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Motor Neuron Disease
What is motor neuron disease?
Motor neuron disease (MND) is a neurodegenerative condition caused by
loss of upper and lower motor neurons in the spinal cord, cranial nerve
nuclei and motor cortex.
It affects the human motor system
It is a broad term which contains multiple conditions according to the site
where it affects
It is difficult to diagnose and till now there is no cure but therapy can improve
lifestyle
Motor neuron disease means disease of the motor neurons
What are the motor neurons?
Neuron that passes from the central nervous system toward or to a muscle
and conducts a nerve impulse is called motor neuron
NERVE IMPULSE
Types of motor neuron?
According to the site of origin and ending motor neurons are two types. These
are
.. Upper motor neuron
.. Lower motor neuron
Upper motor neuron: A neuron that starts in the motor cortex of the brain and
terminates within the medulla (another part of the brain) or within the spinal
cord up to anterior horn cell
Lower motor neuron: A nerve cell that goes from the anterior horn cell spinal
cord to a muscle
Upper motor neuron and lower motor neuron
Etiology of MND
..Most of the cases are sporadic only10% cases are hereditary
..The exact causes are unclear
..There are some theories explaining the causes. These may include
--Exposure to viruses
--Exposure to certain toxins and chemicals
--Genetic factors
--Immune system response
--Nerve growth factors
--Growth, repair and ageing of motor neurons
Types of motor neuron disease
Motor neuron disease can be classified according to the neurons that are affected
..Only upper motor neuron lesion: Primary lateral sclerosis, Pseudobulbar palsy
..Only lower motor neuron lesion: progressive muscular atrophy, Monomelik
amyotrophy
..Both upper motor and lower motor neuron lesion: amyotrophic lateral sclerosis,
progressive bulbar palsy
Signs and symptoms of upper motor neuron lesion
..Muscle weakness
..Muscle tightness or spastic paralysis
..Overactive reflex
..Clonus
..Babinski response
Signs and symptoms of lower motor neuron lesion
..Muscle weakness
..Muscle wasting
..Flaccid paralysis
..Fasciculation
..Hypotonia or atonia
..Hyporeflexia
Among different types of MND there are four main types
..Amyotrophic lateral sclerosis (ALS): 60-70%
..Progressive bulbar palsy (PBP)
..Progressive muscular atrophy (PMA)
..Primary lateral sclerosis (PLS)
Commonly known as "Lou
Gehrig's disease," named after
the famous New York Yankees
baseball player who was
forced to retire after
developing the disease in
1939 and died 1941
Amyotrophic Lateral Sclerosis (ALS)
Lou Gehrig's disease
Amyotrophic Lateral Sclerosis (ALS)
..This is the commonest type of motor neuron disease
..Here both upper and lower motor neurons are affected
..As both upper and lower motor neurons are affected so symptoms of both upper
motor and lower motor neuron lesion are present. They are discussed in previous
slides
..Signs and symptoms might include:
-Difficulty walking or doing normal daily activities
-Tripping and falling
-Weakness in your leg, feet or ankles
-Hand weakness or clumsiness
-Slurred speech or trouble swallowing
-Muscle cramps and twitching in your arms, shoulders and tongue
Inappropriate crying, laughing or yawning
-Cognitive and behavioral changes
Amyotrophic Lateral Sclerosis (ALS)
..The earliest symptoms of ALS are muscle weakness or muscle atrophy. Other
presenting symptoms include trouble swallowing or breathing, cramping, or stiffness
of affected muscles; muscle weakness affecting an arm or a leg; or slurred and nasal
speech. It depends on the site of the neurons that are affected.
..As the disease progress patient is unable to walk or do normal works. Later they
are unable to talk eat or move. They have difficulty in breathing.
.. The most common cause of death for people with ALS is respiratory failure when
the respiratory muscles are paralyzed.
Primary Lateral Sclerosis (PLS)
..Primary lateral sclerosis (PLS) is a type of motor neuron disease that causes
nerves within the brain(UMN) to slowly break down. This makes the nerves unable
to activate the motor neurons in the spinal cord, which control muscles.
..PLS causes weakness in your voluntary muscles, such as those you use to control
your legs, arms and tongue.
..Primary lateral sclerosis progresses more slowly than ALS, and in most cases isn't
fatal.
Progressive Bulbar Palsy (PBP)
..Progressive Bulbar Palsy involves both upper and lower motor neurons
..PBP involves mainly the brain stem. The brain stem is the part of the brain
needed for swallowing, speaking, chewing, and other functions.
.. Signs and symptoms of progressive bulbar palsy include difficulty swallowing,
weak jaw and facial muscles, progressive loss of speech, and weakening of the
tongue.
Progressive muscular atrophy (PMA)
..Progressive muscular atrophy (PMA) is clinically characterized solely by signs of
lower motor neuron (LMN) dysfunction.
