Motor Neuron Disease

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Motor neuron diseases are a group of conditions that cause the nerves in the spine and
brain to progressively lose function. They are a rare but serious and incurable form of
progressive neurodegeneration.

Motor neurons are nerve cells that send electrical output signals to the muscles, affecting
the muscles’ ability to function.

Motor neuron disease (MND) can

appear at any age, but most patients
are over 40 years old at diagnosis. It
affects men more than women.

Key points about motor neuron

diseases.
Motor neuron diseases (MND) are a
group of conditions that affect the nerve
cells that send muscles to the brain.
There is a progressive weakening of all
the muscles in the body, which
eventually affects ability to breathe.
Genetic, viral, and environmental issues
may play a role in causing MND.
There is no cure, but supportive treatment can improve the quality of life.
Life expectancy after diagnosis ranges from 3 years to longer than 10 years.

Classification:
Motor neuron diseases affect either upper motor neurons (UMN) or lower motor neurons
(LMN), or both:

Type UMN degeneration LMN degeneration

Amyotrophic lateral sclerosis (ALS) Yes Yes
Hereditary spastic paraplegia (HSP) Yes No
Primary lateral sclerosis (PLS) Yes No
Progressive muscular atrophy (PMA) No Yes
Progressive bulbar palsy (PBP) No Yes, bulbar
region
Pseudobulbar palsy Yes,bulbar region No

ALS, or Lou Gehrig’s disease, is the most common type, affecting muscles of the arms,
legs, mouth, and respiratory system. Mean survival time is 3 to 5 years, but some people
live 10 years or more beyond diagnosis with supportive care.

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Progressive bulbar palsy (PBP) involves the brain stem. People with ALS often have PBP
too. The condition causes frequent choking spells, difficulty speaking, eating, and
swallowing.

Progressive muscular atrophy (PMA) slowly but

progressively causes muscle wasting, especially in
the arms, legs, and mouth. It may be a variation of
ALS.

Primary lateral sclerosis (PLS) is a rare form of MND

that advances more slowly than ALS. It is not fatal, but
it can affect the quality of life. In children, it is known
as juvenile primary lateral sclerosis.

Spinal muscular atrophy (SMA) is an inherited MND that affects children. There are three
types, all caused by an abnormal gene known as SMA1. It tends to affect the trunk, legs,
and arms. Long-term outlook varies according to type.

Symptoms:
MND can be divided into three stages, early, middle, and advanced.

Early stage signs and symptoms

Symptoms develop slowly and can be confused with symptoms of some other unrelated
neurological conditions.

Early symptoms depend upon which body system is affected first. Typical symptoms
begin in one of three areas:

the arms and legs, the mouth (bulbar), or the respiratory system.

They include:

a weakening grip, making it hard to pick up and hold things

fatigue
muscle pains, cramps, and twitches
slurred and sometimes garbled speech
weakness in the arms and legs
increased clumsiness and stumbling
difficulty swallowing
trouble breathing or shortness of breath
Middle stage signs and symptoms

[Motor neuron mobility]

Motor neuron diseases can leave those with the disease severely restricted in mobility.
As the condition progresses, symptoms become more severe.

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Muscle pain and weakness increase, and spasms and twinges worsen.
Limbs become progressively weaker.
Limb muscles start to shrink.
Movement in affected limbs becomes more difficult.
Limb muscles may become abnormally stiff.
Joint pain grows.
Eating, drinking, and swallowing become harder.
Drooling occurs, due to problems controlling saliva.
Yawning occurs, sometimes in uncontrollable bouts.
Jaw pain may result from excessive yawning.
Speech problems worsen, as muscles in the throat and mouth become weaker.
The person may show changes in personality and emotional state, with bouts of
uncontrollable crying or laughing.

Previously, it was believed MND did not significantly affect brain function or memory, but
studies have now shown that up to 50 percent of people with ALS have some type of brain
function involvement.

This includes difficulties with memory, planning, language, behavior, and spatial
relationships. Up to 15 percent of people with ALS have a form of dementia known as
frontotemporal dementia (FTD).

