Motor Neurone Disease

Question 1

What is the median survival upon onset of symptoms?

Answer 1

3 years

Question 2

At what age is motor neurone disease likely to present?

Answer 2

50-75 years

Question 3

What is the epidemiology of MND?

Answer 3

90% sporadic

10% Familial link

Question 4

What are the four types of MND

Answer 4

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

Question 5

What is the most common subtype of MND?

Answer 5

ALS - Amyotrophic lateral sclerosis

Question 6

How does ALS present?

Answer 6

With both Lower and Upper motor neurones features

Poor prognosis

Question 7

How does PLS present?

Answer 7

Upper motor neurone features

Good survival >5yrs

Question 8

How does Progressive Muscular Atrophy present?

Answer 8

LMN features

30% have UMN features

Question 9

UMN - Clinical signs

Answer 9

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

Question 10

LMN - Clinical signs

Answer 10

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

Question 11

What form of MND affects the lower cranial nerves?

Answer 11

progressive bulbar palsy

Question 12

Who is affected by progressive bulbar palsy?

Answer 12

F>M

60 - 80 years

Question 13

What muscles are affected by Progressive bulbar palsy?

Answer 13

Facial Tongue Pharyngeal

Question 14

What are the main issues surrounding Progressive bulbar Palsy?

Answer 14

Dysphagia - risk of choking

Dysarthia - Can’t communicate

Question 15

Why is it called progressive bulbar palsy?

Answer 15

As the disease always progresses to become ALS.

Question 16

What are the treatments for Progressive Bulbar Palsy?

Answer 16

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

Question 17

What common syndrome presents in ALS?

Answer 17

Split Hand syndrome - selective wasting of muscles within the hand

Question 18

What muscles show wasting in Split Hand syndrome?

Answer 18

First dorsal Interosseus

Abductor Pollicis

Question 19

What muscles are preserved in Split Hand syndrome?

Answer 19

Abductor Digit minimi

Question 20

What form of dementia is linked to ALS?

Answer 20

Frontotemporal

Question 21

What are the diagnostic criteria for ALS?

Answer 21

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

Question 22

What is the primary treatment for ALS?

Answer 22

Rilozole 50mg x2

Question 23

Why is nutrition and hydration support in ALS so impotent?

Answer 23

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

Question 24

What is a clinical priority in regards to ALS?

Answer 24

Insertion of a Gastrostomy

Question 25

What is sialorrhoea?

Answer 25

Excessive drooling

Question 26

What is the treatment for sialorrhoea?

Answer 26

Hyoscine Hydrobromide Glycopyrronium Botulin injection

Question 27

In ALS what is used to treat Muscle Cramps?

Answer 27

Baclofen | Quinine

Question 28

In ALS what is used to treat Muscle Spasms?

Answer 28

Baclofen Gabapentin Tizanidine

Question 29

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

Answer 29

Respiratory muscles fail.

Question 30

What is used to treat SOB in advanced ALS?

Answer 30

Lorazepam

Question 31

What is key to the treatment of respiratory dysfunction in ALS?

Answer 31

Non invasive CPAP during the night | Tracheostomy in advanced disease

Question 32

Emotionally how can people with MND present?

Answer 32

Apathetic Disinhibition Poor planning Inappropriate laughing or crying

Question 33

Does MND result in cognitive impairment?

Answer 33

50% present with some dysfunction

Question 34

Hypokinetic

Answer 34

Too little moment

Question 35

What are some hypokinetic diseases?

Answer 35

Parkinsons | Akinetic rigid syndromes

Question 36

Hyperkinetic

Answer 36

Too much movement

Question 37

What are some hyperkinetic diseases?

Answer 37

``` Tremor Tics Chorea Myoclonus Dystonia Athetosis ```

Question 38

What 4 things are linked to parkinsonism?

Answer 38

T - Tremor R - Rigidity A - Akinesia / Bradykinesia P - Postural instability

Question 39

What is rigidity?

Answer 39

Resistance to movement is felt throughout the full range of movement. No increase with higher mobilising speed.

Question 40

What is cogwheel rigidity?

Answer 40

Rigidity + Resting tremor

Question 41

Cogwheel rigidity is a sign off....

Answer 41

Parkinsonism

Question 42

Froments test is what?

Answer 42

To feel increased rigidity on one sign move the contralateral side whilst examining the same side.

Question 43

What is Akinesia?

Answer 43

Slow movement

Question 44

What movements are particularly affected by akinesia?

Answer 44

Repetitive or alternating movements

Question 45

When assessing akinesia what should be looked for?

Answer 45

Speed Amplitude Rhythm

Question 46

In Akinesia what does it appear like on examination?

Answer 46

Slow Small Decreasing rate

Question 47

When walking someone with postural or gait impairment will show.

Answer 47

Slow short shuffling steps Freezing and start hesitation when turning or starting Decreased arm swing

Question 48

On standing how might someone with postural impairment will show this.

Answer 48

Stooped - some might show extreme anterior truncal flexion. | Impaired postural reflex - instability exhibited in pull test

Question 49

What is festination?

Answer 49

Small fast steps but very little movement

Question 50

Festination is characteristic of what?

Answer 50

Parkinsons

Question 51

What type of tremor occurs in Parkinsonism?

Answer 51

Rest

Question 52

What types of tremor are there?

Answer 52

Rest Postural - arms outstretched Kineti - occurs during movement

Question 53

What is dystonia?

Answer 53

Sustained or intermittent muscle contractions causing an abnormal repetitive movement

Question 54

Describe the movement in dystonia?

Answer 54

Patterned, twisting or tremulous | Initiated or worsened by voluntary movement

Question 55

What is chorea?

Answer 55

Brief irregular purposeless movements that flow from one side of the body to another. Chorea = Dance

Question 56

List some causes of chorea.

Answer 56

Drugs - oral contraceptive Basal ganglion lesion Huntingtons

Question 57

What is ballism?

Answer 57

Variant of chorea | Proximal joints large amplitude flinging movements

Question 58

Describe the location of ballism movement in relation to the lesion.

Answer 58

It is usually hemiplegic and occurring on the contralateral side to the lesion.

Question 59

What is myoclonus?

Answer 59

Brief electrical shock like jerks resulting in an activation of a group of muscles.

Question 60

What are normal variants of myoclonic contractions?

Answer 60

Hiccups or jerks whilst falling asleep.

Question 61

What are tics?

Answer 61

Semi voluntary or involuntary repetitive or stereotyped movements or vocalisations.

Question 62

What are Primary tics?

Answer 62

Develop in childhood

Question 63

What are secondary tics?

Answer 63

Onset in adulthood - v.rare | Due to secondary cause

Question 64

Can tics be suppressed?

Answer 64

Yes, usually uncomfortable to do so and is usually followed upon release by a flurry.

Question 65

What is tourrettes?

Answer 65

Persistent motor and vocal tics + associated psychiatric disturbances ADHD OCD etc

Question 66

What is an essential tremor?

Answer 66

Benign postural tremor

Question 67

Describe the epidemiology of an essential tremor?

Answer 67

Inherited autosomal dominant with a high penetrance | Varying age of onset.

Question 68

Why does an essential tremor not get worse with proximity to its target?

Answer 68

As the issue doesn't lie within the cerebellum.

Question 69

Onset of an essential tremor is usually symmetrical. T/F

Answer 69

False it is usually asymmetrical

Question 70

What can cause a essential tremor to be acutely suppressed?

Answer 70

Alcohol