Motor Neurone Disease

Question 1

What is motor neurone disease?

Answer 1

Cluster of neurodegenerative disease characterised by selective loss of neurones in motor cortex, cranial nerve nuclei and anterior horn cells.

Question 2

What distinguishes motor neurone disease from multiple sclerosis and polyneuropathies?

Answer 2

UMN and LMN affected, but no sensory loss or sphincter disturbance.

Question 3

What distinguishes motor neurone disease from myasthenia gravis?

Answer 3

MND never affects eye movements.

Question 4

What is ALS and what percentage of motor neuron disease patients have it?

Answer 4

1. Amyotrophic lateral sclerosis

2. Up to 80%

Question 5

What is this a presentation of?
>40 years old, stumbling spastic gait, foot drop, proximal myopathy, weak grip and weak shoulder abduction, speech and swallowing problems.

Answer 5

Motor neurone disease

Question 6

What are the UMN signs of motor neurone disease?

Answer 6

Spasticity, brisk reflexes, upgoing plantars.

Question 7

What are the LMN signs of motor neurone disease?

Answer 7

Wasting, fasciculations of the tongue/abdomen/back/thigh.

Question 8

How is suspected motor neurone disease (ALS) diagnosed?

Answer 8

1. Revised El Escorial criteria: LMN and UMN signs in 3 regions
2. Brain/cord MRI to exclude structural causes
3. LP to exclude inflammatory causes

Question 9

What is the management for motor neurone disease?

Answer 9

1. Poor prognosis, <3 years post-onset in half of patients
2. Riluzole only medication shown to improve survival
3. Supportive/symptomatic treatment