Breathing problems may occur as the diaphragm, the main breathing muscle, deteriorates.
There may be a shortage of breath, even when sleeping or resting. Ultimately, breathing
assistance will be necessary.

Secondary symptoms include insomnia, anxiety, and depression.

Advanced stage signs and symptoms

Eventually, the patient will be unable to move, eat, or breathe without assistance. Without
supportive care, an individual will pass away. Despite the best of care currently available,
complications of the respiratory system are the most common causes of death.
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Causes:

Motor neurons send signals from the brain to the muscles and bones, and this makes the
muscles move. They are involved in both conscious movements and automatic
movements, such as swallowing and breathing.

Some MNDs are inherited while others happen randomly. The exact causes are unclear,
but the National Institute of Neurological Diseases and Stroke (NINDS) notes that genetic,
toxic, viral, and other environmental factors likely play a role.

Risk factors:

Heredity: In the United States (U.S.), around 1 in every 10 cases of ALS is inherited. SMA
is also known to be an inherited condition.

Age: After the age of 40 years, the risk rises significantly, although it is still very small. ALS
is most likely to appear between the ages of 55 and 75 years.

Sex: Men are more likely to develop an MND.

Diagnosis:

In the early stage, MND can be hard to diagnose, because the signs and symptoms are
common to other conditions, such as multiple sclerosis (MS), an inflamed nerve, or
Parkinson’s disease.

A primary care physician will normally refer the patient to a neurologist, a doctor specialized
in the diagnosis and treatment of diseases and conditions of the nervous system.

The neurologist will start with a complete history and physical exam of the neurologic
system.

Other tests may be helpful.

Blood and urine tests: These analyses can rule out other conditions and detect any rise in
creatinine kinase. This is produced when muscle breaks down, and it is sometimes be
found in the blood of patients with MND.

MRI brain scan: This cannot detect an MND, but it can help rule out other conditions, such
as stroke, brain tumor, brain circulation problems, or abnormal brain structure.

Electromyography (EMG) and nerve conduction study (NCS): These are often
performed together. An EMG tests the amount of electrical activity within muscles, while
NCS tests the speed at which electricity moves through muscles.

Spinal tap, or lumbar puncture: This analyzes the cerebrospinal fluid, the fluid that
surrounds the brain and spinal cord.

Muscle biopsy: If the doctor thinks the patient may have a muscle disease, rather than
MND, a muscle biopsy may be performed.

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After tests, a doctor will normally monitor the patient for some time before confirming that
they have MND.

Criteria known as El Escorial criteria can

help a doctor check for distinctive
neurological signs, that may aid in the
diagnosis of ALS.

These include:

muscle shrinking, weakness or twitching

muscle stiffness or abnormal reflexes
symptoms spreading into new muscle
groups
having no other factors that explain the
symptoms

Treatment:

There is no cure for MND, so treatments focus on slowing the progression and maximizing
patient independence and comfort.

This can include the use of breathing, feeding, mobility and communication appliances and
devices.

Rehabilitation therapy may include physical, occupational and speech therapy.

Muscle cramps and stiffness

Muscle cramps and stiffness can be treated with physical therapy and medications, such
botulinum toxin (BTA) injections. BTA blocks the signals from the brain to the stiff muscles
for about 3 months.

Baclofen, a muscle relaxer, may reduce muscle stiffness. A small pump is surgically
implanted outside the body and connected to the space around the spinal cord. A regular
dose of baclofen is delivered into the nervous system.

Baclofen blocks some of the nerve signals that cause spasticity. It may help with extreme
yawning.

Treatment for drooling

Scopolamine, a drug for motion sickness, may help control symptoms of drooling. It is worn
as a patch behind the ear.

Uncontrolled laughter or crying

Antidepressants, called serotonin reuptake inhibitors (SSRIs), may help with episodes of
uncontrollable laughter or crying, known as emotional lability.

Speech, occupational and physical therapy

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Patients with speech and communication difficulties may learn some useful techniques with
a qualified speech and language therapist. As the disease advances, patients often need
some communication aids.

