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Neurology > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

What is motor neuron disease

A

An untreatable and rapidly progressive neurodegenerative condition affecting the motor nerves
Affects both the upper and lower motor neurons

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2
Q

What is the most common subtype of MND

A

Amyotrophic lateral sclerosis

ALS

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3
Q

What are the symptoms of MND

A

Muscle weakness - focal onset but will spread continuously until generalised paresis
Problems with speech, swallowing and breathing
Cognitive impairment and emotional changes

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4
Q

Does MND cause sensory issues

A

NO

Its a motor disease

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5
Q

What causes MND

A

Motor neurone degeneration or death

90% of cases are sporadic
The rest are familial cases
Some genetic aetiology but as yet unknown

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6
Q

At what age does sporadic MND usually present

A

Between 50-75

Most are in mid 60s

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7
Q

Which ethnic group is most affected by ALS

A

Caucasians

Much less common in non-Caucasian populations

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8
Q

What is the median survival after a diagnosis of MND

A

3 years

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9
Q

Is MND becoming more or less common

A

More common

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10
Q

How many new cases of MND are there in Scotland per year

A

200 (approx.)

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11
Q

Which sex is more affected by MND

A

Both sexes are affected

Slightly more common in men

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12
Q

What is the main way to classify MND

A

Classified by the part of the nerve pathway that is primarily affected
Primary lateral sclerosis – UMN disease
ALS - mixed UMN and LMN
Progressive muscular atrophies - LMN disease

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13
Q

How can you classify MND

A

UMN vs LMN
Motor disease or extramotor (frontotemporal dementia symptoms)
Region of onset
Based on genes - not usually done

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14
Q

Is MND a single illness

A

NOPE
Its an umbrella term that includes all types - ALS, PLS, PMA, PBP
All are different and have different outcomes

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15
Q

Which subtype of MND kills quickly

A

ALS
Mixed UMN and LMN signs
Particularly the bulbar subtype of ALS as heavy bulbar and respiratory involvement
Can also have Pick’s alongside it

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16
Q

What is progressive muscular atrophy

A

LMN variant of MND
Therefore present with purely the peripheral component, without the UMN issues (e.g. no spasticity, brisk reflexes etc)

Has a variable prognosis and can last for years
Patients will flail their arms and legs
Makes up 10% of cases

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17
Q

What is primary lateral sclerosis

A

UMN variant of MND
Good survival of over 5 years
Only seen in 1-3% of cases

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18
Q

What are the upper motor neuron signs in MND

A 
Increased tone - spasticity 
Pyramidal weakness
Hyperreflexia 
Extensor plantar response 
Spastic gait 
Exaggerated jaw jerk 
Slowed movements 
Pseudobulbar effects - speech and swallowing issue
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19
Q

What are the lower motor neuron signs in MND

A 
Muscle wasting 
Weakness 
Fasciculations 
Absent or reduced deep tendon reflexes 
Muscle cramps
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20
Q

What are the bulbar symptoms of MND

A

Problems with speech and/or swallowing
Hypersalivation
Hard to compensate for
Seen in 1/3 of MND patients

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21
Q

What is the most common symptoms that patients present with

A

Limb impairment is the most common

Followed by bulbar symptoms - speech/swallowing

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22
Q

How can MND affect weight

A

It makes people lose weight

They become cachectic and wasted and can’t maintain body weight

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23
Q

Are upper or lower limbs more commonly affected in MND

A

Upper

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24
Q

Who is more affected by the bulbar variant of MND

A

Women

25
Q

How does the bulbar variant of MND present

A

Problems with speech - will eventually struggle to communicate
Struggle with eating - swallowing difficulty
Get wasted, fasciculating tongue
Will also cause wasting of the facial muscles and the pharynx is effected (affects speech)
Has the worst prognosis

26
Q

How do you manage bulbar variant MND

A

Early communication devices (voice banking etc)
Nutritional support - PEG tube
May get a gastrostomy
Respiratory tract care - know how to spot a chest infection and deal with choking)
Non-invasive ventialtion like BiPAP for respiratory symptoms

27
Q

What type of MND is diagnosed if the patient first presents with bulbar issues

A

ALS

The bulbar variant of MND is a subtype of ALS

28
Q

What is often the cause of death in MND

A

Respiratory complications such as choking or aspiration pneumonia
Disease makes them weak and malnourished so struggle to fight of chest infections
Weakness of respiratory muscles

29
Q

What is split hand syndrome

A 
A pattern of hand muscle wasting seen in ALS 
The thenar (thumb) muscles are wasted but the others are fine
30
Q

What symptoms of MND are seen in the upper and lower limbs

A

Muscle wasting
Loss of tone and contractures
Can lose function - if you loose hand function you are completely dependant

31
Q

How do the upper and lower limb symptoms of MND affect day to day life

A

Affects washing, dressing, feeding, turning in bed
Become dependent on others for these tasks
They often need mobility aids and hoists - lots of OT involvement

