ALS is a degenerative and debilitating condition

Stephen Hawking was a rare case in which the disease progressed slowly and he survived for a longer period

New Delhi, March 17 , 2018 : Amyotrophic lateral sclerosis incurable, neurodegenerative disorder that causes muscle weakness, disability, and eventually death.  ALS has an annual incidence of one to three cases per 100,000 people that is believed to be the same worldwide. There appears to be no ethnic or racial predisposition to ALS.

The median survival from the time of diagnosis is three to five years. However, about ten percent of ALS patients can live 10 years or more.

Survival beyond 20 years is possible but rare and in part depends on treatment decisions made by patients and their families. Stephen Hawking was a rare case and survived over 50 years since he was diagnosed with ALS at the young age of 21.

ALS is a rare condition that progressively damages motor neurons in the brain and spinal cord, stopping them from sending messages to muscles. The muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost.

Speaking about this, Padma Shri Awardee Dr K K Aggarwal, President Heart Care Foundation of India (HCFI) and Immediate Past National President Indian Medical Association (IMA), said, “Each muscle in the brain is controlled by motor neurons in the frontal lobe. These are located in three areas of the nervous system – the frontal lobe, the lower brain, and the spine. Motor neurons in the brain are called upper motor neurons and the ones in the spine are called lower motor neurons. ALS leads to the weakening of either of these or both together. There are two ways in which patients suffering from ALS can possibly die: respiratory failure and deterioration of swallowing muscles which can lead to dehydration and malnutrition. Hawking’s survival was probably due to a slower progression of the condition, which also indicates why one should not lose hope.”

Early symptoms of ALS include muscle weakness or stiffness. As the condition progresses, all muscles under voluntary control are affected, and individuals lose their strength and the ability to speak, eat, move, and even breathe.

Adding further, Dr Aggarwal, who is also the Group Editor of IJCP, said, “There is no cure for ALS as on date. However, the primary aim is to control symptoms, prevent unnecessary complications, and make living with the disease easier. Medications may be prescribed to manage symptoms such as muscle cramps, stiffness, excess saliva and phlegm; help individuals with pain, depression, sleep disturbances, and constipation. Speech therapy, and nutritional and breathing support is also provided.”

Research suggests that increased consumption of foods containing colorful carotenoids, particularly beta-carotene and lutein, may prevent or delay the onset of ALS. Vitamin C or carotenoids are also antioxidants and studies have shown that those who have a high intake of antioxidants, have a reduced ALS risk.

 Facts

1. The clinical hallmark of amyotrophic lateral sclerosis is the combination of upper motor neuron and lower motor neuron signs and symptoms.
2. Upper motor neuron findings of weakness, hyperreflexia, and spasticity result from degeneration of frontal motor neurons.
3. The lower motor neuron findings of weakness, atrophy or amyotrophy, and fasciculations are a direct consequence of degeneration of lower motor neurons in the brainstem and spinal cord.
4. Spectrum of motor neuron disease includes progressive muscular atrophy, primary lateral sclerosis, and progressive bulbar palsy, all of which may be variants of ALS, or represent different patterns of evolution to ALS.
5. Asymmetric limb weakness is the most common presentation of ALS (80 percent).
6. Bulbar onset, usually manifested as dysarthria or dysphagia, is the next most common pattern (20 percent).
7. Cognitive impairment, typically related to frontotemporal executive dysfunction, may precede or follow the onset of upper motor neuron and/or lower motor neuron dysfunction in patients with ALS.
8. Frontotemporal dementia may be associated with ALS in 15 to 50 percent of cases.
9. ALS is a relentlessly progressive disorder with a clinical course that is nearly always linear.
10. He progressive course of ALS eventually produces one or both of the life-threatening aspects of the disease, neuromuscular respiratory failure and dysphagia.