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ClinEd 1 > PRELIMS: Motor Neuron Disease > Flashcards

PRELIMS: Motor Neuron Disease Flashcards

1
Q

What are the three types of neurons and their primary functions?

A

The three types of neurons are:

Sensory neurons: Detect external stimuli and convert it into information for the nervous system.
Interneurons: Connect sensory and motor neurons, transmitting information between them.
Motor neurons: Use information from sensory neurons to control muscle and gland activity.

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2
Q

What is the primary function of motor neurons?

A

Motor neurons transmit signals from the brain and spinal cord to muscle cells, controlling voluntary and involuntary movements.

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2
Q

How many neurons are estimated to be in the human body?

A

There are approximately 80 billion neurons in the human body.

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3
Q

Where do upper motor neurons originate, and what is their function?

A

Upper motor neurons originate in the motor cortex of the brain or brainstem and transmit signals to interneurons and lower motor neurons.

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4
Q

What are the two names for Amyotrophic Lateral Sclerosis (ALS) based on location?

A

In the US and Canada, it’s called Lou Gehrig’s disease; in the UK and Australia, it’s called Motor Neurone Disease (MND).

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4
Q

What is the role of lower motor neurons?

A

Lower motor neurons, found in the brainstem and spinal cord, communicate directly with muscles and glands, controlling their functional output.

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5
Q

What is Motor Neuron Disease (MND), and what does it affect?

A

MND is an uncommon condition that affects the brain and nerves, causing progressive muscle weakness and loss of function over time.

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5
Q

What is a common outcome for individuals in the late stages of ALS/MND?

A

Most people with ALS die in their sleep at home due to respiratory failure.

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5
Q

What is the primary characteristic of ALS/MND?

A

: ALS/MND is a neurodegenerative disease that results in the progressive loss of motor neurons, leading to muscle atrophy and weakness.

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6
Q

What is the DNA damage theory related to hereditary MND?

A

The DNA damage theory suggests that mutations in the TARDBP (TAR DNA-binding protein 43) gene, involved in DNA repair, contribute to the development of MND.

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7
Q

Types of Motor Neuron Disease (MND)
Affects the brainstem, causing difficulty with speaking, swallowing, and breathing.

A

Progressive Bulbar Palsy (PBP)

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7
Q

How do symptoms progress in MND?

A

As MND progresses, individuals may lose the ability to walk, use their hands, speak, swallow, and breathe independently.

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7
Q

What are some initial symptoms of Motor Neuron Disease?

A

Initial symptoms include muscle weakness or atrophy, trouble swallowing or breathing, cramping, stiffness, and slurred or nasal speech.

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8
Q

What percentage of Motor Neuron Diseases (MNDs) are hereditary, and what are potential causes for the others?

A

Around 10% of MNDs are hereditary. The other 90% occur randomly, with potential causes including environmental, toxic, viral, or genetic factors.

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9
Q

Types of Motor Neuron Disease (MND)
Affects lower motor neurons, leading to progressive muscle wasting, particularly in the arms, legs, and mouth.

A

Progressive Muscular Atrophy (PMA)

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9
Q

Types of Motor Neuron Disease (MND)
A rare form affecting neurons in the brain, progressing slowly and not fatal.

A

Primary Lateral Sclerosis (PLS):

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10
Q

Types of Motor Neuron Disease (MND)
An inherited form of MND affecting children, with three types caused by a genetic mutation (SMA1), primarily affecting the trunk, legs, and arms.

A

Spinal Muscular Atrophy (SMA)

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11
Q

What are the two main types of ALS presentations?

A

The two main types of ALS presentations are:

Spinal-onset ALS (70%): Characterized by muscle weakness and atrophy in the limbs and trunk.
Bulbar-onset ALS (50%): Involves cognitive and language impairments, with 85% of patients exhibiting bulbar changes as the disease progresses.

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11
Q

a neurological disease that attacks the nerve cells controlling voluntary muscles. It’s named after the baseball player Lou Gehrig, who was diagnosed with the disease.

