Motor neurone disease Flashcards Preview

Neurology > Motor neurone disease > Flashcards

Flashcards in Motor neurone disease Deck (34)
1

Would you expect brisk or slow reflexes in MND?

Brisk

2

What is motor neurone disease also called?

Amyotrophic lateral sclerolsis

3

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

Both

4

What type of dementia is associated with MND?

Fronto temporal dementia

5

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

Upper

6

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

Lower

7

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

Lower; bulbar regions

8

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

Upper; bulbar region

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.

Motor neurone disease

10

How does progressive muscular atrophy present?

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.

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?

Progressive bulbar/pseudobulbar palsy

12

What nerves does progressive bulbar palsy affect?

Cranial nerves only

13

What is the average survival time in MND?

3 years

14

Where is the most common starting point for MND?

Extremities (more often the upper limbs)

15

Does bulbar onset MND remain confined to the bulbar region?

No. It will always generalize into ALS

16

What is primary lateral sclerosis?

Progressive muscle weakness in vouluntary muscles

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.

Kennedys disease

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.

Primary lateral sclerosis

19

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

Fronto - temporal dementia

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

Fronto temporal dementia

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.

Fronto temporal dementia and motor neurone disease

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.

Progressive muscular atrophy

23

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

Flail arm syndrome

24

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

Flail leg syndrome

25

Is spasticity an upper or lower motor neurone sign?

Upper

26

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

Upper

27

Is hyperreflexia an upper or lower motor neurone sign?

Upper

28

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

Upper

29

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

Upper

30

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

Lower (moderate weakness seen in upper motor neurone problems)

31

Is hyporeflexia seen in upper or lower motor neurone problems?

Lower

32

Are fasiculations seen in upper or lower motor neurone problems?

Lower

33

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

Lower

34

What is the average age of diagnosis in MND?

65