All you need to know about: Amyotrophic lateral sclerosis

All you need to know about: Amyotrophic lateral sclerosis


Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurological disorder that affects muscle movement. The name originates from the Greek amyotrophic, meaning lack of muscle nourishment, with ‘a’ meaning ‘no’, ‘myo’ referring to ‘muscle’ and ‘trophic’ to ‘nourishment’. Lateral indicates the spinal cord, where portions of nerve cells that signal and control muscles, are located. Degeneration of this area leads to scarring or hardening, known as sclerosis.

What is ALS?

The human body’s muscle movements are governed by motor neurons, which are nerve cells in the brain and spinal cord. In people with ALS, the motor neurons degenerate and die, and so, messages sent by the brain do not reach the muscles, with the result that the muscles atrophy (lose strength and become weak). Eventually, the brain loses control over voluntary movements including walking, chewing and even breathing, as the condition progresses. 

Over 90% of ALS cases are sporadic, with no familial history of the disease. The condition is linked to lifestyle, occupational and environmental factors, while it also believed that genetic mutations could play a role. 

Risk factors

ALS can occur at any age but symptoms commonly develop between the age of 55 and 75, with men more than women likely to develop the condition, the NINDS states. High blood pressure and smoking are risk factors.

The disorder is commonly known as Lou Gehrig’s disease, named after an American baseball player, who had to retire from active sports because of the condition. 

Symptoms and progression

Early symptoms of ALS include muscle twitches in the arms, leg, shoulders or tongue; muscle cramps; tightness or stiffness of muscles; muscle weakness in a limb or the neck; slurred or nasal speech; and difficulty in chewing or swallowing. 

As the condition advances it manifests as difficulty in chewing food and swallowing; drooling; difficulty in breathing; unintentional emotional displays; and constipation. People with advanced ALS face difficulties in standing, walking, using their limbs and though their ability to reason, remember and understand is not affected, patients can get depression and frontotemporal dementia, known as FTD-ALS.

The United States’ National Institute of Neurological Disorders and Stroke (NINDS) states that most people with ALS die from respiratory failure, within three to five years from the time symptoms appear. One in 10 people survive for 10 years or more.

Though there is currently no cure for the condition, various treatments and therapies can help manage symptoms and improve quality of life.  

ALS in India

A study, ‘Clinical Profile of Indian Patients with Amyotrophic Lateral Sclerosis (ALS): A Retrospective Hospital Based Study’ published in 2013 in the peer-reviewed journal Neurology evaluated the clinical profile of persons with ALS from a tertiary referral centre in northern India. The study was conducted by Nirendra Rai et al. Dr. Rai is a neurologist who was associated with trauma and emergency medicine at the All India Institute of Medical Sciences, Bhopal at the time of the study. It found that the onset of the condition was earlier in Indian ALS patients. 

Men were younger than women at onset of the disorder, but presented late for treatment. Bulbar onset ALS (a type of ALS in which motor neuron degeneration begins in the brainstem’s bulbar region) was more frequent in elderly patients. The study concluded that the difference in presentation in men and women called for further evaluation, including genetic/hormonal influences.

Diagnosis and treatment

Diagnosis involves testing blood and urine samples, a spinal tap where a sample of spinal fluid is taken by inserting a needle between two bones of the lower back; nerve conduction studies to measure the nerves’ ability to send impulses to different parts of the body; an electromyogram to record the activity of muscles; and muscle and nerve biopsies.

There is no treatment to reverse nerve degeneration, but some therapy may help to delay progression. A treatment plan would include physical, occupational and speech therapy to improve quality of life. Physical therapy helps to reduce the risk of falls and joint pain, improves mobility and independence. Exercises such as walking, swimming and biking help maximise muscle strength and enable them retain their function.

Occupational therapy and speech therapy also help enable persons to live independently. As the condition advances, assistive devices can further help patients.

Research into the condition

Research is ongoing to identify biomarkers associated with ALS. Studies are also ongoing to understand what triggers motor neuron degeneration in ALS. Studies done so far suggest that glial support cells (cells that support neurons) and inflammation cells of the nervous system may have an important role in ALS.

A 2016 paper, ‘Projected increase in amyotrophic lateral sclerosis from 2015 to 2040’, published in Nature Communications, by Arthur, K., Calvo, A., Price, T. et al notes thatthe number of cases of ALS in the world will increase from 222,801 in 2015 to 376,674 in 2040, representing an increase of 69%. The largest increase will be seen in Africa with 116%, followed by Asia with 81% and South America with 73%, it said.

(R. Sujatha is an independent journalist based in Chennai. sujatha.raghunath@gmail.com)

 

Published – November 26, 2025 04:45 pm IST



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