Motor Neurone Disease

Question 1

define MND

Answer 1

disease of the UMNs and LMNs WITH NO sensory signs

Question 2

what condition is MND linked to?

Answer 2

frontotemporal dementia

Question 3

most common region of onset in MND?

Answer 3

spinal/limb onset

Question 4

a patient presents with frontotemporal dementia and no motor signs, what should be considered?

Answer 4

do they have MND?

Question 5

main protein affected in MND?

Answer 5

TDP-43

Question 6

what lobe is responsible for memory

Answer 6

temporal

Question 7

frontal lobe is for…

Answer 7

executive function
making lists
behaviour

Question 8

alpha synuclein in lewy bodies is the classic presentation of what disease

Answer 8

parkinsons

Question 9

the ____ extremities are more commonly affected in MND

Answer 9

upper

Question 10

__ motor neuron signs are more common in MND

Answer 10

lower

Question 11

most common subtype of MND

Answer 11

amyotrophic lateral sclerosis (ALS)

Question 12

cognitive impairment is a feature of MND T or F

Answer 12

T

Question 13

sporadic MND peaks at the ages of __ - __ years and declines after the age of __

Answer 13

50-75

80

Question 14

average survival time of MND is _ years

Answer 14

3

Question 15

is MND genetic?

Answer 15

yes but most cases are sporadic

Question 16

presentation of MND

Answer 16

muscle weakness and wasting

speech/swallowing/breathing problems

Question 17

relevant FH questions in MND?

Answer 17

family with MND or FT dementia?

Question 18

babinski sign is a/an __ motor neurone sign

Answer 18

upper

Question 19

fasciculations are a/an ___ motor neurone sign

Answer 19

lower

Question 20

fasciculations are a sign of MND T or F

Answer 20

T

Question 21

muscle cramps are a/an __ MN sign

Answer 21

lower

Question 22

typical pattern of atrophy seen in MND?

Answer 22

preferential wasting of the thenar muscles

hypothenar muscles spared

Question 23

name the 3 different possible sites of onset of MND

Answer 23

extremities
bulbar
thoracic

Question 24

who gets bulbar onset MND

Answer 24

women in their 60s-80s

Question 25

diagnostic criteria for MND

Answer 25

signs of LMN and UMN signs in bulbar +/- spinal regions without sensory loss with spread to other regions

Question 26

Ix for MND

Answer 26

clinical
EMG
brain imaging

Question 27

what is the bulbar region of the brain?

Answer 27

brainstem + cerebellum (without the midbrain)

Question 28

thoracic MND can present with __ only

Answer 28

dyspnoea

Question 29

false diagnoses of MND?

Answer 29

carpal tunnel
stroke
neuropathy

Question 30

Tx for MND

Answer 30

supportive