Grey’s Anatomy star Eric Dane, much loved for embodying the character nicknamed ‘Mc Steamy,’ has died at 53 within a year of being diagnosed with ALS (amyotrophic lateral sclerosis), a progressive neuro-degenerative disease that affects nerve cells in the brain and spinal cord, paralysing the muscles and making it harder for people to move, talk, eat and eventually breathe independently.
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Although he became an advocate for its early diagnosis and management, he himself had mistaken a weakness in his right hand as a numbness from frequent texting. “Although it is widely recognised due to public awareness and high-profile cases, the overall incidence remains very low. In India, ALS is considered a very rare disease. It typically affects individuals in their 40s and 50s and is seen slightly more commonly in males, though it can occur in both genders. Only about 5% of cases are familial; the majority are sporadic,” says Dr Arjun Shah, consultant neurologist at Saifee Hospital, Mumbai.
Dr P N Renjen, senior consultant, neurology, Indraprastha Apollo Hospitals, Delhi, has not seen many patients either, maybe one or two a year. “I remember this 60-year-old man complaining of a routine nerve problem in his palm and wrist. Then he gradually lost functionality of his right arm, couldn’t even swim and had quick muscle wasting. Early diagnosis helps us administer drugs to slow down the progression of this disease,” he says. However, there is limited awareness about the disease outside large urban centres. “Access to neurologists, advanced diagnostic tools, ventilatory support and long-term rehabilitation services may not be uniformly available to the even few who need it,” he adds.
How to diagnose ALS?
Early diagnosis is important for better management of the condition even though there is no definitive cure. “ALS remains predominantly a clinical diagnosis, supported by tests like nerve conduction studies and EMG (electromyography). Identifying the condition early helps avoid misdiagnosis, especially since some rare but treatable disorders may mimic ALS. Early detection allows timely initiation of supportive medications, anticipatory guidance, and management of potential complications,” says Dr Shah.
Why does the disease progress rapidly if not diagnosed correctly?
The disease usually begins subtly. A person may first notice weakness in one hand, difficulty gripping objects, frequent tripping or slurred speech. Muscle cramps and twitching are also common early signs. “Because these symptoms can seem mild or unrelated, they are sometimes ignored or mistaken for fatigue, vitamin deficiencies or other nerve problems. This can delay diagnosis,” says Dr Renjen.
As ALS progresses, the weakness spreads to other parts of the body. Muscles in the arms and legs lose strength, making it hard to walk, climb stairs or lift everyday items. When the muscles of the throat and tongue are affected, speech becomes unclear and swallowing becomes difficult. “This increases the risk of choking and weight loss. Importantly, ALS does not usually affect the senses. Vision, hearing, touch and awareness remain intact in most patients, which can make the experience emotionally distressing, as the person is fully aware of the physical decline,” says Dr Renjen.
In advanced stages, the breathing muscles become weak. “That’s why respiratory failure is the most common cause of death in ALS,” explains Dr Renjen.
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Who is at risk?
ALS usually affects people between the ages of 45 and 70, though it can happen earlier in cases of juvenile motor neuron disease. “In most cases, there is no clear cause. Only a small percentage of people inherit it from a parent. The condition is rare and in India, it affects five in 100,000 people. ALS is mostly seen in men, very rarely in women,” says Dr Renjen.
What about disease management?
While there is no cure anywhere in the world, the disease can be managed with supportive care.
Can ALS be treated?
“Medicines like Riluzole and Edaravone can slow disease progression and extend survival. Physical therapy can help keep the muscles working for longer, and speech therapy becomes useful when talking or swallowing becomes difficult. As breathing muscles weaken, special equipment can help ease breathing. Wheelchairs, communication devices and feeding tubes can help maintain independence,” says Dr Renjen.
“Multidisciplinary management including neurologists, physiotherapists and supportive therapies helps maintain quality of life. Emotional support, realistic counselling and structured care planning play a crucial role in managing the disease effectively,” adds Dr Shah.
© The Indian Express Pvt Ltd