Physical and occupational therapy can help maintain mobility and function, and reduce
stress.

Swallowing difficulties (dysphagia)

As eating and drinking become harder, the patient may need a percutaneous endoscopic
gastrostomy (PEG), a feeding tube that is placed on the abdomen, a relatively minor
procedure.

Pain

A non-steroidal anti-inflammatory drug (NSAID), such as ibuprofen, will help with mild to
moderate pain from muscle cramping as spasms. Drugs such as morphine can help relieve
severe joint and muscle pain in the advanced stages.

Breathing problems

Respiratory muscles usually weaken gradually, but a sudden deterioration is possible.

Mechanical ventilation can help with breathing. A machine takes in air, filters it, and pumps
it into the lungs often through a tracheostomy, a surgical hole in the neck that allows for
assisted breathing.

Some people use complementary therapies, including special diets that are high in
vitamins. These will not cure MND, but following a healthful diet can improve overall health
and wellbeing.

Physiotherapy exercise for MND:

Physical exercise can help maintain or improve strength in the muscles not affected by
MND, and maintain flexibility in muscles that are affected. It can help prevent stiffness in
the joints. Physiotherapy may also help people with breathing difficulties to clear their
chests and maintain lung capacity. However, people living with MND can tire very easily
and find they need to conserve energy, so strenuous exercise is not normally
recommended.

Several people had found physiotherapy and gentle exercise helpful, including
hydrotherapy or swimming.

Chest Care:

Muscle weakness is a common feature in MND and can include the muscles in and around
the chest affecting
breathing.
Breathing – INSPIRATION (taking air in )
– EXPIRATION ( breathing out)
The main muscle of inspiration is the diaphragm. It is located between the chest cavity and
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the abdomen and moves
down in inspiration against the abdominal contents. At the same time the ribs move up and
out causing an increase in the volume of the chest cavity allowing the lungs to expand.
Expiration is the recoil of these muscles.
It is important to maintain well aerated lungs to:-
Expand the bases of the lungs to prevent collapse therefore reducing the risk of a chest
infection.
Improve the level of oxygen in the body (a lack will manifest as a shortness of breath)
Remove any build up of carbon dioxide
Improve the effectiveness of a cough which will help to remove any secretions.
Prevent stiffness of the ribs and spine.

It is important to monitor your own chest. Any deterioration should be acted on immediately
via your G.P,
Physiotherapist. Consultant etc.
To maintain a clear chest you should try to incorporate breathing exercises into your daily
routine i.e. first thing in the morning, before each mealtime, before going to bed. This way
you will be regularly expanding your lungs and removing any secretions.

BREATHING EXERCISES (Active Cycle of Breathing)

3 deep breaths
Normal breathing
3 deep breaths
Cough
BREATH STACKING—(for those who find it difficult to take one deep breath)
Inhale until your chest expands slightly.
Hold the breath for 2 seconds.
Breath in again.
Hold the breath for another 2 seconds without exhaling Repeat one more time then exhale
slowly through the
mouth.
Repeat the cycle 4 times.
ASSISTED COUGH – is useful if you can not generate an effective cough. It involves the
carer applying pressure to
the sides of the chest to provide external pressure to the cough. (the Physiotherapist will
demonstrate this)

Positioning:
Chest care can, and should, be performed in different positions i.e. sitting standing, side
lying ,reclining etc. This will help aerate different areas of the lungs and move secretions.
Gravity can assist to move the abdominal contents down therefore
making it easier for the diaphragm to move.

EXERCISE:
Any form of exercise that will increase the speed or depth of breathing will help to aerate
and clear the lungs.
During the night our breathing becomes shallower. If the chest musculature is weakened
due to MND this can lead to :-
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Waking during the night.
Fatigue during the day.
Dreaming +++
Poor appetite.
Morning headaches.
Confusion.
If this occurs NIV (Non-invasive ventilation) may be discussed.NIV assists breathing via a
mask. Initially it is used during the night and assists the patient to aerate their lungs thus
improve the level of oxygen in the body. This in turn makes the patient feel better when
awake.

Maintain flexibility of the chest musculature.

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