What is ALS (Amyotrophic Lateral Sclerosis)? - Causes, Symptoms and Treatment

What is ALS (Amyotrophic Lateral Sclerosis)? - Causes, Symptoms and Treatment

ALS or Lou Gehrig disease is a disease that causes progressive degeneration of the motor neurons. These are the nerve cells that affect the brain’s ability to move the muscles. They control all voluntary and some involuntary movements in the body. The exact cause of ALS is unknown. However, recent studies indicate that it could be caused by a genetic mutation. Exposure to certain toxic chemicals has also been linked to ALS. The symptoms of ALS start out mild, with patients experiencing muscle spasms, weakness and slightly slurred speech. These symptoms worsen with time, causing all sorts of problems like trouble walking, eating, swallowing, and speaking. At the terminal stage, patients often have difficulty breathing on their own. ALS cannot be cured, but patients are equipped with strategies to help delay the on-set of serious symptoms once they are diagnosed. Being diagnosed with ALS can be a gut-wrenching discovery for the patient and their loved ones. However, with the right support at the right time, the patient can delay their symptoms and live out their days in comfort.

Amyotrophic lateral sclerosis (ALS) is a motor neuron disease, i.e. it affects the neurons involved in physical movements. ALS is often also known as Lou Gehrig disease, after the famous US baseball player who died from it. It causes progressive degeneration of the motor nerve cells in the spinal cord and the brain. When motor neurons die, the brain loses its ability to initiate and control muscles. ALS doesn’t affect sensory perception (sight, touch, smell, hearing and taste) or cognition (understanding and critical thinking). The exact cause of the disease is still unknown. ALS symptoms usually begin with muscle twitching or spasms and weakness in the arms and legs. It progresses to total loss of muscle control, leaving the patient unable to move, speak, eat or even breathe. ALS is fatal. Currently, we have no cure for this condition.

The symptoms of ALS start out mild and get progressively worse with time. The speed at which the degeneration of motor neurons occurs varies from person to person. The first symptoms of ALS are usually muscle weakness and stiffness, typically in the arms and legs. The patient’s speech usually becomes slurred and they also have trouble swallowing. It may become more difficult for the patient to do simple everyday tasks like writing, buttoning their shirt or picking up objects. They might find it difficult to do co-ordinated movements like walking. ALS patients are very prone to falls.

As the disease progresses, patients will find it difficult to do any movements independently. They will not be able to get out of bed on their own, or even feed themselves. Facial movements also become difficult. They may not even be able to hold up their own head. Eventually, they will even lose the ability to speak, swallow their own saliva and find themselves unable to breathe independently.

Some ALS patients also experience “emotional lability”. This refers to episodes of rapid, exaggerated changes in mood. They may find themselves crying or laughing uncontrollably or having a temper tantrum, quite unlike their original character.

The cause of ALS is still a mystery to the scientific community. However, recent studies have pointed to genetics as a major factor. Researchers from the Neuromuscular Diseases Group and the Dementia Neurobiology Group of the Sant Pau Research Institute, led by Dr. Ricard Rojas-Garcia, have identified a mutation in the ARPP21 gene that could be the cause of ALS. They identified this common genetic mutation in 10 ALS patients from 7 unrelated families. Their investigation was initiated after realising that the La Rioja region of Spain had an unusually high number of ALS patients. Although their results are localised to this specific geographic region, the hope is that this discovery can help scientists working in other parts of the world. This could open help diagnose ALS more precisely and lead to research on personalised therapist help patients.

Exposure to certain toxic chemicals like lead and mercury have also been linked to ALS. Studies are still inconclusive.

Being diagnosed with a fatal illness that has no cure takes a huge toll on the patient’s mental health. Mental health and the lack of hope advances the disease more quickly. It’s really a vicious cycle. Many ALS patients get diagnosed with depression soon after their ALS diagnosis. Patients become highly dependent on their caretaker and medical staff. Most patients with ALS survive 3 to 5 years after their diagnosis. About 10% of patients survive up to 10 years after their diagnosis.

There is no single conclusive test to diagnose ALS. The neurologist may suspect ALS on the basis of the patient’s physical exam and their own report of their symptoms. They will then confirm their diagnosis by ruling out other conditions like peripheral neuropathy and myopathy. This is done by conducting a series of tests. This would include an electromyograph, which detects electrical signals in the muscles. A nerve conduction study may also be performed. The doctor may also request an MRI scan to rule out a spinal cord tumour or a herniated disk which may be causing the patient’s symptoms. Blood and urine tests may also be needed to rule out diseases like HIV, Lyme disease, multiple sclerosis and West Nile virus, which cause similar symptoms to early stage ALS.

ALS is confirmed if there are symptoms of degeneration in both upper and lower motor neurons.

There is no cure for ALS at present. Neurologists can only prescribe treatments to alleviate symptoms and slow the progression of the disease. A multi-prong approach, including medication, physical therapy, occupational therapy, speech therapy and nutritional strategies, is typically suggested here. Medications such as Riluzole, Edaravone, Sodium phenylbutyrate and Toferson can help slow the progression of the disease. The patient may also be prescribed medication for symptomatic relief from muscle cramps, stiffness, pain and the mental health challenges associated with ALS.

