Understanding ALS

What is ALS?

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a fatal neurological disorder that attacks nerve cells responsible for controlling voluntary muscles such as those in the arms, legs, and face. It is the most common form of motor neuron disease, characterized by the gradual degeneration and death of these neurons.

As the motor neurons deteriorate, muscles receive fewer signals from the brain, weakening and leading to atrophy and fasciculations (subtle twitches). Eventually, ALS patients lose the ability to initiate and control voluntary movements. Most critically, when respiratory muscles fail, individuals require ventilatory support, and the majority succumb to respiratory failure within three to five years from the onset of symptoms. Notably, around 10% of patients may survive more than ten years.

Despite primarily affecting motor functions, ALS does not usually impact cognitive abilities. However, some patients might experience changes in cognitive functions such as decision-making and memory, and emotional health can be affected, with instances of depression. Counseling and medication may offer necessary support.

Typically, ALS spares the muscles controlling eye movement, as well as sensory functions like sight, smell, taste, hearing, and touch.

Who Gets ALS?

ALS is rare but is the predominant motor neuron disease globally. Each year, it affects about 2.2 people per 100,000 in Europe and leads to more than 5,600 diagnoses in the United States, where it accounts for two deaths per 100,000 annually. The disease usually appears randomly, with 90 to 95% of cases having no identifiable risk factors or family history. However, 5 to 10% of cases are inherited, often due to mutations in specific genes.

Symptoms of ALS

ALS begins subtly, with initial symptoms including muscle cramps, stiffness, weakness in an arm or leg, and difficulty with speech and swallowing. As the disease progresses, these symptoms extend to more severe muscle atrophy, affecting manual dexterity and mobility. Symptoms such as difficulty swallowing and speaking increase with disease progression, and eventually, individuals lose the ability to perform daily activities independently.

Over time, ALS patients may require assistance with eating due to the risk of choking and will experience significant weight loss. Although mental faculties generally remain intact, some individuals develop cognitive impairments or dementia. Health care professionals play a crucial role in outlining the disease trajectory and discussing treatment options to help patients make informed decisions. In advanced stages, patients depend on ventilatory support as respiratory muscles weaken, increasing their risk of complications like pneumonia.