A

ALS, also known as Lou Gehrig’s disease or Motor Neuron Disease (MND)

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12
Q

Types of Motor Neuron Disease (MND)
The most common type, affecting both upper and lower motor neurons. It impacts muscles in the arms, legs, mouth, and respiratory system.

A

Amyotrophic Lateral Sclerosis (ALS

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13
Q

What are the aims and goals of physiotherapy management for MND patients?

A

Delaying loss of strength
Maintaining endurance
Limiting pain
Improving speech and swallowing
Preventing chest complications
Preventing musculoskeletal complications
Promoting functional independence
Ambulation support

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14
Q

What are the types of Spinal Muscular Atrophy (SMA)?

A

SMA Type 0 (Congenital)
SMA Type 1 (Severe, Werdnig-Hoffman Disease)
SMA Type 2 (Intermediate, Dubowitz Disease)
SMA Type 3 (Mild, Kugelberg-Welander Disease)
SMA Type 4 (Adult-onset)

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14
Q

What types of exercises are recommended to delay loss of strength in MND patients?

A

Regular active or active-assisted exercises
Small set of walks in the corridor
Task-oriented activities
Suspension or hydrotherapy
Resisted exercises (e.g., using Theraband springs)

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14
Q

: How can endurance be maintained in MND patients?

A

Slow static cycling
Treadmill exercises
Aerobic exercises (if respiratory function is not compromised)
Small sets of brisk walking

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15
Q

What physiotherapy interventions can help limit pain in MND patients?

A

TENS or MENS for radiating pain
Ultrasound for muscle stiffness or joint stiffness
SWD for muscular spasm and pain
Moist heat or cold therapy, depending on the patient’s sensations

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15
Q

What techniques can improve speech and swallowing in MND patients?

A

Propped-up positioning during feeding to prevent choking and aspiration
Oromotor retraining, such as icing and sensory integration over lips and throat
Avoiding saliva accumulation to prevent aspiration
Incorporating O2 during sleep and elevating the head end to manage sleep apnea and breathing problems

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15
Q

What exercises can prevent chest complications in MND patients?

A

Incentive spirometry with single breath counting
Effective huffing and coughing, deep breathing exercises
Segmental breathing exercises
Hourly side-lateral positioning to prevent secretion accumulation
Cardiovascular endurance exercises like walking on a treadmill for 5 minutes or cycling for 5 to 10 minutes

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15
Q

What is the most common type of Motor Neuron Disease (MND)?

A

Amyotrophic Lateral Sclerosis (ALS) is the most common type of MND.

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15
Q

How can musculoskeletal complications be prevented in MND patients?

A

Use of assistive devices and splints
Exercises to prevent contracture deformity or tightness
Passive sustained stretching for spasticity
Tone-inducing or tone-reducing techniques
Active free exercises

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15
Q

Which type of MND primarily affects the neurons in the brain?

A

Primary Lateral Sclerosis (PLS) affects the neurons in the brain and progresses more slowly than ALS.

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15
Q

What muscles are affected by Amyotrophic Lateral Sclerosis (ALS)?

A

ALS affects the muscles of the arms, legs, mouth, and respiratory system.

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15
Q

What is the only known mode of transmission for ALS?

A

Familial and sporadic ALS, primarily stemming from genetic factors.

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15
Q

What are the primary risk factors for developing ALS?

A

Heredity and genetics
Age (50s to mid-60s)
Gender (more common in men)

15
Q

How are cramps managed in ALS?

A

Cramps can be managed with exercise, physical therapy, massage, and hydrotherapy.

15
Q

What distinguishes Classic ALS from other forms of ALS?

A

Classic ALS involves both upper and lower motor neurons, unlike other forms that may involve only one.

15
Q

What medications are used to treat sialorrhea in ALS patients?

A

Amitriptyline, atropine drops, and scopolamine patches.

16
Q

What are the symptoms of SMA Type 1?

A

Symptoms include limited head control, decreased muscle tone (hypotonia), difficulty swallowing and breathing, with death often occurring before the second birthday.

17
Q

Which SMA type typically appears after age 21?

