Motor Neuron Disease

Question 1

Average survival time if you have MND

Answer 1

3 years

Question 2

Which cranial nerves are usually particularly affected in bulbar palsy?

Answer 2

• Glossopharyngeal
• Vagus
• Hypoglossal

Question 3

What age group are usually affected in bulbar palsy?

Answer 3

60-80 (women>men)

Question 4

Do you get numbness, tingling or pain in MND?

Answer 4

No, the sensory system is not involved

Question 5

Disease affecting UMNs only?

Answer 5

PLS

Question 6

Disease affecting LMNs only?

Answer 6

PMA, SMAs

Question 7

Disease affecting UMN and LMN?

Answer 7

ALS

Question 8

In what order are different parts of the body affected in PLS?

Answer 8

lower extremities –> trunk –> upper extremities –> bulbar muscles (kind of like bottom to top)

Question 9

Findings of ALS?

Answer 9

• Cramps are common but not specific

- Classic sign = a brisk reflex in a wasted muscle (i.e. lower and upper motor neurone signs)

Question 10

SMA

Answer 10

Spinal muscular atrophy

Question 11

PMA

Answer 11

Progressive muscular atrophy

Question 12

What is Kennedy’s disease?

Answer 12

• affects males
• LMNs only
• usually age 20-40
• X-linked

Early symptoms include tremor of the outstretched hand, muscle cramps with exertion and fasciculations (visible muscle twitching under the skin). Eventually pt develops limb weakness. Usually begins in shoulders or pelvic region
-Weakness of facial and tongue muscle follow

Question 13

Emotional and cognitive difficulties that may be present in some ALS patients?

Answer 13

• involuntary laughing or crying
• depression
• impaired executive function
• malaldaptive social behaviour

Question 14

What does bulbar palsy progress into?

Answer 14

Generalised ALS

Question 15

CK levels in MND?

Answer 15

Raised

Question 16

What is progressive muscular atrophy? (PMA)

Answer 16

Only affects LMNs

Question 17

TDP43 inclusions

Answer 17

Recognised as the pathological signature in MND in 95% of cases

Question 18

In UMN lesions-

In the upper limbs, are extensors or flexors usually weaker?

Answer 18

Extensors are weaker than flexors

Question 19

In UMN lesions-

In the lower limbs, are extensors or flexors usually weaker?

Answer 19

Flexors are usually weaker than extensors