Motor Neurone Disease

Question 1

UMN location

Answer 1

CNS

- brain and SC

Question 2

LMN location

Answer 2

PNS

- from spinal nerve roots and moving peripherally

Question 3

What is the positive babinski sign?

Answer 3

Plantars upgoing (extensor plantar response)

Question 4

UMN signs

• tone
• atrophy
• fasciculations
• reflexes
• plantars
• clonus

Answer 4

• tone = INCREASED
• atrophy = ABSENT
• fasciculations = ABSENT
• reflexes = BRISK
• plantars = UPGOING
• clonus = PRESENT

Question 5

LMN signs

• tone
• atrophy
• fasciculations
• reflexes
• plantars
• clonus

Answer 5

• tone = DECREASED (flaccid)
• atrophy = PRESENT EARLY
• fasciculations = PRESENT
• reflexes = DECREASED
• plantars = DOWNGOING
• clonus = ABSENT

Question 6

UMN pattern of weakness

Answer 6

Weak extensors in the arm

Weak flexors in the leg

Question 7

MND - definition

Answer 7

Rapidly progressive motor neuron degeneration

Variety of different subtypes

Question 8

Where does MND usually stare? the extremities/core ?

Answer 8

Extremities

e.g. lower or upper limb

Question 9

MND is usually a mixture of UMN and LMN abnormalities together with sensory disturbance. True or false?

Answer 9

False

- there is NO sensory abnormalities in MND

Question 10

MND - genetic component

Answer 10

TDP-43

Question 11

MND - there is a spectrum of different clinical phenotypes with MND at one end of the spectrum and ___ at the other end of the spectrum

Answer 11

Fronto-temporal dementia (FTD)

Question 12

Classic MND

Answer 12

Amyotrophic lateral sclerosis (ALS)

Question 13

Classic MND (ALS) - diagnostic criteria

Answer 13

UMN + LMN signs in bulbar and at least 2 of the following spinal regions:

• cervical
• thoracic
• lumbosacral

Question 14

MND - primary bulbar onset always generalises into amyotrophic lateral sclerosis. True or false?

Answer 14

True

Question 15

MND - management of bulbar symptoms

Answer 15

Nutritional support

SALT

Question 16

MND - respiratory onset is common. True or false?

Answer 16

False

- rare

Question 17

MND - clinical features (broad)

Answer 17

UMN and/or LMN signs with NO sensory problems

There may be a link with cognitive impairment 
Muscle weakness 
Speech problems 
Swallowing difficulty 
Breathing problems

Question 18

MND - pattern of muscle weakness

Answer 18

Usually focal onset and then continuous spread.

Usually starts at extremities

Question 19

What is spasticity and is it a UMN or LMN sign?

Answer 19

UMN sign

Increased tone

Question 20

What is split hand syndrome?

Answer 20

Typical pattern of atrophy where there is preferential wasting of thinner group

Question 21

Which presents with more weakness, UMN or LMN dysfunction?

Answer 21

LMN dysfunction

Question 22

What is the role of imaging in MND?

Answer 22

To rule out other (potentially curable) diseases

Question 23

MND is incurable. True or false?

Answer 23

True

Question 24

MND - management

Answer 24

There is no treatment

Question 25

Which type of MN presents with UMN signs only?

Answer 25

Primary lateral sclerosis

Question 26

Which type of MND is likely to present with early complications such as pneumonia?

Answer 26

Progressive bulbar palsy

Question 27

Which type of dementia is MND associated with?

Answer 27

Fronto-temporal dementia

Question 28

Which type of MND presents with LMN signs only in distal muscles?

Answer 28

Progressive muscular atrophy

Question 29

Which type of MND presents with LMN signs in the arms and UMN signs in the legs?

Answer 29

Amyotrophic lateral sclerosis