MND

Question 1

What is MND?

Answer 1

Neurodegenerative disorder of upper and lower motor neurone, characterised by progressive weakness regionally, then generalises and progresses relentlessly

Question 2

Where is the pathology in MND?

Answer 2

Anterior horn cells

Question 3

What are the 4 maintypes of MND?

Answer 3

Amytrophic lateral sclerosis- usually LMN in arm and UMN in legs
Progressive muscular atrophy- LMN
Primary lateral sclerosis- UMN
Progressive Bulbar palsy- los of function of brainstem motor nuclei

Question 4

What type of MND has the best prognosis?

Answer 4

Progressive muscular atrophy= best

Bulbar palsy=worst

Question 5

What is the most common type pf MND?

Answer 5

ALS

Question 6

What is the general presentation of MND?

Answer 6

Mixed upper and lower motor neurone signs
No sensory involvement
Pain

Question 7

What are the physical signs of MND?

Answer 7

Mixed U and LMN
Weak, spastic, wasted muscles
Fasciculations and spasms
Unsteady gait
Hyperreflexia
Strained, slow speech
Generally spares extra ocular muscles and sphincters

Question 8

Is there cognitive/behaviour change in MND?

Answer 8

in ~50%

Question 9

What investigations are done for MND?

Answer 9

EMG- normal motor conduction= diagnostic

MRI- exclude other causes

Question 10

What is the management of MND?

Answer 10

Supportive care- NIV, PEG, physio

Question 11

What is the management of ALS?

Answer 11

Supportive care

Riluzole- prolongs life by ~3 months

Question 12

What conditions is MND associated with?

Answer 12

Frontotemporal dementia