Motor Neurone Disease

Question 1

What is MND?

Answer 1

Motor neurone disease

An umbrella term for progressive conditions where motor neurones stop functioning

Question 2

What are the types of MND?

Answer 2

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

Question 3

What are the features of amylotrophic lateral sclerosis?

Answer 3

Chromosome 21

UMN signs in legs
LMN signs in arms

Question 4

What are the features of primary lateral sclerosis?

Answer 4

UMN signs

Question 5

What are the features of progressive bulbar palsy?

Answer 5

Palsy og

• tongue
• mastication muscles
• facial muscles

Worst prognosis

Question 6

What are the features of progressive muscular atrophy?

Answer 6

LMN signs

Affects distal muscles before proximal muscles

Question 7

What are the features of MND?

Answer 7

Older patient >40

Progressive muscle weakness
Spares the extraocular muscles

Increased fatigue after exercise

Clumsiness

Dysarthria

Question 8

What do nerve conduction studies show in MND?

Answer 8

Normal motor conduction

Question 9

How is MND managed?

Answer 9

MDT approach

Riluzole

• prevents stimulation of glutamate receptors
• slows progression

NIV