Motor Neuron disease

Question 1

What is the epidemiology of MND?

Answer 1

usually onset of 65yrs
avg survival 3 yrs
90% sporadic (50-75yrs)

Question 2

What are the different types of MND? What is most common?

Answer 2

Amytrophic lateral sclerosis - most common
primary lateral sclerosis
progressive muscular atrophy
progressive bulbar palsy

Question 3

What is the general presentation of MND?

Answer 3

UMN and LMN symptoms with NO SENSORY ISSUES

bulbar involvement - problems with speech, swallowing and breathing

Question 4

What is the presentation of Amytrophic lateral sclerosis?

Answer 4

UMN and LMN features - starts focal then spreads

Question 5

What is the presentation of Primary lateral sclerosis?

Answer 5

UMN features only

Question 6

What is the presentation of Progressive muscular atrophy?

Answer 6

LMN features, less prominant UMN features

Question 7

What is the prognosis of ALS?

Answer 7

3-5 years

Question 8

What is the prognosis of PLS?

Answer 8

> 5years

Question 9

What are upper motor neuron signs?

Answer 9

increased tone
hyper reflexia
extensor plantar response
spastic gait
exaggerated jaw jerk
slow movements

Question 10

What are lower motor neuron signs?

Answer 10

muscle wasting
weakness
fasiculations
absent/reduced deep tendon reflexes

Question 11

What is the pathophysiology behind MND?

Answer 11

very little known - motor neuron death/degeneration

Question 12

What investivations can be done for MND?

Answer 12

electrophysiology

El Escordial - diagnostic criteria

Question 13

What treatment/management is offered for MND?

Answer 13

OTs for communication/feeding needs
consider gastrostomy feeding tube
home ventilation
Riluzole - adds 3 months to live - can be given at the end
Baclofen for muscle cramps and spasms