..Symptoms of PMA include:
atrophy
fasciculations
muscle weakness
..It is a sibling condition to amyotrophic lateral sclerosis (ALS), which affects both
the upper and lower motor neurons.
Onset and Prognosis
..The onset of symptoms varies but most commonly the disease is first recognized
between 20 and 40 years of age.
..Symptoms often develop very slowly over a number of years. At some stage,
symptoms are likely to affect a person's daily life. Everyone's experience
and progression will be different but most people will experience increasing
disability
.. Life expectancy for about half of those with the condition is three years from the
start of symptoms. However, some people may live for up to 10 years, and in rarer
circumstances even longer.
Diagnosis of MND
..Diagnosing motor neuron disease is often a difficult and lengthy process
..It is mainly diagnosed from physical examination and observing symptoms
..There are no single test to diagnose rather investigations are done to exclude
differentials.
..Tests may include blood test, electromyography(EMG), nerve conduction
test(NCS), MRI, muscle biopsy, lumbar puncture
Treatment of MND
..There is no cure for motor neuron disease
Symptomatic treatment
..Treatment is given to reduce symptoms and improve the quality of life
..Botox and some muscle relaxants may be used for muscle cramp and muscle
twitching
..Painkillers are used to relieve pain
..Antidepressants are used to improve psychological upset
Recent drug
..Riluzole is a drug that is being used in some countries in patient with MND. It can
reduce disease progression and increase life expectancy
Treatment of MND
Therapy
..Speech and language therapy can help with communication and swallowing.
..Physical and occupational therapy can help maintain mobility and function and
encourage people to find new ways to do specific tasks.
Assisted device and others
..In some cases and in last stage some assisted device may be required which will
help the patient to move, feed, breath and communicate with others
Mnd patient suffer from breathing difficulty for which breathing and ventilatory
support may be needed. Tube feeding maybe required who are unable to feed
Carers of MND
Taking on the role of caregiver for a loved one with Motor Neuron Disease was
likely profoundly unexpected and a role that you may not feel adequately prepared
for.
But without the right support, the personal cost of caring can be high, with many
carers experiencing poor health, poverty and disadvantage.
Good outcomes for carers occur when:
--the carer is able to cope with their caring role,
--the carer gets a regular break from caring,
--the carer is informed and knowledgeable about their caring role and the needs
of the person they care for, and
--the carer feels valued, supported and listened to
Life is a mystery
box. Never lose
hope. Never give
up. Keep faith. You
never know what’s
coming
Motor neuron disease

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Motor neuron disease

  • 2.
  • 3. What is motor neuron disease? Motor neuron disease (MND) is a neurodegenerative condition caused by loss of upper and lower motor neurons in the spinal cord, cranial nerve nuclei and motor cortex. It affects the human motor system It is a broad term which contains multiple conditions according to the site where it affects It is difficult to diagnose and till now there is no cure but therapy can improve lifestyle Motor neuron disease means disease of the motor neurons
  • 4. What are the motor neurons? Neuron that passes from the central nervous system toward or to a muscle and conducts a nerve impulse is called motor neuron NERVE IMPULSE
  • 5. Types of motor neuron? According to the site of origin and ending motor neurons are two types. These are .. Upper motor neuron .. Lower motor neuron Upper motor neuron: A neuron that starts in the motor cortex of the brain and terminates within the medulla (another part of the brain) or within the spinal cord up to anterior horn cell Lower motor neuron: A nerve cell that goes from the anterior horn cell spinal cord to a muscle
  • 6. Upper motor neuron and lower motor neuron
  • 7. Etiology of MND ..Most of the cases are sporadic only10% cases are hereditary ..The exact causes are unclear ..There are some theories explaining the causes. These may include --Exposure to viruses --Exposure to certain toxins and chemicals --Genetic factors --Immune system response --Nerve growth factors --Growth, repair and ageing of motor neurons
  • 8. Types of motor neuron disease Motor neuron disease can be classified according to the neurons that are affected ..Only upper motor neuron lesion: Primary lateral sclerosis, Pseudobulbar palsy ..Only lower motor neuron lesion: progressive muscular atrophy, Monomelik amyotrophy ..Both upper motor and lower motor neuron lesion: amyotrophic lateral sclerosis, progressive bulbar palsy
  • 9. Signs and symptoms of upper motor neuron lesion ..Muscle weakness ..Muscle tightness or spastic paralysis ..Overactive reflex ..Clonus ..Babinski response
  • 10. Signs and symptoms of lower motor neuron lesion ..Muscle weakness ..Muscle wasting ..Flaccid paralysis ..Fasciculation ..Hypotonia or atonia ..Hyporeflexia
  • 11. Among different types of MND there are four main types ..Amyotrophic lateral sclerosis (ALS): 60-70% ..Progressive bulbar palsy (PBP) ..Progressive muscular atrophy (PMA) ..Primary lateral sclerosis (PLS)
  • 12. Commonly known as "Lou Gehrig's disease," named after the famous New York Yankees baseball player who was forced to retire after developing the disease in 1939 and died 1941 Amyotrophic Lateral Sclerosis (ALS) Lou Gehrig's disease
  • 13. Amyotrophic Lateral Sclerosis (ALS) ..This is the commonest type of motor neuron disease ..Here both upper and lower motor neurons are affected ..As both upper and lower motor neurons are affected so symptoms of both upper motor and lower motor neuron lesion are present. They are discussed in previous slides ..Signs and symptoms might include: -Difficulty walking or doing normal daily activities -Tripping and falling -Weakness in your leg, feet or ankles -Hand weakness or clumsiness -Slurred speech or trouble swallowing -Muscle cramps and twitching in your arms, shoulders and tongue Inappropriate crying, laughing or yawning -Cognitive and behavioral changes
  • 14. Amyotrophic Lateral Sclerosis (ALS) ..The earliest symptoms of ALS are muscle weakness or muscle atrophy. Other presenting symptoms include trouble swallowing or breathing, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; or slurred and nasal speech. It depends on the site of the neurons that are affected. ..As the disease progress patient is unable to walk or do normal works. Later they are unable to talk eat or move. They have difficulty in breathing. .. The most common cause of death for people with ALS is respiratory failure when the respiratory muscles are paralyzed.
