Motor Neuron disease Flashcards
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
4 - 2 cases per 100,000
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?
- men
2:1 ratio
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
1 - 60-70
Is motor neurone disease (MND) dangerous?
- yes
Progressive disease that eventually becomes fatal
In motor neurone disease (MND) are there any sensory effects?
- no
Affects motor neurons, hence the name MND
Does motor neurone disease (MND) present with an upper or lower motor neuron presentation?
- can have both
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
4 - fasciculations
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
2 - hands (small muscles)
3 - front of shins (tibialis anterior)
In motor neurone disease (MND) which of the following are typically spared?
1 - respiratory muscles
2 - ocular muscles
3 - gastrointestinal muscles
4 - facial muscles
2 - ocular muscles
In motor neurone disease (MND), is there any cerebellar affects?
- no
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
2 - amyotrophic lateral sclerosis
When we talk about bulbar palsy, which part of brain does bulbar relate to?
1 - cerebrum
2 - medulla oblongata
3 - mid brain
4 - pons
2 - medulla oblongata
Does bulbar palsy present as a LMN or UMN presentation?
- LMN
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
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
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
3 - miosis
Everything else is innervated by cranial nerves 9-12
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
2 - CO2 retention flap / cyanosis
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
- 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
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
2 - phrenic nerve
- C3, 4 and 5 keep the diaphragm alive
- Innervates the diaphragm
- Intercostal weakness can also occur
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
4 - gender
In motor neuron disease (MND) is there a rapid/acute or insidious onset of systemic muscle weakness?
- insidious onset
Typically starts in limbs, trunk, face and speech
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
1 - brisk reflexes
Reflexes will typically be reduced or absent in LMN
Brisk reflexes are common in UMN
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
2 - muscle atrophy
Muscle mass is typically maintained
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
3 - spinal cord lesion
All the others are typically LMN presentations
Which of the following would NOT present with hyperreflexia?
1 - stroke
2 - guillain-barré syndrome
3 - spinal cord lesion
4 - myasthenia gravis
2 - guillain-barré syndrome
How is motor neuron disease typically diagnosed?
1- neuroimaging
2 - nerve conduction studies
3 - clinical diagnosis
4 - genetic testing
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)
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
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
