Motor Neurone Disease

Question 1

Definition of motor neurone disease

Answer 1

Conditions that involve the progressive malfunction of motor neurones without sensory disturbance

Question 2

Types of motor neurone disease

Answer 2

Amyotrophic lateral sclerosis
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis

Question 3

Pathophysiology of motor neurone disease

Answer 3

Progressive degeneration of UMN and LMN

Question 4

Risk factors for motor neurone disease

Answer 4

FHx
Smoking
Heavy metal exposure
Pesticides

Question 5

Presentation of motor neurone disease

Answer 5

Middle aged
Male

Insidious progressive weakness of muscles throughout the body affecting the trunk, limbs, speech and face

Often first noticed in upper limbs

Increased fatigue when exercising
Dysarthria- slurred speech
Clumsiness, tripping and dropping things

Question 6

LMN signs

Answer 6

Hyporefelxia
Hypotonia
Fasciculations
Muscle wasting - atrophy of disuse

Question 7

UMN signs

Answer 7

Hypertonia
Hyper-reflexia
Muscle atrophy of denervation
Plantar response

Question 8

Management of motor neurone disease

Answer 8

MDT
Slow the progression:
- Riluzole
- Edaravone

Non invasive ventilation - support breathing at night

Advanced care - EOL planning