration may be necessary. It can become difficult to chew, swallow, speak, walk, and perform daily tasks.
Some patients experience a condition called "pseudobulbar affect," which is characterized by uncontrollable bouts of inappropriate laughter or tears. As respiratory insufficiency becomes more pronounced, patients may notice greater fatigue, headaches, difficulty concentrating. They may also become more susceptible to respiratory infections like pneumonia. The brain generally remains completely aware, and the bladder and bowels retain control. Patients also find their memory and ability to make decisions are impaired; frontotemporal lobe dementia is not uncommon in ALS patients.
Survival Rate
ALS can become fatal in multiple ways. Specifically, the muscles of the diaphragm can become so weak that breathing becomes virtually impossible. Less common, but also possible, is that eating and drinking without a feeding tube become so difficult that patients may die of malnutrition or dehydration. Most patients average around a three- to five-year survival rate, but this is not a certainty.
Lou Gehrig lived only two years after his diagnosis; Stephen Hawking was diagnosed with ALS in 1963 and is still alive. In an interview with Scientific American, Leo McCluskey, neurologist and ALS expert, cites Hawking as an "incredible, incredible example of the variability of the disease." It also shows how many questions about ALS remain unanswered—questions researchers hope to solve.
