Motor Neurone Disease

Question 1

What happens in MND?

Answer 1

Loss of motor neurons in the motor cortex, cranial nerve nuclei and anterior horn cells (neurodegenerative)

Question 2

What disease is linked with MND?

Answer 2

Frontotemporal dementia (FTD occurs in ~ 25%)

Question 3

What type of MND is 80% of MND?

Answer 3

ALS

Question 4

What is a risk factor for MND?

Answer 4

Family history

Question 5

What causes MND?

Answer 5

Idiopathic

Question 6

What is the key thing about MND?

Answer 6

No sensory symptoms (or sphincter disturbance)

Question 7

How does MND present?

Answer 7

• Stumbling spastic gait
• Foot drop
• Proximal myopathy
• Weak grip and shoulder abduction

Question 8

What do you see in ALS?

Answer 8

BOTH UMN + LMN signs

Question 9

What is the main complication of MND?

Answer 9

Respiratory dysfunction (aspiration pneumonia)

Question 10

What does MND never affect (compared to MG)?

Answer 10

Eye movements

Question 11

What is the median age of onset of MND?

Answer 11

60 (>40)

Question 12

What is affected in bulbar MND?

Answer 12

Speech and swallowing

Question 13

What is a key sign of a LMN lesion in bulbar palsy in MND?

Answer 13

Flaccid weak tongue with fasciculations (sack of worms)

Question 14

What is used to diagnose ALS?

Answer 14

El Escorial criteria

Question 15

How do you diagnose MND?

Answer 15

No diagnostic test - do other investigations to exclude other causes (brain/cord MRI and LP) - neurophysiology can detect subclinical denervation

Question 16

What is used to treat MND (ALS) to improve survival?

Answer 16

Riluzole

Question 17

How do you treat MND?

Answer 17

• Riluzole
• Symptomatic treatment
• Supportive (MDT) - augmentative and alternative communication, opioids and NIV (palliative)

Question 18

What symptomatic treatment is used in MND?

Answer 18

• Propantheline (antimuscarinic) or botulinum toxin A for excess saliva
• Gastrostomy for dysphagia
• Baclofen for spasticity