32
Q

How can you stop contractures forming

A

Lots and lots of physio

33
Q

In which type of MND is Pick’s/FTD common

A

Familial ALS

34
Q

Which subtypes of MND have the most benign prognosis

A

Flail arm syndrome
Flail leg syndrome
Primary Lateral Sclerosis - UMN variant

35
Q

What is the main investigation for MND

A

Neuro/Electrophysiology

EMG - looks at how well the muscle is innervated by the nerve
In MND it will show denervation of the nerve but also renervation of surrounding nerves trying to sprout out and compensate

36
Q

How do you diagnose MND

A

It is a clinical diagnosis - most tests are to exclude mimics
Lack of evidence of other disease processes that could explain symptoms - diagnosis of exclusion

MRI to exclude structural cause
CK - mildly raised in MND but very high suggests a muscle pathology

Symptoms of LMN or UMN degeneration on examination or electrophysiology are supportive of diagnosis
Progressive spread of signs

37
Q

Can you get a false positive diagnosis of MND

A

Yes - as its a clinical diagnosis some will get diagnosed with MND when in fact its just a mimic
False positives including multifocal motor neuropathy, Kennedys disease, myopathy (inclusion body), cervical spondylitic radiculomyelopathy

38
Q

Which specialities often see patients with MND first

A

Stroke
ENT
Often people think it could be a neuropathy or carpal tunnel

39
Q

Which drug can be used to manage MND in some patients

A

Riluzole - only drug available
Can prolong life by around 3 months in late stage disease
Lots of side effects - liver and renal failure, muscle issues

40
Q

How is BiPAP used in MND

A

Given to patients who struggle to breathe when lying flat

Used overnight

41
Q

Which allied health professionals are involved in MND care

A

Key worker assessing needs and co-ordinate care
Speech and language - speech therapy, technology etc
Dieticians - nutrition support
Respiratory support and assessment
Occupational therapy
Each patient is also allocated a consultant (neurologist) and 2 nurses to manage their case - specialist MND nurses
GP should also be involved

42
Q

Is MND managed in hospital or at home

A

Aim to manage them at home as they will be more comfortable and aren’t going to be ‘treated’

43
Q

How often are MND patients assessed

A

Every 4-6 weeks

44
Q

What level of weight loss signals a poor prognosis

A

More than 10% weight loss

45
Q

How can you support a MND patient nutritionally

A

Dietician input
Give small high energy supplements regularly
Consider feeding difficulty - may need support at mealtimes
Early insertion of gastrostomy tubes should be clinical priority - can still eat but most nutrition comes through PEG tube
Makes patients more comfortable and removes stress of eating with swallowing issues

46
Q

How can you treat hypersalivation in MND

A

Hyoscine
Buscopan
Botox

47
Q

How can you treat muscle cramps in MND

A

Quinine - not very good

Baclofen - don’t give too much

48
Q

How can you treat muscle spasms in MND

A

Baclofen
Tizanidine
Gabapentin

49
Q

What respiratory interventions are used in MND

A

Non invasive ventilation is offered to support type 2 respiratory failure
BiPAP helps symptoms - particularly overnight

Tracheostomy is next step

50
Q

What are the red flag signs of respiratory failure

A 
Breathlessness
Orthopnoea
Recurrent chest infection
Disturbed sleep
Non-refreshed sleep
Nightmares
Daytime sleepiness
Poor concentration
51
Q

How are benzodiazepines used in MND

A

Lorazepam can be used to help with breathlessness and anxiety

52
Q

What are the emotional symptoms of MND

A

Inappropriate crying or laughing - due to frontal lobe effects
Patience and understanding may be poor
Apathy and disinhibition

53
Q

Does MND lead to cognitive impairment

A

Yes in around 50% of patients
Associated with frontotemporal dementia
Will have apathy, disinhibition, poor planning/decision making

54
Q

When should you refer to palliative care

A

Early on
Need to put in place plans for future care and needs
Helps cope with complex needs
Plan for death

55
Q

What is the only licensed treatment for MND

A

Riluzole
It is an antioxidant
Prolongs survival by 2-3 months
Not all patients chose it as they are often quite disabled by presentation

56
Q

How would ALS present on examination

A

It is a mixed subtype so leads to a confusing exam as both UMN and LMN signs present

57
Q

Why is primary lateral sclerosis hard to diganose

A
  • Often hard to diagnose as you don’t get the classic peripheral signs of MND
    Get a lot of tests looking for purely central issue - again a diagnosis of exclusion
58
Q

Those with primary lateral sclerosis often progress and develop peripheral symptoms - true of false

A

True

59
Q

Why is primary muscular atrophy hard to diganose

A

Can present like other motor neuropathies so many end up on treatment for those
The diagnosis comes when they don’t respond - diagnosis of exclusion

Neurology (18 decks)

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