Why in news?
A recent epidemiological study projected that global cases of Amyotrophic Lateral Sclerosis (ALS) will rise by nearly 70 per cent by 2040, underscoring the need for greater awareness and research into this debilitating condition.
Background
ALS, also called Lou Gehrig’s disease, is a progressive neuro‑degenerative disorder that attacks the nerve cells (motor neurons) controlling voluntary muscles. It usually begins in middle age (55–75 years) and gradually weakens muscles responsible for movement, speech, swallowing and breathing. Most cases are sporadic, though about 10 per cent have a genetic link.
How ALS affects the body
- Motor neuron loss: In ALS, both upper and lower motor neurons deteriorate. As these nerve cells die, electrical signals no longer reach muscles, causing them to atrophy.
- Progression: Symptoms may start with muscle twitches, cramps and weakness in a limb or the neck. Over time patients experience difficulty walking, chewing, speaking and even breathing. Sensory nerves and cognitive function are usually preserved.
- Complications: Progressive muscle weakness can lead to paralysis, respiratory failure, malnutrition (due to swallowing problems) and pneumonia. Some patients develop fronto ‑temporal dementia.
Causes and risk factors
- Genetic mutations: About 5–10 per cent of ALS cases are inherited. Mutations in genes such as SOD1, C9orf72, TARDBP and FUS are implicated.
- Environmental factors: Smoking, exposure to environmental toxins, military service and traumatic injuries have been associated with increased risk, although exact mechanisms are unclear.
- Age and sex: The disease typically strikes people in their sixties and seventies and is slightly more common in men than women.
Treatment and management
- No cure: Currently there is no therapy to halt nerve degeneration. Medications such as riluzole and edaravone may modestly slow disease progression.
- Supportive care: Physical, occupational and speech therapy help maintain mobility and communication. Nutritional support and ventilation may be needed in advanced stages.
- Research: Scientists are investigating gene therapy, stem cell treatments and neuroprotective drugs. Early diagnosis and multi‑disciplinary care can improve quality of life.
Source: TH
