Know more about ALS

You've seen videos and pictures of celebrities participating the ice bucket challenge on the internet. And though most you haven't attempted the challenged, you've done your small contribution by spreading the message on Facebook or Twitter. But how many of you actually know about the ALS disease -- it's diagnosis, symptoms, causes and treatment? Read on to know more.

What is it?
ALS stands for Amyotrophic Lateral Sclerosis. "It is a chronic disorder in which motor neurons of nervous system undergo progressive degeneration. In ALS, motor neurons which help us to contract our muscles, helping body movements slowly degenerate. This results in a progressive weakness of the muscles affected and resulting in severe disability," explains Dr P P Ashok, consultant senior neurologist, Hinduja Healthcare Surgical.

Whom does it affect?
Incidence of ALS is higher in men than in women with an overall male-to-female ratio of 1.5-2: 1. Later in life, the incidence tends to equalise; this occurs at age 40-50 years in some populations and after the age of 65-70 years in others. Onset of ALS may occur from the teenage years to the late 80s; the incidence rises with increasing age until approximately age 75-80 years, informs Dr Ashok. This disease is not of a modern age and has been there for ages. Globally, it affects one in 100,000 individuals and there has been no increase in the number of incidences, he adds.

Symptoms
The basic symptom is the weakness and wasting away of the muscles of the body, and can occur in any part of the body. Dr Darpan J Thakre, consultant neurosurgeon, AVIVA Hospitals encounters, who encounters one case every six months (usually among labour class) has observed the following symptoms:
- Thining of the hands, especially the palmar surface.
- Difficulty in swallowing.
- Heavyness of the tongue.
- Tightness of the leg.

Diagnosis
It is diagnosed by typical history and clinical findings of the patient. EMG (neurophysiological) test confirms the diagnosis. MRI cervical spine also helps in excluding other cause like cervical cord diseases, which could mimic this disease.

Causes
As of now, there is no established cause for ALS. However in about 10 per cent of patients, they have identified at defect on Chromosome 21, which codes for SOD1 enzyme. This enzyme is a powerful antioxidant, which protects the cells from damage by a chemical called superoxide. Hence, the loss of SOD1 function leads to accumulation of free radical in the cells leading to its death. This defect could possibly explain the incidence of this disease in 10 per cent of all the cases of ALS. Other chemical like excess Glutamate has been suggested but not confirmed. There have been an increased incidence in army personnel, possibly because of exposure to chemicals, electromagnetic field exposure, physical trauma, etc. However, in more than 90 per cent of the patients of ALS, we do not know the cause and in the remaining 10 per cent, it is still speculated, says Dr Ashok who, on an average, sees one to two cases per month.

Treatment
Unfortunately, there is no cure for ALS. However, detection in the early stages is beneficial. In India, there is hardly any awareness and people have a tendency to delay seeking professional help, ignoring symptoms for a period of six months, and till it becomes too late, says Dr Thakre. He advises that individuals need to seek professional help, conduct thorough tests and get a clinical diagnosis. Besides counseling, physiotherapy is part of the treatment to maintain the tone of the muscles, to avoid spasticity and rigidity.

A drug called Riluzole, has some doubtful role in slowing the progression of the disease. Other measures that can be taken are to treat are occupational rehabilitation, alternate pathway for feeding when required, and to assist ventilation as and when required, says Dr Ashok.

ALS is often called Lou Gehrig's disease, after the famous American baseball player who was diagnosed with it. He was diagnosed with the disease in 1939, and he passed away in 1941.

B-Town ice bucket challenge
Here's some of the many Bollywood celebs who took the ice bucket challenge.