Motor neuron disease (MND)

Question 1

What is MND?

Answer 1

Umbrella term for progressive and ultimately fatal conditions where the motor neurons stop functioning

Question 2

Is MND a condition that affects the UMN, LMN or both?

Answer 2

Both

Question 3

MND subtypes (1)
1) What is the commonest form of MND?
2) What is the second commonest form of MND?
3) This type of MND primarily affects muscles involved with what?
4) Name another form of MND

Answer 3

1) Amyotrophic lateral sclerosis (ALS)
2) Progressive bulbar palsy
3) Talking, swallowing and chewing
4) Progressive muscular atrophy and primary lateral sclerosis

Question 4

MND subtypes (2)
1) Describe the LMN and UMN signs in ALS
2) Describe the LMN and UMN signs in primary lateral sclerosis
3) Describe the LMN and UMN signs in progressive muscular atrophy
4) Describe the LMN and UMN signs in progressive bulbar palsy
5) Which 3 cranial nerves are affected in this type?
6) As well as MND, name another cause of bulbar palsy
7) What is the difference between bulbar palsy and pseudobulbar palsy?

Answer 4

1) Typically LMN signs in arms and UMN signs in legs
2) UMN signs only
3) LMN signs only
4) LMN signs only
5) 9, 10 and 12
6) Myasthenia gravis, Guillain-Barré syndrome, brainstem stroke
7) In pseudobulbar palsy UMN affected not LMN

Question 5

MND subtypes (3)
1) Which type of MND has the best prognosis?
2) Which type of MND has the worst prognosis?

Answer 5

1) Progressive muscular atrophy
2) Progressive bulbar palsy

Question 6

Name 2 risk factors for developing MND

Answer 6

• Smoking
• Genetic link
• Exposure to heavy metals
• Exposure to pesticides

Question 7

Name 2 signs of a LMN disease

Answer 7

• Muscle wasting
• Reduced tone
• Fasciculations (twitches in the muscles)
• Reduced reflexes

Question 8

Name 2 signs of an UMN disease

Answer 8

• Increased tone or spasticity
• Brisk reflexes
• Upgoing plantar responses

Question 9

What is a MND diagnosis based on?

Answer 9

Clinical presentation and excluding other conditions that can cause motor neurone symptoms

Question 10

1) What drug can slow the progression of the disease and extend survival by a few months in ALS?
2) How does this drug work?
3) Name another aspect of the management of MND

Answer 10

1) Riluzole
2) Prevents stimulation of glutamate receptors
3) Non-invasive ventilation to support breathing at night, end of life care planning, advanced directives to document the patient’s wishes as the disease progresses, pain management and treatment of symptoms where possible