Motor Neurone Disease

Question 1

What is motor neuron disease?

Answer 1

Progressive degeneration of motor neurons, leading to muscle wasting and weakness but no sensory problems

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 (extensor plantar response)
Spastic gait
Slowed movements

Question 5

List lower MND signs

Answer 5

Muscle wasting
Weakness (foot drop)
Decreased reflexes
Reduced tone
Fasciculations

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 muscular dystrophy (flail arm and flail leg variants)
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, cognitive behavioural symptoms etc.)
Neurophysiology (EMG) to exclude other diseases

Question 14

Outline management of MND

Answer 14

Assess needs and organise care
Communication (speech therapy, technology, voice banking)
Nutrition support
Ventilatory support
Riluzole

Question 15

What is a main contributor towards risk in MND?

Answer 15

Genetics (C9ORF hexonucleotide repeat expansion)

Question 16

MND is more common in males than females. True/ False?

Answer 16

True

Question 17

List, in order of frequency, the sites of onset in MND

Answer 17

Extremities (spinal/ limbs)
Bulbar
Frontal/ cognitive defects
Thoracic

Question 18

List the clinical features of MND cognitive impairment

Answer 18

BEHAVIOURAL ABNORMALITIES
Socially inadequate
Dis-inhibition
Reduced impulse control
Increased food intake
Weight gain
No insight

Question 19

List frequent misdiagnoses in MND

Answer 19

Cervical spondylotic radiculopathy
Carpal tunnel syndrome
Stroke
Multifocal motor neuropathy
Kennedy's disease
Inclusion body myopathy