Brainscape's Knowledge GenomeTM

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


See full index

Neurology > Motor neurone disease > Flashcards

Motor neurone disease Flashcards

1
Q

What is motor neurone disease

A

A devastating condition causing degeneration of the motor neurones, causing progressive weakness and death

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

What usually causes death in MND?

A

Respiratory failure or aspiration

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

Describe the aetiology of MND

A

50-75 y/o
ALS more common in non-Caucasian populations

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

What is the median survival after symptom onset in MND?

A

3 years

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

What causes MND?

A

The reason for it is unknown

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

What conditions are found under the term MND?

A

ALS
PLS
Progressive bulbar palsy
PMA

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

What are some benign variants of ALS?

A

Flail arm syndrome
Flail leg syndrome
(These will eventually progress into ALS)

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

What is the most common form of MND?

A

ALS - Amyotrophic Lateral Sclerosis

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

What does ALS stand for?

A

Amyotrophic Lateral Sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What does PLS stand for?

A

Primary Lateral Sclerosis

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

What does PMA stand for?

A

Progressive muscular atrophy

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

Describe the pattern of disease in ALS

A

Classic paraneoplastic presentation with simultaneous involvement of upper and lower motor neurones, usually in one limb, spreading gradually to other limbs and trunk muscles

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

What is split hand syndrome?

A

Preferential wasting of thenar group - a typical pattern of atrophy seen in ALS

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

Describe the pattern of disease in in progressive muscular atrophy (PMA)

A

Pure lower motor neurone presentation with weakness, muscle wasting and fasciculations, usually starting in one limb and gradually spreading to involve other adjacent spinal segments

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

Describe the pattern of disease in progressive bulbar and pseudo bulbar palsy

A

The lower cranial nerve nuclei and their supranuclear connections are initially involved, affecting the tongue, facial and pharyngeal muscles

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

How does progressive bulbar and pseudo palsy present?

A

Dysarthria
Dysphagia
Nasal regurgitation of fluids
Choking
Hypo/Hyper sialorrhoea
Wasting of the tongue and pharynx

17
Q

Describe the pattern of disease in primary lateral sclerosis (PLS)

A

Confined to upper motor neurones, causing a slowly progressive tetraparesis and pseudobulbar palsy

18
Q

What are some common presentations of MND?

A

Muscle weakness and possible speech and swallow problems
UMN and LMN signs without sensory signs
Focal onset and continuous spread

19
Q

What is MND catabolism?

A

Loss of weight in MND despite normal eating - Cause unknown

20
Q

What are some UMN signs of MND?

A
  • Weakness or paralysis
  • Spasticity
  • Increased muscle tone (Hypertonia)
  • Over-responsive reflexes (Hyperreflexia)
  • Emotional lability
  • Positive Babinski sign
21
Q

What is meant by a positive Babinski sign?

A

When the bottom of the foot is stroked, instead of the toes curling in, the big toe extends up and the other toes fan out

22
Q

Wha are some LMN signs of MND?

A
  • Weakness or paralysis
  • Decreased or absent muscle tone (Hypotonia or atonia)
  • Decreased or absent reflexes (Hyporeflexia or areflexia)
  • Involuntary muscle twitches (Fasiculations)
  • Muscle atrophy
23
Q

What are some red flag symptoms of respiratory failure in MND?

A
  • Breathlessness
  • Orthopnea
  • Recurrent chest infection
  • Disturbed sleep
  • Non-refreshed sleep
  • Nightmares
  • Daytime sleepiness
  • Poor concentration
24
Q

What is meant by emotional lability in UMN MND?

A

Inappropriate crying or laughing

25
Q

How is a diagnosis of MND made?

A

Mainly clinical diagnosis
Electrophysiology
Possible neuroimaging and lab studies

26
Q

What are some on-going management strategies required in MND?

A
  • Assessment and coordination of care
  • Communication - SALT, AAC
  • Nutrition - Gastrostomy, dieticians
  • Respiratory - Assessment, home ventilation
27
Q

What drug is the only drug licensed for MND?

A

Riluzole

28
Q

What is riluzole?

A

A sodium-channel blocker that inhibits glutamate release, giving patients an extra 3 months of life

29
Q

Why do most patients not choose to take riluzole?

A
  • This will give the patient an extra 3 months of life, however, this will be during the end of their life, when they are in their worst state
  • It also causes lethargy, sickness and possible cognitive impairment
30
Q

Symptomatic treatment - Sialorrhoea

A

Hyoscine/buscopan
Glycopyrronium
Botox
Suction

31
Q

Symptomatic treatment - Nutritional support

A

Supplements/thickeners
Liquid drug preparations

32
Q

Symptomatic treatment - Bulbar dysfunction

A

Early communicator
Nutritional support
Care for upper respiratory tract

33
Q

Symptomatic treatment - Limb dysfunction

A

Washing
Dressing
Feeding
Turning in bed
Mobility aids/hoists often necessary

34
Q

Symptomatic treatment - Muscle cramps

A

Quinine
Baclofen

35
Q

Symptomatic treatment - Muscle spasms

A

Baclofen
Tizanidine
Dantroline
Gabapentin

36
Q

Symptomatic treatment - Emotional lability

A

Sometimes treated with antidepressants

Neurology (28 decks)

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