According to the United Nations, the number of individuals in the world above age 60 is expected to increase rapidly. This ageing pattern is especially significant in developing countries, where the proportion of older individuals will increase from about 9% in 2015 to 16% by 2040. The number of individuals diagnosed with chronic disease has grown due to this trend. For instance, one study found that the number of individuals with Parkinson’s disease will double between 2005 and 2030, and the weight of disease will shift from developed nations to developing nations.
Today, Health Plus is placing the spotlight on a disease not well known but is considered the most common adult-onset motor neuron disease, Amyotrophic Lateral Sclerosis (ALS) and we consult with Dr. Avidesh Panday, Consultant at EWMSC in Neurology and Neurophysiology to share from his expertise and insights.
What is ALS?
Our neurological system is divided into the central nervous system and the peripheral nervous system. The central nervous system comprises the brain and spinal cord whereas the peripheral nervous system comprises the nerve and muscle. These components result in the motor (movement/strength), sensory (sensation, balance) as well coordination functions.
ALS comes from a large group of disorders whereby there is progressive loss of function in the motor components in both the central and peripheral nervous system.
It comes from a larger group of disorders known as Motor Neurone Disorder where certain persons may get motor loss arising from the central nervous system, motor loss in the peripheral nervous system or both in the case of ALS.
Who gets ALS? How common is this disease pattern?
The worldwide prevalence of ALS is 2-7 patients/100,000. Its cause is unknown although there are multiple factors including genetic, smoking and certain environmental factors. ALS can occur among many age ranges. Locally we have seen patients in their late 20’s and in their early 70’s. It’s really diverse in its age range.
Why is this important?
Unfortunately, this condition is classified as a neurodegenerative condition. That means, it progressively gets worse with time and there is no cure or meaningful medication to slow its clinical course. Loss of motor function (strength) results in physical weakness which can reduce hand grip strength and limb strength among others but more importantly bodily function such as phonation of speech, difficulty in swallowing and with time difficulties in breathing. This not only impacts the quality of life but threatens preservation of life ultimately.
What are the symptoms one should be familiar with?
The symptoms are quite diverse but the predominant common denominator here is loss of muscle strength as well as weight and muscle mass loss. Some patients complain of «muscle jumps/twitches». While we should be familiar with these symptoms, the presence of sensory symptoms such as numbness and paraesthesia «tingling or as we know in local parlance – «jejuni» should serve as red flags, that this is not ALS.
How is ALS diagnosed?
Most medical conditions can be diagnosed with a specific test. For example, a blood test can definitively tell you if there is kidney disease or an echocardiogram can help to determine if there is heart disease. ALS is a diagnosis of exclusion where you have a suspicion of the illness based on an examination and history and then use imaging techniques such as MRI scans of the Brain and Spine as well as Neurophysiology Tests such as an Electromyogram/Nerve Conduction Studies to help exclude any other Neurological illnesses. In some instances, blood tests and a spinal tap may be needed to aid in exclusion of other diagnoses.
Usually, this diagnosis takes some time to establish as several investigations are required to ensure there is no other Neurological Diagnosis that will account for the patient’s symptoms. In early and late presentation, it may be challenging to diagnose and on some occasions a person may be diagnosed with another illness but with time their diagnosis may be revised to ALS and vice versa. In some instances, this diagnosis may require two independent Neurologists.
Is there a cure?
How is ALS treated?
As mentioned above, there is no cure. The treatment really is supportive. In the early stages of disease, the patients may require physical and occupational therapy to maintain strength, dexterity and limb function. As the disease progresses, multiple physicians are involved such as Pulmonologists to assist with respiratory function, Gastroenterologists/Speech and Swallow Therapists to assist with swallowing and digestive function. It is important to recognise that not only are patients affected, rather this involves the entire family, both in terms of physical and emotional support. In some instances, the Psychologist, Psychiatrist and Medical Social Worker may be necessary.
As the illness progresses families may have tough decisions with regards to end-of-life care. It is advisable that patients enlist the services of Palliative Care Physicians, early on the course of their illness to assist with maintain the best quality of life for the patient and relatives for as long as possible.
A multidisciplinary approach is needed
It is my desire to conceptualize a multi-disciplinary clinic not only for ALS but for many Neurological Illnesses where patients can access Neurology/Physical Therapy and Rehabilitation/Pulmonology/Mental Health Services all in one clinic visit.
Dr. Avidesh Panday is a Consultant at the Eric Williams Medical Sciences Complex in Neurology and Neurophysiology which includes Neuromuscular Conditions such as ALS as well as other illnesses such as Epilepsy/Parkinson’s Disease and Multiple Sclerosis.
LINK ORIGINAL: The Trinidad Guardian