Types, Causes, Symptoms and Treatment of Motor Neuron Disease
by The Welthi Bureau, July 23rd, 2021
Image used for representational purpose only
With Leeds Rhinos great Rob Burrow revelation, focus has once again been shifted to life-shortening Motor Neurone Disease (MND). "It's a group of diseases that affect the nerves (motor neurones) in the brain and spinal cord that tell your muscles what to do. The disease eventually blocks messages from the brain reaching the muscles, which caused them to weaken, stiffen and waste away. It affects how you complete basic everyday tasks such as walk, talk, eat, drink and breathe, and though millions is going into developing a treatment for it, there is currently no cure and it proves life-shortening, with MND affecting one in every 300 people and most likely to affect those aged over 50," says Dr. Rajnish Kumar, Neurologist, Paras Hospital, Gurugram.
Types of MND
Though there are several different variants of MND, they all share the fundamental principle of motor neuron degeneration. They vary in their range and degree of symptoms, as well as disease progression. Amyotrophic lateral sclerosis (ALS) ALS or Lou Gehrig's disease is the most common form of the disease. Initially, the patient has symptoms like limb muscle weakness, stiffness, and spasms. Individuals increasingly trip while walking, or drop things. As the disease progresses, other muscles deteriorate, such as those for speech, swallowing, and breathing. Patients also experience unstable and rapidly changing emotions.
Progressive bulbar palsy (PBP)
PBP affects 4.1% of individuals with MND. This variant affects the motor neurons in the bulbar region of the brain, leading to weakness of the muscles of the face and neck. Symptoms include difficulties in chewing, swallowing, speech and neck extension; limbs are usually affected later as the disease progresses. This rapidly progressing variant of MND leads to a shorter life expectancy of 6 months to 3 years.
Primary lateral sclerosis (PLS)
This rare disease leads to weakness, stiffness and spasticity in the lower limbs, which progresses to the trunk, arms, hands, tongue and jaw. At an advanced stage of the disease, some individuals develop speech impairments and difficulty with breathing or swallowing. Those with adult PLS do not have a reduced life expectancy as the disease is not fatal, but it may be quite debilitating and reduce their quality of life
What causes MND?
There is no identifiable cause for MND. 10% of cases are hereditary, which means individuals have inherited a faulty copy of a gene. Most cases of MND are sporadic, meaning they occur randomly without a known cause. An ongoing research has suggested several causes which include:
Intense physical activity
Loss of growth factors to developing motor neurons
Oxidative injury to neurons
Excessive excitability of neurons
Early symptoms may be mild and include:
• Stumbling due to weakness of the leg muscles
• Difficulty holding objects caused by weakness of the hand muscles
• Slurring of speech or swallowing difficulties due to weakness of the tongue and throat muscles
• Cramps and muscle twitching
As the disease progresses symptoms may include:
• Breathing difficulties from decreased lung capacity caused by muscle weakness
• Fatigue caused by muscle exhaustion, decreased lung capacity, metabolic changes, weight loss and reduced food intake
• Insomnia caused by discomfort, pain from stiff joints and muscles, excessive saliva, dry mouth or breathing problems
• Mild changes in cognitive skills and processes and/or behavioural change
• Fronto-temporal cognitive changes (a type of dementia), which is prominent in 5-10% of MND cases
• Excessive laughing or crying due to damage to the upper motor neurones
• Some pain or discomfort
There is no cure for MND, but treatment may slow progress
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