An uncommon neurological condition called amyotrophic lateral sclerosis (ALS) predominantly affects the nerve cells (neurons) in charge of regulating voluntary muscular activity (those muscles we choose to move). Talking, walking, and chewing are all movements made voluntarily by the muscles. Since the illness is progressive, its signs and symptoms progressively worsen. There is currently no viable treatment to slow or stop the disease’s progression from ALS.

ALS is a member of a larger class of conditions known as motor neuron diseases, which are brought on by the death and slow degeneration of motor neurons. The nerve cells known as motor neurons travel from the brain to the spinal cord and then to the muscles all over the body.

As motor neurons age, they stop communicating with the muscles, which causes the muscles to progressively deteriorate, twitch, and die (atrophy). The capacity of the brain to initiate and regulate voluntary motions eventually declines.

Muscle stiffness or weakness in the hand and legs are frequently early signs that you might be suffering from  ALS. Individuals slowly lose strength, and the capacity to speak, eat, move, and even breathe, and all voluntary muscles are damaged. Within 3 to 5 years of their first symptom onset, most ALS patients pass away from respiratory failure.

The Early Signs

When ALS first manifests, the symptoms may be so mild that they go unnoticed, but over time, they progress to more pronounced atrophy or weakness.

Early signs consist of:

  • Muscle jerks in the mouth, shoulder, arm, or leg
  • muscle pain
  • muscular spasms and stiffness (spasticity)
  • weakening of the neck, diaphragm, arm, or leg muscles
  • nasal and slurred speech
  • difficulty in swallowing or chewing

The initial sign of ALS typically manifests in the hand or arm and can manifest as difficulties performing routine actions like writing, buttoning a garment, or turning a lock key. Other times, only one leg is initially affected by symptoms.

Muscle atrophy and weakening expand to additional body areas as the disease worsens. People may experience issues with their ability to move, swallow (dysphagia), speak or form words (dysarthria), and breathe (dyspnea). Individuals gradually lose the ability to stand or walk, get in or out of bed independently, or use their hands and arms, though the order in which symptoms first appear and the speed at which the disease advances might differ from person to person.

It might be challenging for those with ALS to swallow and chew their diet because of this. They also burn calories more quickly than the majority of people who do not have ALS. These factors contribute to the rapid weight loss and potential malnutrition that ALS patients experience.

Current Research On ALS

The principal federal supporter of research on diseases of the brain and nervous system, including conditions like ALS, is the National Institute of Neurological Disorders and Stroke (NINDS). The National Institutes of Health (NIH), the world’s largest funder of biomedical research, includes NINDS as one of its divisions.

Understanding the molecular mechanisms behind the onset and course of the disease, looking into the impact of genetics and other potential risk factors, finding biomarkers, and creating new treatments are the main objectives of ALS research at NINDS.

Cell Defects: Current research aims to comprehend the mechanisms that specifically cause motor neurons to degenerate in ALS, which may result in efficient methods to stop this process. Motor neuron death may be brought on by a number of cellular problems, such as those affecting protein recycling and gene control, as well as motor neuron structural flaws, according to research employing cellular culture systems and animal models. Inflammatory cells of the neurological system and glial support cells may both play a significant role in ALS, according to growing data.

Stem Cell Therapy: In order to create stem cells that can differentiate into any sort of cell, including motor neurons and other cells that may be involved in the disease, scientists are using skin cells from ALS patients. In order to better understand the function of the neurons involved in ALS, researchers supported by NINDS are growing human spinal cord sections on tissue chips utilising stem cells.

Epigenetics: NINDS NIH National Institute of Neurological Disorders and Stroke-funded clinical research projects are examining how ALS symptoms evolve over time in individuals with C9ORF72 mutations. Additional genes that may contribute to or increase a person’s risk for either familial or sporadic ALS are being sought after by other studies.

New choices for treatment

In a variety of illness models, this effort involves testing drug-like substances, gene therapy techniques, antibodies, and cell-based therapeutics. ALS patients are also being examined for a number of investigational therapies.