Motor Neuron Disease

Question 1

What is motor neuron disease?

Answer 1

Progressive degeneration of motor neurons, leading to muscle wasting and weakness

Question 2

What is the average survival time for a patient with MND?

Answer 2

3 years

Question 3

Describe pattern of spread of MND

Answer 3

Focal onset

Continuous spread

Question 4

List upper MND signs

Answer 4

Increased tone, spasticity
Increased reflexes
Babinski sign

Question 5

List lower MND signs

Answer 5

Muscle wasting
Weakness
Decreased reflexes

Question 6

MND usually affects the extremities, but which - upper or lower - is more commonly affected?

Answer 6

Upper extremities more commonly affected

Question 7

90% of MND involves lower motor neuron. True/False?

Answer 7

True

Question 8

What is the most common phenotype of MND?

Answer 8

Amyotrophic lateral sclerosis (ALS)

Question 9

List the main MND phenotypes

Answer 9

Amyotrophic lateral sclerosis
Primary lateral sclerosis
Progressive bulbar palsy

Question 10

What is the main difference between ALS and PLS?

Answer 10

ALS has both UMN and LMN features, PLS UMN only

Question 11

Preferential wasting of which muscles is typical in ALS?

Answer 11

Thenar muscles (split hand syndrome)

Question 12

ALS is associated with which type of dementia?

Answer 12

Frontotemporal dementia

Question 13

List investigations for MND

Answer 13

Clinical diagnosis (onset: bulbar, chest etc.)
Neurophysiology (EMG)

Question 14

Outline management of MND

Answer 14

Assess needs and organise care
Speech therapy
Nutrition support
Ventilatory support