A

SMA Type 4 (Adult-onset), which has mild symptoms and doesn’t usually affect life expectancy.

17
Q

a chronic autoimmune disease caused by autoantibodies that block acetylcholine receptors at motor end plates, leading to rapid fatigability of striated muscles.

A

Myasthenia Gravis

17
Q

What types of tests are used to diagnose SMA?

A

Creatine kinase blood test
Electromyogram (EMG) and nerve conduction study
Muscle biopsy
Amniocentesis
Chorionic villus sampling (CVS)

18
Q

What is the epidemiology of Myasthenia Gravis?

A

Not hereditary
Incidence: approx. 3 in 100,000
Can manifest at any age
More common in women (20-30 years)
More common in men (40-60 years)

18
Q

What complications are associated with SMA?

A

Respiratory issues, such as breathing difficulties and infections
Orthopedic problems, including scoliosis and joint deformities
Nutritional challenges, like feeding problems and malnutrition
Reduced mobility and independence due to progressive weakness

18
Q

What are the types of Myasthenia Gravis?

A

Neonatal Myasthenia Gravis: Transient, affects 10-15% of infants born to mothers with MG.
Congenital Myasthenia
Juvenile Myasthenia: Onset around 10 years.
Ocular Myasthenia
Generalized Autoimmune Myasthenia

18
Q

What is the purpose of physical therapy in SMA management?

A

To maintain muscle strength, flexibility, and mobility through exercises, stretching, and mobility aids.

19
Q

List some clinical manifestations of Myasthenia Gravis.

A

Fatigue and progressive weakness
Difficulty talking, walking up stairs, facial paralysis
Difficulty breathing, swallowing, or chewing
Drooping eyelids, double vision
Symptoms worsen with physical activity, improve with rest

19
Q

Explain the pathophysiology of Myasthenia Gravis.

A

Autoantibodies target acetylcholine receptors (AChR) at neuromuscular junctions, leading to receptor degradation and reduced efficacy. Additionally, some patients may have antibodies against muscle-specific kinase (MuSK) or LRP4, which are involved in AChR clustering.

19
Q

What is the role of the thymus in Myasthenia Gravis?

A

The thymus is abnormal in 75% of patients with MG, with 65% showing hyperplasia and 10% having thymomas. Muscle-like cells in the thymus may express AChR, potentially triggering an autoimmune response.

19
Q

What is Myasthenic Crisis?

A

Myasthenic Crisis is extreme muscle weakness that causes quadriparesis or quadriplegia, difficulty swallowing, and shortness of breath, leading to a risk of respiratory arrest.

20
Q

results from anticholinesterase drug toxicity, causing symptoms similar to Myasthenic Crisis but with additional gastrointestinal symptoms, bradycardia, increased salivation, and sweating, also posing a risk of respiratory arrest.

A

Cholinergic Crisis

21
Q

an acute infectious viral disease caused by the poliovirus, primarily affecting young children and leading to the destruction of motor neurons, which can result in muscle weakness, flaccid paralysis, and permanent disability.

A

Poliomyelitis

22
Q

What are the three types of polioviruses?

A

Type 1 (Brunhilde), Type 2 (Lansing), and Type 3 (Leon).

22
Q

How is poliomyelitis transmitted?

A

Through contaminated food and water, droplet infection, and oral ingestion.

23
Q

What is the incubation period for poliomyelitis?

A

The incubation period varies from 3 to 30 days.

24
Q

What are the three stages of poliomyelitis pathogenesis?

A

Alimentary stage, viremic stage, and neural stage.

25
Q

What happens during the neural stage of poliomyelitis?

A

The poliovirus enters the central nervous system (CNS) and destroys anterior horn cells of the spinal cord, leading to muscle weakness and paralysis.

26
Q

What are the symptoms of subclinical poliomyelitis infection?

A

Headache, slight fever, vomiting, general discomfort, back and neck pain, leg and muscle pain, and tenderness over affected muscle groups.

27
Q

Describe the acute stage of poliomyelitis.

A

Lasts 7 to 10 days, with symptoms including restlessness, irritability, pain, muscle spasms, and low-grade fever.