The physical therapy for ALS patients is aimed at helping them perform their day-to-day activities in an independent and safe manner. Physical therapy and rehabilitation can help the patient keep their muscles strong and retain as much of their fine-motor skills (delicate tasks with their hands, such as writing or picking up small objects) as possible.

Occupational therapy teaches the patient techniques to move around independently. Patients will be taught how to correctly and safely use assistive devices like walkers, braces and wheelchairs. Speech therapy addresses the patient’s difficulty with speech and swallowing. The patient can be taught how to retain their ability to speak for as long as possible and non-verbal communication techniques for when they lose their speech. Patients with ALS also have difficulty eating and swallowing. Nutritional strategies can help create a proper diet plan to ensure patients get adequate nutrients through foods that are easy to swallow. At the terminal stage, ALS patients require a feeding tube and a mechanical respirator.

Finding out you have ALS is gut-wrenching. It is a difficult time for the person who is diagnosed, as well as their loved ones. Below are some strategies to cope with this painful situation:

Take the time to grieve – This is painful news to hear. Processing it now is important. It doesn’t help to live in denial. Taking the time to grieve and accept what is about to happen to you can help you approach the difficulties to come with a good attitude. Remember that you may still have many good years left. Processing your grief now can help you fully enjoy the years you have left.

Reach out for help – There are many ALS support groups that can help answer your questions and offer community support. There are groups for the ALS patients as well as for their caregivers and close family members. We often forget that the caregivers experience as much (if not more) psychological pain as the patient. Having a supportive circle of friends and family is very important, so try not to isolate yourself and suffer alone. You are loved no matter what, and help is always available in some form or another. Try to stay in touch with friends and family and participate in as many community activities as you feel comfortable with.

Prepare for the inevitable – Once you have been diagnosed, it is certain that the degeneration of the nerves will worsen with time. You can begin preparing for this by making your home wheel-chair friendly, and buying things like easy-to-hold cutlery to for your new needs. You can get assistive devices to prepare for the inevitable loss of mobility.

Make decisions about your future medical care – Plan for the future so you can stay in control of the condition. Decide who you would like making medical decisions for you when you are not able to. You can also choose what life-extending procedures you want to consent to, with the help of your neurologist. You may also decide if you want to be part of any clinical trials for ALS, so you can make your contribution to finding a cure for this disease.

Research financial aid – ALS treatment can get expensive as the disease progresses. This may be a good time to get your financial affairs in order and apply for financial assistance if required.

Care for the Care givers – Care givers are barely cared for. Their suffering is always deemed lesser than the patient. But watching a loved one suffer is so draining, and care givers need time off to recuperate. Try to get a friend or a relative to come relieve your primary caregiver once in a while. They need rest to come back and be your rock again.

At Kauvery Hospital, with branches in Chennai, Hosur, Salem, Tirunelveli, and Trichy, we provide expert care for individuals dealing with ALS. Our multi-disciplinary team is dedicated to improving the lives of our patients through comprehensive treatment strategies and unrivaled support services. Trust in Kauvery Hospital, where we blend expertise with empathy for the best patient care.

What is ALS?
ALS (Amyotrophic Lateral Sclerosis) is a progressive neurological disease that affects motor neurons, leading to muscle weakness and loss of movement.

What are the early symptoms of ALS?
Early signs include muscle weakness, twitching, stiffness, slurred speech, and difficulty swallowing or walking.

What causes ALS?
The exact cause is unknown, but genetic mutations and exposure to toxic chemicals are linked to ALS development.

How is ALS diagnosed?
Diagnosis involves a physical exam, electromyography (EMG), nerve conduction studies, MRI scans, and blood tests to rule out other conditions.

Is there a cure for ALS?
Currently, there is no cure for ALS, but treatments like medication, therapy, and assistive devices can help manage symptoms and improve quality of life.

How long do ALS patients typically live?
Most ALS patients survive 3 to 5 years after diagnosis, but about 10% live for 10 years or longer.

What treatment options are available for ALS?
Treatment includes medications (Riluzole, Edaravone), physical therapy, speech therapy, occupational therapy, and nutritional support.

Where can ALS patients receive specialized care?
Kauvery Hospital provides expert multidisciplinary care for ALS patients in Chennai, Hosur, Salem, Tirunelveli, and Trichy.

Kauvery Hospital is globally known for its multidisciplinary services at all its Centers of Excellence, and for its comprehensive, Avant-Grade technology, especially in diagnostics and remedial care in heart diseases, transplantation, vascular and neurosciences medicine. Located in the heart of Trichy (Tennur, Royal Road and Alexandria Road (Cantonment), Chennai (Alwarpet & Vadapalani), Hosur, Salem, Tirunelveli and Bengaluru, the hospital also renders adult and pediatric trauma care.

Chennai Alwarpet – 044 4000 6000 •  Chennai Vadapalani – 044 4000 6000 • Trichy – Cantonment – 0431 4077777 • Trichy – Heartcity – 0431 4003500 • Trichy – Tennur – 0431 4022555 • Hosur – 04344 272727 • Salem – 0427 2677777 • Tirunelveli – 0462 4006000 • Bengaluru – 080 6801 6801