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Neurology Week 5 2018/19 > 7: Motor neuron disease > Flashcards

Flashcards in 7: Motor neuron disease Deck (30)
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1

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

What is its alternate name?

Amyotrophic lateral sclerosis

or Lou Gehrig's disease

2

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

Frontotemporal dementia

3

Frontotemporal dementia is related to ___ ___ ___.

motor neuron disease

4

Where do MND symptoms tend to start?

Limbs

e.g foot drop

5

What are the bulbar muscles responsible for?

Swallowing

Speaking

Chewing

6

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

Bulbar muscles

7

Which protein is implicated in MND?

TDP-43

8

What percentage of MND diseases are 

a) spontaneous

b) inherited?

a) 90%

b) 10%

9

MND is a neuro___ condition.

neurodegenerative disease

10

What are the symptoms of MND?

Muscle weakness

Difficulty speaking, breathing and swallowing

Sensory loss is usually ABSENT

11

Which group of signs are seen in MND?

UMN and LMN signs

12

What is the course of MND?

Rapidly progressive

starts in a distal limb or bulbar muscles and continually spreads

13

What is the most common type of MND?

Amyotrophic lateral sclerosis (ALS)

ice bucket challenge

14

What is the prognosis of newly diagnosed MND?

2 - 3 years to live

15

MND is more common in (males / females).

males

16

Describe the signs of upper motor neuron disease.

Hypertonia

Hyperreflexia

Upgoing plantars

No fasciculations

No atrophy

17

Describe the signs of lower motor neuron disease.

Hypotonia

Slow / absent reflexes

Downgoing plantars

Fasciculations

Atrophy

18

How can you elicit bulbar UMN signs?

Jaw jerk

may be brisk in bulbar MND

19

Which distal muscles waste especially in MND?

Thenar muscles

first interosseous muscle on dorsal surface

20

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

Carpal tunnel syndrome

21

Patients with MND often develop a cognitive deficit.

Why?

Which protein is thought to be involved?

Association with frontotemporal dementia

TDP-43

22

How are patients with bulbar MND managed?

Refer to SLT and occupational therapy

Consider feeding tube, oxygen as disease progresses

23

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

Poor

because important muscles are affected sooner

24

Which clinical signs are diagnostic of MND?

Upper motor neuron signs

Lower motor neuron signs

Bulbar onset

Cognitive decline

25

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

eliminate mimic diseases

using an EEG, imaging etc.

26

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

EMG

27

How is MND investigated and diagnosed?

Largely a clinical diagnosis

Any investigations are to rule out other diseases

28

What is the treatment for MND?

Supportive

No treatment exists

29

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

better prognosis

30

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

Frontotemporal dementia