27
Q

What are Acute Motor Axonal Neuropathy (AMAN) and Acute Motor-Sensory Axonal Neuropathy (AMSAN)?

A

These are variants of GBS, often preceded by diarrhea (e.g., from Campylobacter jejuni), with rapid onset of weakness, quadriplegia, and respiratory insufficiency.

28
Q

What occurs during the recovery phase of poliomyelitis?

A

Begins after acute symptoms subside and lasts 3 to 12 months. Muscle strength may improve, but paralysis may also develop.

29
Q

What are common deformities associated with poliomyelitis?

A

Contractures in the hip, knee, ankle, foot, shoulder, elbow, forearm, abdomen, back, scapula, and neck due to muscle imbalances.

30
Q

What are potential late complications of poliomyelitis?

A

Joint pains, degenerative joint disease, myofascial pain, decreased endurance and strength, fractures, and respiratory insufficiency.

30
Q

What are the goals of treatment during the chronic stage of poliomyelitis?

A

To correct muscle imbalances, prevent or correct soft tissue or bony deformities, and maintain functional stability.

30
Q

What are common clinical features of GBS?

A

Rapidly evolving symmetric motor paralysis, often with sensory and autonomic disturbances, starting with numbness or tingling in the fingers and toes.

31
Q

What is the role of plasma exchange in GBS management?

A

Plasma exchange removes immune components from the blood, reducing the time on a ventilator and improving strength recovery at one year.

31
Q

What is molecular mimicry in the context of GBS?

A

Molecular mimicry occurs when a microbe’s cell surface antigen resembles a self-protein, leading to an autoimmune response where the body’s immune system mistakenly attacks its own tissues.

31
Q

What are the initial symptoms of GBS?

A

Initial symptoms include numbness or tingling in the fingers and toes, progressing to proximal muscle weakness in the lower extremities, which can extend to the arms, trunk, cranial nerves, and respiratory muscles.

31
Q

What are the key cranial nerve involvements in GBS?

A

Facial droop (mimicking Bell’s palsy), diplopia, dysphagia, ophthalmoplegia, and pupillary disturbances.

31
Q

What are the most common antecedent events triggering GBS?

A

Infections (e.g., influenza, EBV, Campylobacter jejuni), systemic diseases (e.g., Hodgkin’s disease, hyperthyroidism), and other events like surgery, immunization, and pregnancy.

31
Q

What are the mortality rates associated with severe cases of GBS?

A

Mortality rates for severe cases of GBS, such as those with AIDP, range from 3% to 5%.

32
Q

What is Guillain-Barré Syndrome (GBS)?

A

GBS is an acute inflammatory polyradiculoneuropathy that results in weakness and diminished reflexes, typically following a triggering event like an infection.

32
Q

What is the typical approach to diagnosing GBS?

A

Diagnosis is usually clinical, supported by elevated CSF protein levels, normal glucose, and a normal cell count, along with imaging like MRI showing thickened nerve roots.

33
Q

What are some autonomic changes seen in GBS?

A

Tachycardia, bradycardia, facial flushing, orthostatic hypotension, anhidrosis/diaphoresis, and urinary retention.

33
Q

What are the respiratory complications associated with GBS?

A

Respiratory complications include dyspnea on exertion, difficulty swallowing, slurred speech, shortness of breath, and ventilatory failure, which may require respiratory support.

34
Q

What is Acute Inflammatory Demyelinating Polyradiculopathy (AIDP)?

A

AIDP is the most common form of GBS, characterized by muscle weakness starting in the lower body and spreading upward, often preceded by an identifiable event.

34
Q

What is Miller Fisher Syndrome?

A

A variant of GBS where paralysis starts in the eyes, causing double vision, droopy eyelids, and uncoordinated movement, often associated with ophthalmoplegia, ataxia, and areflexia.

35
Q

What psychological challenges do patients with GBS face?

A

Patients may experience fear, helplessness, pain, sleep deprivation, hallucinations, and depression.

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ClinEd 1 (12 decks)

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