Motor neurone disease

Question 1

Would you expect brisk or slow reflexes in MND?

Answer 1

Brisk

Question 2

What is motor neurone disease also called?

Answer 2

Amyotrophic lateral sclerolsis

Question 3

Is ALS a disease of upper motor neurones, lower motor neurones or both?

Answer 3

Both

Question 4

What type of dementia is associated with MND?

Answer 4

Fronto temporal dementia

Question 5

Is primary lateral sclerosis a disease of upper motor neurones, lower motor neurones or both?

Answer 5

Upper

Question 6

is progressive muscular atrophy a disease of upper motor neurones, lower motor neurones or both?

Answer 6

Lower

Question 7

In progressive bulbar palsy a disease of upper motor neurones, lower motor neurones or both?

Answer 7

Lower; bulbar regions

Question 8

Is pseudobulbar palsy a disease of upper motor neurones, lower motor neurones or both?

Answer 8

Upper; bulbar region

Question 9

A 76 year old man presents with weakness. he said it first started in his left hand but now he is struggling to move his right hand. His wife reports that he has been acting strangely lately; becoming more rude than usual. One examination he has brisk reflexes and muscle wasting in his hands.

Answer 9

Motor neurone disease

Question 10

How does progressive muscular atrophy present?

Answer 10

Since it is a pure lower motor neurone problem it presents with
- Weakness
- Wasting
- Fasiculations
Usually starting in one limb and then moving to adjacent spinal segments.

Question 11

A 65 year old lady presents with difficulty swallowing. She also seems to be having problems speaking. What type of MND could this be?

Answer 11

Progressive bulbar/pseudobulbar palsy

Question 12

What nerves does progressive bulbar palsy affect?

Answer 12

Cranial nerves only

Question 13

What is the average survival time in MND?

Answer 13

3 years

Question 14

Where is the most common starting point for MND?

Answer 14

Extremities (more often the upper limbs)

Question 15

Does bulbar onset MND remain confined to the bulbar region?

Answer 15

No. It will always generalize into ALS

Question 16

What is primary lateral sclerosis?

Answer 16

Progressive muscle weakness in vouluntary muscles

Question 17

A rare neuromuscular disorder that causes progressive weakening and wasting of the muscles in the arms and legs in particular. It only affects males and is slowly progressive meaning life expectancy is near normal.

Answer 17

Kennedys disease

Question 18

A rare neuromuscular disease that causes progressive muscle weakness in voluntary muscles. It only affects upper motor neurones and there is no degeneration so spinal motor neurones or muscle wasting. Patients will also have spasticity and hyperreflexia. Life expectancy is near normal.

Answer 18

Primary lateral sclerosis

Question 19

What other neurological disease is often seen with motor neurone disease?

Answer 19

Fronto - temporal dementia

Question 20

A lady presents with her husband. He says she has started showing some behavioural abnormalities: dysinhibition, decreased impulse control, increased food intake, weight gain, socially inadequate, positive mood, no insight

Answer 20

Fronto temporal dementia

Question 21

18 months prior to onset of motor symptoms behavioural abnormalities: dysinhibition, decreased impulse control, increased food intake, weight gain, socially inadequate, positive mood, no insight. She has now developed right thigh weakness and a left sided foot drop.

Answer 21

Fronto temporal dementia and motor neurone disease

Question 22

A rare type of motor neurone disease that only affects the lower motor neuorones. Symptoms are usually of atrophy, fasiculations and muscle weakness. No spasticity or hyperreflexia.

Answer 22

Progressive muscular atrophy

Question 23

A rare subtype of MND is which you get atrophy, fasiculation and muscle weakness in the arms only

Answer 23

Flail arm syndrome

Question 24

A rare subtype of MND is which you get atrophy, fasiculation and muscle weakness in the legs only

Answer 24

Flail leg syndrome

Question 25

Is spasticity an upper or lower motor neurone sign?

Answer 25

Upper

Question 26

Is the babinski sign an upper or lower motor neurone sign?

Answer 26

Upper

Question 27

Is hyperreflexia an upper or lower motor neurone sign?

Answer 27

Upper

Question 28

Is a clonic jaw jerk an upper or lower motor neurone sign?

Answer 28

Upper

Question 29

Are pseudobulbar signs due to upper or lower motor neurone problems?

Answer 29

Upper

Question 30

Is severe muscle weakness an upper or lower motor neurone sign?

Answer 30

Lower (moderate weakness seen in upper motor neurone problems)

Question 31

Is hyporeflexia seen in upper or lower motor neurone problems?

Answer 31

Lower

Question 32

Are fasiculations seen in upper or lower motor neurone problems?

Answer 32

Lower

Question 33

Is muscle hypotonicity seen in upper or lower motor neurone problems?

Answer 33

Lower

Question 34

What is the average age of diagnosis in MND?

Answer 34

65