Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Neurology Year 3 > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

What is the median survival upon onset of symptoms?

A

3 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

At what age is motor neurone disease likely to present?

A

50-75 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the epidemiology of MND?

A

90% sporadic

10% Familial link

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the four types of MND

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the most common subtype of MND?

A

ALS - Amyotrophic lateral sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How does ALS present?

A

With both Lower and Upper motor neurones features

Poor prognosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How does PLS present?

A

Upper motor neurone features

Good survival >5yrs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How does Progressive Muscular Atrophy present?

A

LMN features

30% have UMN features

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

UMN - Clinical signs

A 
Increased tone 
Hyper reflexa
Extensor Plantar Response
Spastic gait
Exaggerated Jaw jerk 
Slowed movement
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

LMN - Clinical signs

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What form of MND affects the lower cranial nerves?

A

progressive bulbar palsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Who is affected by progressive bulbar palsy?

A

F>M

60 - 80 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What muscles are affected by Progressive bulbar palsy?

A

Facial Tongue Pharyngeal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the main issues surrounding Progressive bulbar Palsy?

A

Dysphagia - risk of choking

Dysarthia - Can’t communicate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Why is it called progressive bulbar palsy?

A

As the disease always progresses to become ALS.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the treatments for Progressive Bulbar Palsy?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What common syndrome presents in ALS?

A

Split Hand syndrome - selective wasting of muscles within the hand

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What muscles show wasting in Split Hand syndrome?

A

First dorsal Interosseus

Abductor Pollicis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What muscles are preserved in Split Hand syndrome?

A

Abductor Digit minimi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What form of dementia is linked to ALS?

A

Frontotemporal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the diagnostic criteria for ALS?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the primary treatment for ALS?

A

Rilozole 50mg x2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Why is nutrition and hydration support in ALS so impotent?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is a clinical priority in regards to ALS?

A

Insertion of a Gastrostomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is sialorrhoea?

A

Excessive drooling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is the treatment for sialorrhoea?

A

Hyoscine Hydrobromide
Glycopyrronium
Botulin injection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

In ALS what is used to treat Muscle Cramps?

A

Baclofen

Quinine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

In ALS what is used to treat Muscle Spasms?

A

Baclofen
Gabapentin
Tizanidine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

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

A

Respiratory muscles fail.

30
Q

What is used to treat SOB in advanced ALS?

A

Lorazepam

31
Q

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

A

Non invasive CPAP during the night

Tracheostomy in advanced disease

32
Q

Emotionally how can people with MND present?

A

Apathetic
Disinhibition
Poor planning
Inappropriate laughing or crying

33
Q

Does MND result in cognitive impairment?

A

50% present with some dysfunction

34
Q

Hypokinetic

A

Too little moment

35
Q

What are some hypokinetic diseases?

A

Parkinsons

Akinetic rigid syndromes

36
Q

Hyperkinetic

A

Too much movement

37
Q

What are some hyperkinetic diseases?

A 
Tremor
Tics Chorea
Myoclonus
Dystonia
Athetosis
38
Q

What 4 things are linked to parkinsonism?

A

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

39
Q

What is rigidity?

A

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

40
Q

What is cogwheel rigidity?

A

Rigidity + Resting tremor

41
Q

Cogwheel rigidity is a sign off….

A

Parkinsonism

42
Q

Froments test is what?

A

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

43
Q

What is Akinesia?

A

Slow movement

44
Q

What movements are particularly affected by akinesia?

A

Repetitive or alternating movements

45
Q

When assessing akinesia what should be looked for?

A

Speed
Amplitude
Rhythm

46
Q

In Akinesia what does it appear like on examination?

A

Slow
Small
Decreasing rate

47
Q

When walking someone with postural or gait impairment will show.

A

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

48
Q

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

A

Stooped - some might show extreme anterior truncal flexion.

Impaired postural reflex - instability exhibited in pull test

49
Q

What is festination?

A

Small fast steps but very little movement

50
Q

Festination is characteristic of what?

A

Parkinsons

51
Q

What type of tremor occurs in Parkinsonism?

A

Rest

52
Q

What types of tremor are there?

A

Rest
Postural - arms outstretched
Kineti - occurs during movement

53
Q

What is dystonia?

A

Sustained or intermittent muscle contractions causing an abnormal repetitive movement

54
Q

Describe the movement in dystonia?

A

Patterned, twisting or tremulous

Initiated or worsened by voluntary movement

55
Q

What is chorea?

A

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

56
Q

List some causes of chorea.

A

Drugs - oral contraceptive
Basal ganglion lesion
Huntingtons

57
Q

What is ballism?

A

Variant of chorea

Proximal joints large amplitude flinging movements

58
Q

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

A

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

59
Q

What is myoclonus?

A

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

60
Q

What are normal variants of myoclonic contractions?

A

Hiccups or jerks whilst falling asleep.

61
Q

What are tics?

A

Semi voluntary or involuntary repetitive or stereotyped movements or vocalisations.

62
Q

What are Primary tics?

A

Develop in childhood

63
Q

What are secondary tics?

A

Onset in adulthood - v.rare

Due to secondary cause

64
Q

Can tics be suppressed?

A

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

65
Q

What is tourrettes?

A

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

66
Q

What is an essential tremor?

A

Benign postural tremor

67
Q

Describe the epidemiology of an essential tremor?

A

Inherited autosomal dominant with a high penetrance

Varying age of onset.

68
Q

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

A

As the issue doesn’t lie within the cerebellum.

69
Q

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

A

False it is usually asymmetrical

70
Q

What can cause a essential tremor to be acutely suppressed?

A

Alcohol

Neurology Year 3 (29 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions