Motor Neuron disease

Question 1

Motor neurone disease (MND) is an uncommon condition that affects the brain and nerves. It causes weakness that gets worse over time. What is the incidence of MND?

1 - 2000 cases per 100,000
2 - 200 cases per 100,000
3 - 20 cases per 100,000
4 - 2 cases per 100,000

Answer 1

4 - 2 cases per 100,000

Question 2

Motor neurone disease (MND) is an uncommon condition that affects the brain and nerves. It causes weakness that gets worse over time. Is this more common in men or women?

Answer 2

• men

2:1 ratio

Question 3

Motor neurone disease (MND) is an uncommon condition that affects the brain and nerves. It causes weakness that gets worse over time. What age does the incidence of MND typically peak?

1 - 60-70
2 - 50-60
3 - 40-50
4 - 30-40

Answer 3

1 - 60-70

Question 4

Is motor neurone disease (MND) dangerous?

Answer 4

• yes

Progressive disease that eventually becomes fatal

Question 5

In motor neurone disease (MND) are there any sensory effects?

Answer 5

• no

Affects motor neurons, hence the name MND

Question 6

Does motor neurone disease (MND) present with an upper or lower motor neuron presentation?

Answer 6

• can have both

Question 7

Which of the following is a tell tale sign of motor neurone disease (MND)?

1 - resting tremor
2 - pill rolling tremor
3 - essential tremor
4 - fasciculations

Answer 7

4 - fasciculations

Question 8

There is typically wasting of a specific muscles in the body for some reason in motor neurone disease (MND). Which 2 parts of the body is this most common?

1 - should (deltoids)
2 - hands (small muscles)
3 - front of shins (tibialis anterior)
4 - foot muscles

Answer 8

2 - hands (small muscles)
3 - front of shins (tibialis anterior)

Question 9

In motor neurone disease (MND) which of the following are typically spared?

1 - respiratory muscles
2 - ocular muscles
3 - gastrointestinal muscles
4 - facial muscles

Answer 9

2 - ocular muscles

Question 10

In motor neurone disease (MND), is there any cerebellar affects?

Answer 10

• no

Question 11

There are different types of motor neuron disease (MND). Which of the following is the most common form of MND?

1 - progressive bulbar palsy
2 - amyotrophic lateral sclerosis
3 - progressive muscular atrophy
4 - kennedy’s disease

Answer 11

2 - amyotrophic lateral sclerosis

Question 12

When we talk about bulbar palsy, which part of brain does bulbar relate to?

1 - cerebrum
2 - medulla oblongata
3 - mid brain
4 - pons

Answer 12

2 - medulla oblongata

Question 13

Does bulbar palsy present as a LMN or UMN presentation?

Answer 13

• LMN

Question 14

When we talk about bulbar palsy, this relates to the medulla oblongata. Which of the following cranial nerves is not affected in bulbar palsy?

1 - vestibulocochlear nerve
2 - glossopharyngeal nerve
3 - vagus nerve
4 - accessory nerve
5 - hypoglossal nerve

Answer 14

1 - vestibulocochlear nerve

Remember 2 2 4 4 formation

CN 1 and 2 = cerebrum
CN 3 and 4 = mid brain
CN 5, 6, 7 and 8 = pons
CN 9, 10, 11 and 12 = medulla

Question 15

There are different types of motor neuron disease (MND). In progressive bulbar palsy, which of the following is least likely to be a symptom in patients?

1 - dysphagia
2 - dysphonia (impaired voice production)
3 - miosis (small pupil)
4 - dysarthria (weak speech muscles)
5 - drooling
6 - nasal regurgitation

Answer 15

3 - miosis

Everything else is innervated by cranial nerves 9-12

Question 16

In acute neuromuscular respiratory weakness what can be a good clinical sign to look for?

1 - babinski sign
2 - CO2 retention flap / cyanosis
3 - increased muscle strength
4 - increased FVC

Answer 16

2 - CO2 retention flap / cyanosis

Question 17

In acute neuromuscular respiratory weakness, which of the following is the best measure of respiratory function and CO2 retention?

1 - forced vital capacity (FVC)
2 - blood gas
3 - O2 saturations
4 - all equally good

Answer 17

• FVC

Blood gases can be normal and crash abruptly, so falsely reassuring

On a blood gas respiratory acidosis, which is Type = low PO2 and high CO2

Question 18

Acute neuromuscular respiratory weakness can be dangerous, which can be caused by high spinal cord lesions. Which nerve if affected can cause the most respiratory weakness?

1 - vagus nerve
2 - phrenic nerve
3 - lateral cutaneous
4 - median nerve

Answer 18

2 - phrenic nerve

• C3, 4 and 5 keep the diaphragm alive
• Innervates the diaphragm
• Intercostal weakness can also occur

Question 19

Although the cause is unknown for motor neuron disease (MND), which of the following is NOT a risk factor that has been associated with MND?

1 - exposure to heavy metals
2 - exposure to pesticides
3 - smoking
4 - gender
5 - family history

Answer 19

4 - gender

Question 20

In motor neuron disease (MND) is there a rapid/acute or insidious onset of systemic muscle weakness?

Answer 20

• insidious onset

Typically starts in limbs, trunk, face and speech

Question 21

Which of the following is NOT a sign of a lower motor neuron problem that could be seen in motor neuron disease (MND)?

1 - brisk reflexes
2 - muscle atrophy
3 - muscle weakness
4 - fasciculations
5 - hypotonia

Answer 21

1 - brisk reflexes

Reflexes will typically be reduced or absent in LMN

Brisk reflexes are common in UMN

Question 22

Which of the following is NOT a sign of a upper motor neuron problem that could be seen in motor neuron disease (MND)?

1 - brisk reflexes
2 - muscle atrophy
3 - hypertonia/spasticity
4 - upgoing plantar reflex

Answer 22

2 - muscle atrophy

Muscle mass is typically maintained

Question 23

Which of the following would NOT present with ptosis, diplopia and ophthalmoplegia or bulbar symptoms?

1 - stroke
2 - guillain-barré syndrome
3 - spinal cord lesion
4 - myasthenia gravis

Answer 23

3 - spinal cord lesion

All the others are typically LMN presentations

Question 24

Which of the following would NOT present with hyperreflexia?

1 - stroke
2 - guillain-barré syndrome
3 - spinal cord lesion
4 - myasthenia gravis

Answer 24

2 - guillain-barré syndrome

Question 25

How is motor neuron disease typically diagnosed?

1- neuroimaging
2 - nerve conduction studies
3 - clinical diagnosis
4 - genetic testing

Answer 25

3 - clinical diagnosis

Electromyography shows a reduced action potential.

MRI imaging may rule out other causes, such as cervical cord compression and myelopathy (nerve root compression)

Question 26

There is no treatment to stop or reverse motor neuron disease (MND). Which of the following may extend life by around 3 months in amyotrophic lateral sclerosis?

1 - Riluzole
2 - Physiotherapy
3 - Bilevel positive airway pressure (BIPAP)
4 - Dexamethasone

Answer 26

1 - Riluzole

• inhibit effects of glutamate release as high glutamate damages neurons
• inactivates voltage-dependent sodium channels
• interferes with intracellular events that follow transmitter binding at excitatory amino acid receptors

Bilevel positive airway pressure (BIPAP) may also help when respiratory muscles weaken