Motor Neurone Disease (MND)

Question 1

What is MND?

Answer 1

Progressive degeneration of the motor neurones that leads to a mixture of UMNs and LMNs

Question 2

What is the most common type of MND?

Answer 2

Amylotrophic lateral sclerosis

Question 3

What are the features of ALS?

Answer 3

Upper motor and lower motor neuron signs

Question 4

Are there any cerebellar effects from MND?

Answer 4

No

Question 5

What is pseudobulbar affect and can it occur in MND?

Answer 5

Uncontrollable and/or inappropriate laughing or crying. Can be a feature of MND.

Question 6

How is the diagnosis of MND made and differentiated from other diseases?

Answer 6

It is a clinical diagnosis. Nerve conduction studies show to be normal and MRI brain would be to rule out other pathology.

Question 7

How is the lung function in MND managed?

Answer 7

BiPAP may be necessary at later stage. Mucolytics to help break down mucus.

Question 8

What is the drug that extends life of person with MND by 2 months?

Answer 8

Riluzole

Question 9

What other management strategies can help a person with MND?

Answer 9

1. Treatments for mental health, low mood common
2. Change in feeding, e.g. nasogastric tube

Question 10

What are UMN signs?

UMN = above the anterior horn cell

Answer 10

1. Spasticity
2. Hyperreflexia
3. Hypertonia
4. Absence of fasciculations
5. Diffuse atrophy
6. Upgoing Babinski reflex

Question 11

What are LMN signs?

LMN = below the anterior horn cell

Answer 11

1. Flaccid
2. Hyporeflexia
3. Hypotonia
4. Fasciculations
5. Wasting atrophy
6. Normal Babinski test