  • 15.
  • 16. Primary Lateral Sclerosis (PLS) ..Primary lateral sclerosis (PLS) is a type of motor neuron disease that causes nerves within the brain(UMN) to slowly break down. This makes the nerves unable to activate the motor neurons in the spinal cord, which control muscles. ..PLS causes weakness in your voluntary muscles, such as those you use to control your legs, arms and tongue. ..Primary lateral sclerosis progresses more slowly than ALS, and in most cases isn't fatal.
  • 17. Progressive Bulbar Palsy (PBP) ..Progressive Bulbar Palsy involves both upper and lower motor neurons ..PBP involves mainly the brain stem. The brain stem is the part of the brain needed for swallowing, speaking, chewing, and other functions. .. Signs and symptoms of progressive bulbar palsy include difficulty swallowing, weak jaw and facial muscles, progressive loss of speech, and weakening of the tongue.
  • 18. Progressive muscular atrophy (PMA) ..Progressive muscular atrophy (PMA) is clinically characterized solely by signs of lower motor neuron (LMN) dysfunction. ..Symptoms of PMA include: atrophy fasciculations muscle weakness ..It is a sibling condition to amyotrophic lateral sclerosis (ALS), which affects both the upper and lower motor neurons.
  • 19. Onset and Prognosis ..The onset of symptoms varies but most commonly the disease is first recognized between 20 and 40 years of age. ..Symptoms often develop very slowly over a number of years. At some stage, symptoms are likely to affect a person's daily life. Everyone's experience and progression will be different but most people will experience increasing disability .. Life expectancy for about half of those with the condition is three years from the start of symptoms. However, some people may live for up to 10 years, and in rarer circumstances even longer.
  • 20. Diagnosis of MND ..Diagnosing motor neuron disease is often a difficult and lengthy process ..It is mainly diagnosed from physical examination and observing symptoms ..There are no single test to diagnose rather investigations are done to exclude differentials. ..Tests may include blood test, electromyography(EMG), nerve conduction test(NCS), MRI, muscle biopsy, lumbar puncture
  • 21. Treatment of MND ..There is no cure for motor neuron disease Symptomatic treatment ..Treatment is given to reduce symptoms and improve the quality of life ..Botox and some muscle relaxants may be used for muscle cramp and muscle twitching ..Painkillers are used to relieve pain ..Antidepressants are used to improve psychological upset Recent drug ..Riluzole is a drug that is being used in some countries in patient with MND. It can reduce disease progression and increase life expectancy
  • 22. Treatment of MND Therapy ..Speech and language therapy can help with communication and swallowing. ..Physical and occupational therapy can help maintain mobility and function and encourage people to find new ways to do specific tasks. Assisted device and others ..In some cases and in last stage some assisted device may be required which will help the patient to move, feed, breath and communicate with others Mnd patient suffer from breathing difficulty for which breathing and ventilatory support may be needed. Tube feeding maybe required who are unable to feed
  • 23. Carers of MND Taking on the role of caregiver for a loved one with Motor Neuron Disease was likely profoundly unexpected and a role that you may not feel adequately prepared for. But without the right support, the personal cost of caring can be high, with many carers experiencing poor health, poverty and disadvantage. Good outcomes for carers occur when: --the carer is able to cope with their caring role, --the carer gets a regular break from caring, --the carer is informed and knowledgeable about their caring role and the needs of the person they care for, and --the carer feels valued, supported and listened to
  • 24. Life is a mystery box. Never lose hope. Never give up. Keep faith. You never know what’s coming