Motor Neurone Disease Flashcards Preview

Neurology Year 3 > Motor Neurone Disease > Flashcards

Flashcards in Motor Neurone Disease Deck (70)
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1

What is the median survival upon onset of symptoms?

3 years

2

At what age is motor neurone disease likely to present?

50-75 years

3

What is the epidemiology of MND?

90% sporadic
10% Familial link

4

What are the four types of MND

Amyotrophic Lateral Sclerosis
Primary Lateral Sclerosis
Progressive Muscular Atrophy
Progressive Bulbar Palsy

5

What is the most common subtype of MND?

ALS - Amyotrophic lateral sclerosis

6

How does ALS present?

With both Lower and Upper motor neurones features
Poor prognosis

7

How does PLS present?

Upper motor neurone features
Good survival >5yrs

8

How does Progressive Muscular Atrophy present?

LMN features
30% have UMN features

9

UMN - Clinical signs

Increased tone
Hyper reflexa
Extensor Plantar Response
Spastic gait
Exaggerated Jaw jerk
Slowed movement

10

LMN - Clinical signs

Muscle wasting
Weakness
Fasciculations(spontaneous muscle twitch)
Absent or reduced tendon reflexs

11

What form of MND affects the lower cranial nerves?

progressive bulbar palsy

12

Who is affected by progressive bulbar palsy?

F>M
60 - 80 years

13

What muscles are affected by Progressive bulbar palsy?

Facial Tongue Pharyngeal

14

What are the main issues surrounding Progressive bulbar Palsy?

Dysphagia - risk of choking
Dysarthia - Can't communicate

15

Why is it called progressive bulbar palsy?

As the disease always progresses to become ALS.

16

What are the treatments for Progressive Bulbar Palsy?

Therapeutic - communication devise, nutritional support, care for the URT.

17

What common syndrome presents in ALS?

Split Hand syndrome - selective wasting of muscles within the hand

18

What muscles show wasting in Split Hand syndrome?

First dorsal Interosseus
Abductor Pollicis

19

What muscles are preserved in Split Hand syndrome?

Abductor Digit minimi

20

What form of dementia is linked to ALS?

Frontotemporal

21

What are the diagnostic criteria for ALS?

Signs of UMN LMN degeneration
Electrophysiological and Neuropathological investigation
Signs in 2/3 vertebral segments on MRI

22

What is the primary treatment for ALS?

Rilozole 50mg x2

23

Why is nutrition and hydration support in ALS so impotent?

Metabolic rate is doubled and weight loss indicates a very poor prognosis.

24

What is a clinical priority in regards to ALS?

Insertion of a Gastrostomy

25

What is sialorrhoea?

Excessive drooling

26

What is the treatment for sialorrhoea?

Hyoscine Hydrobromide
Glycopyrronium
Botulin injection

27

In ALS what is used to treat Muscle Cramps?

Baclofen
Quinine

28

In ALS what is used to treat Muscle Spasms?

Baclofen
Gabapentin
Tizanidine

29

Why is respiratory dysfunction one of the main causes of death in ALS?

Respiratory muscles fail.

30

What is used to treat SOB in advanced ALS?

Lorazepam