Motor Neuron Disease

Question 1

Motor neurone disease is a neurodegenerative condition affecting the _________ and ___________

Answer 1

brain

spinal cord

Question 2

In motor neuron disease, degeneration of motor neurones leads to progressive muscle weakness; resulting symptoms include ___________, __________ and _________, ____________, reduced ________ function and ______________.

Answer 2

muscle cramps

wasting

stiffness

loss of dexterity

respiratory

cognitive dysfunction

Question 3

The most common form of motor neuron disease is ____________

Answer 3

ALS

Question 4

Is there a cure for motor neuron disease?

Answer 4

No; the aims of treatment are to maintain functional ability and manage symptoms

Question 5

What are the components of non-drug treatment for motor neuron disease? (5)

Answer 5

1. Nutrition
2. Psychosocial support
3. Physiotherapy
4. Exercise programmes
5. Use of special equipment and mobility aids

Question 6

What is the first line treatment for muscular cramps in motor neuron disease?

Answer 6

First line: Quinine (unlicensed)

Second line: baclofen (unlicensed)

Third line: tizanidine (unlicensed), dantrolene (unlicensed), gabapentin (unlicensed)

Question 7

What is the treatment of increased muscle tone, stiffness, and spasticity in motor neuron disease? (4)

Answer 7

• Baclofen
• tizanidine
• dantrolene
• gabapentin (unlicensed)

*severe spasticity may require specialist referral

Question 8

What is the treatment of salivation problems in patients with motor neuron disease? (4)

Answer 8

Trial of antimuscarinic drugs (unlicensed) can be considered for excessive drooling

• In patients with cognitive impairment, glycopyrronium bromide is preferred
• if initial treatment is ineffective, not tolerated, or contraindicated, botulinum toxin may be required
• for thick, tenaicious saliva, humidification, nébuliseurs and carbocisteine may be used

Question 9

What is the treatment of salivation problems in patients with motor neuron disease and cognitive impairment?

Answer 9

Glycopyrronium bromide

Question 10

How should respiratory symptoms in a patient with motor neuron disease be approached? (3)

Answer 10

1. First rule out/treat reversible causes of worsening impairment (eg RTI or secretion problems)
2. Opioids OR benzodiazepines if the patients symptoms are related to anxiety (both unlicensed)
3. Non-invasive ventilation should be considered in patients with respiratory impairment

Question 11

Which drug is used in the treatment of ALS to extend life or extend the time to mechanical ventilation?

Answer 11

Riluzole

Question 12

What is the mechanism of action of riluzole?

Answer 12

Glutamate antagonist, blocks release from neurons

Only disease modifying drug available for the treatment of ALS