What is Motor Neuron Disease?

Motor neuron diseases are a group of neurodegenerative disorders that selectively affect the cells which control all the voluntary muscles of the body. The voluntary muscles are responsible for performing movements under one’s will and thus motor neuron diseases affect one’s ability to perform voluntary movements.

How does the condition progress?

The progress of the condition varies from one patient to another, people with MND will find some or all of these activities increasingly problematic.  Eventually they may become impossible.

What are the symptoms of Motor neuron disease?

The onset of symptoms begin gradually over weeks and months, sometimes starting on one side of the body and then spreading. Generally, the first things people notice are:

• Weakness in the hands and grip, which can cause difficulty in picking up or holding objects
• Slurred speech
• Weakness in the legs, and a tendency to trip
• Weakness of the shoulder, making lifting the arm difficult
• Cramps and muscles twitching
• Foot-drop caused by weak ankle muscles
• Dragging of the leg

As the disease progresses, people with MND can have little or no movement and can have trouble talking, breathing and swallowing.  However, symptoms can be managed to help achieve the best possible quality of life.

What are the various types of Motor neuron disease?

Motor neuron disease is an umbrella term and under which there are several types of diseases.

Amyotrophic lateral sclerosis or Lou Gehrig’s disease:  The most common type affecting the muscles of the arms, legs, mouth, and respiratory system.

Progressive bulbar palsy (PBP):  People with ALS often suffer from progressive bulbar palsy as well.  Choking spells, difficulty speaking, eating, and swallowing are the predominant symptoms of this condition.

Progressive muscular atrophy (PMA):  This type of MND can lead to muscle wasting, mainly in the arms, legs and mouth.  It can be a variation of amyotrophic lateral sclerosis.

Primary lateral sclerosis (PLS):  It is a rare form of motor neuron disease that progresses slower than ALS.  Though not life-threatening, it can severely affect the quality of life.  When children get affected by this condition, it is called juvenile primary lateral sclerosis.

Spinal muscular atrophy (SMA):  It is an inherited form of MND that affects children due to an abnormality in the gene known as SMA1.  Trunks, legs and arms are the areas that are primarily being affected.    

Is motor neuron disease inherited?

In majority of the cases, there is no clear-cut family history of the disease, though a variety of genetic and environmental factors may be involved, which could be subjective. 

Which functions are not affected by MND?

The senses are usually not impaired by motor neuron disease, so patients can still see, hear, taste smell and feel sensations.  Bladder and bowels are generally not affected by MND.  As the affected person gets dehydrated and less mobile, constipation can also be a problem. MND may not affect one’s libido, but sexual expression may be more difficult as mobility and movements are restricted.

What is Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder characterized by progressive loss of anterior horn cells of the spinal cord.  It affects the nerve cells and causes disability. 

What are the early signs of Amyotrophic Lateral Sclerosis?

Early signs and symptoms of amyotrophic lateral sclerosis include:

• Difficulty walking or doing your normal daily activities
• Tripping and falling
• Weakness in leg, feet or ankles
• Hand weakness or clumsiness
• Slurred speech or trouble swallowing
• Muscle cramps and twitching in arms, shoulders and tongue
• Difficulty holding the head up or keeping good posture

ALS generally starts in the hands and feet and then radiates to other parts of body.  Chewing, swallowing, speaking and breathing are eventually impacted. 

What is the treatment for motor neuron disease/amyotrophic lateral sclerosis at Plexus?

The treatment at Plexus helps in slowing down the progression of the disease. Our aim is to stop the progression of the disease by using a unique combination of therapies that is custom made for every patient based on the problems.

Physical Therapy

Individuals with ALS/MND have severe weakness in their muscles, due to extensive wasting and reduced mobility, joint structures become weak. They have problem in walking, maintaining balance and in performing free movements of the extremities. Many patients complain of severe pain in their shoulders and legs. Physiotherapy helps to alleviate pain and improve flexibility. Keeping in mind the principles of fatigue management, our team of physiotherapists design an individual exercise protocol in order to strengthen their muscles and prevent further loss of strength.

Occupational Therapy

Occupational Therapists evaluate the patient’s ability to perform daily life tasks like eating, bathing, dressing, managing mobility in the community and ability to perform work –related activities. Wasting of the hand muscles is one of the most disabling features of ALS which affect an individual’s independence. Due to thumb drop and loss of grip, mundane daily tasks gradually become impossible. Functional splinting is an important area in the treatment of ALS where specific types of splints are customized to suit the patient’s needs. Treatment mainly focuses on training of ADL tasks which pose a challenge for the patient. At times the use of functional splints makes it easier to perform daily tasks. Activities to maintain strength, improve endurance, adaptive ways to manage fatigue, training of hand functions and fostering independence in daily life tasks and community mobility are the major goals of occupational therapy intervention at Plexus.

Speech Therapy

Speech and swallowing difficulties are common in individuals with ALS and these problems are seen to progress over time. Together with the individual, the Speech and Language Pathologist determines the best strategies for improved speech and swallowing. Specific oro-motor exercises to improve the strength of the oral structures that aid in speech, help to improve the clarity of speech. Dysphagia Management is a very important aspect of speech therapy as it helps to improve swallowing and prevent choking in patients with ALS.

Motor Neuron Disease…there is hope!

Mr. K, a 42 year old software engineer was diagnosed with this devastating neurodegenerative disorder in March, 2017. The first sign he observed was not being able to clip his finger nails using a nail clipper which was followed by slow onset of pain in the right shoulder. As days melted into months, his disability went on increasing and mundane daily activities like dressing and bathing became difficult. He was unable to lift his right shoulder, there was severe weakness and pain on movement. He also noticed that he was unable to rotate his forearm, meaning that he was not able to place his hand with the palm facing the roof. The weakness went on progressing to the other extremities as well and started affecting his walk. He went for multiple consultations and underwent various neurological examinations where he was diagnosed with Amyotrophic Lateral Sclerosis, a motor neuron disease that causes the death of neurons controlling the voluntary muscles. He came to Plexus in March, 2019 with difficulty in walking and severe pain and weakness in his upper limbs. His main complaint was not being able to use his hands for his daily activities. A thorough examination was carried out which revealed that the extent of the problem was much more than it appeared to be. Being a neurodegenerative condition, the disease had progressed to a great extent. There was not only weakness but also severe tightness in all his joints. He was inducted into the comprehensive neuro-rehabilitation program with the goal of stopping or slowing down the disease progression. He underwent a custom made treatment program comprising of physiotherapy that focused on relieving his joint pain and stiffness, improving the strength of his lower limbs and improve walking; occupational therapy that focused on the hand functions to help improve his ability to perform his daily life activities and speech therapy to strengthen his oral structures and improve his breathing and swallowing as a preventive measure. He was also provided with a customized splint that focused on his thumb drop and helped in attaining an active grip. After a period 45 days of undergoing treatment, it was seen that he was able to perform all his daily activities independently. He was able to grip objects of varied sizes including the fine objects which were impossible to grip when he enrolled into the treatment; pain and tightness in the arms disappeared and his walking improved. Being a progressive neurodegenerative condition, it was a herculean task to tackle the disease at this stage but results were overwhelming for both the patient and the treating team.