7: Motor neuron disease

Question 1

What is the most common type of motor neuron disease in adults?

What is its alternate name?

Answer 1

Amyotrophic lateral sclerosis

or Lou Gehrig’s disease

Question 2

Which disease, causing progressive cognitive decline, is on the spectrum of motor neuron disease?

Answer 2

Frontotemporal dementia

Question 3

Frontotemporal dementia is related to ___ ___ ___.

Answer 3

motor neuron disease

Question 4

Where do MND symptoms tend to start?

Answer 4

Limbs

e.g foot drop

Question 5

What are the bulbar muscles responsible for?

Answer 5

Swallowing

Speaking

Chewing

Question 6

Which muscles, if affected by MND, cause difficulty swallowing, speaking and chewing?

Answer 6

Bulbar muscles

Question 7

Which protein is implicated in MND?

Answer 7

TDP-43

Question 8

What percentage of MND diseases are

a) spontaneous
b) inherited?

Answer 8

a) 90%

b) 10%

Question 9

MND is a neuro___ condition.

Answer 9

neurodegenerative disease

Question 10

What are the symptoms of MND?

Answer 10

Muscle weakness

Difficulty speaking, breathing and swallowing

Sensory loss is usually ABSENT

Question 11

Which group of signs are seen in MND?

Answer 11

UMN and LMN signs

Question 12

What is the course of MND?

Answer 12

Rapidly progressive

starts in a distal limb or bulbar muscles and continually spreads

Question 13

What is the most common type of MND?

Answer 13

Amyotrophic lateral sclerosis (ALS)

ice bucket challenge

Question 14

What is the prognosis of newly diagnosed MND?

Answer 14

2 - 3 years to live

Question 15

MND is more common in (males / females).

Answer 15

males

Question 16

Describe the signs of upper motor neuron disease.

Answer 16

Hypertonia

Hyperreflexia

Upgoing plantars

No fasciculations

No atrophy

Question 17

Describe the signs of lower motor neuron disease.

Answer 17

Hypotonia

Slow / absent reflexes

Downgoing plantars

Fasciculations

Atrophy

Question 18

How can you elicit bulbar UMN signs?

Answer 18

Jaw jerk

may be brisk in bulbar MND

Question 19

Which distal muscles waste especially in MND?

Answer 19

Thenar muscles

first interosseous muscle on dorsal surface

Question 20

When a doctor sees thenar atrophy, what do they often misdiagnose the patient with?

Answer 20

Carpal tunnel syndrome

Question 21

Patients with MND often develop a cognitive deficit.

Why?

Which protein is thought to be involved?

Answer 21

Association with frontotemporal dementia

TDP-43

Question 22

How are patients with bulbar MND managed?

Answer 22

Refer to SLT and occupational therapy

Consider feeding tube, oxygen as disease progresses

Question 23

What is the prognosis of bulbar-onset MND compared to ALS?

Answer 23

Poor

because important muscles are affected sooner

Question 24

Which clinical signs are diagnostic of MND?

Answer 24

Upper motor neuron signs

Lower motor neuron signs

Bulbar onset

Cognitive decline

Question 25

Before diagnosing MND, it is important to ___ mimic diseases.

Answer 25

eliminate mimic diseases

using an EEG, imaging etc.

Question 26

Which investigation can be used to examine muscles for the degeneration seen in MND?

Answer 26

EMG

Question 27

How is MND investigated and diagnosed?

Answer 27

Largely a clinical diagnosis

Any investigations are to rule out other diseases

Question 28

What is the treatment for MND?

Answer 28

Supportive

No treatment exists

Question 29

Variants of ALS tend to have a (better / worse) prognosis.

Answer 29

better prognosis

Question 30

Which disease, causing progressive cognitive decline, is associated with MND?

Answer 30

Frontotemporal dementia