Motor Neurone Disease Flashcards

(30 cards)

1
Q

What is motor neurone disease?

A

Painless, progressive limb weakness

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2
Q

Which neurones does MND affect?

A

The motor neurones
The sensory neurones are spared

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3
Q

What are the different types of MND?

A

Amyotrophic lateral sclerosis
Primary lateral sclerosis
Progressive muscular atrophy
Progressive bulbar palsy

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4
Q

Which form of MND is the most common?

A

Amyotrophic lateral sclerosis

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5
Q

What signs do you get with amyotrophic lateral sclerosis?

A

LMN signs in arms
UMN signs in legs

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6
Q

What signs do you get with primary lateral sclerosis?

A

UMN signs only

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7
Q

What signs do you get with progressive muscular atrophy?

A

LMN signs only
Affects distal muscles before proximal

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8
Q

How does progressive bulbar palsy present?

A

Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei

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9
Q

What are the signs of progressive bulbar palsy due to?

A

Loss of function of brainstem motor nuclei

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10
Q

Which form of MND has the worst prognosis?

A

Progressive bulbar palsy

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11
Q

Does MND present symmetrically or asymmetrically?

A

Asymmetrically

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12
Q

What are the 4 classic signs of MND?

A

Clumsy
Tripping
Dropping
Slurring

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13
Q

What upper motor neurone signs might present in the legs?

A

Hypertonia
Hyperreflexia

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14
Q

What lower motor neurone signs might present in the arms?

A

Hypotonia
Hyporeflexia

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15
Q

How does MND normally first present?

A

Symptoms in the arms

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16
Q

What are other symptoms of MND?

A

Fasciculations
Affects the small muscles of the hand

17
Q

Are the eyes affected in MND?

A

No
The eyes are normally spared

18
Q

How is MND initially diagnosed?

19
Q

What can you do to confirm the diagnosis of MND?

20
Q

Why might you do an MRI in someone with MND?

A

To rule out spinal cord compression

21
Q

What is the management of MND?

22
Q

What type of drug is riluzole?

A

Anti-glutaminergic

23
Q

How does riluzole act?

A

Slows the progression of the disease

24
Q

What other management is there for MND?

A

Non-invasive ventilation at night

BIPAP

25
What do people with MND normally die from?
Respiratory or bulbar failure
26
What form of nutritional support is preferred in those presenting with MND?
PEG
27
What would point towards a diganosis of MND rather than anything else?
asymmetric limb weakness mixture of LMN and UMN signs wasting of the small hand muscles/tibialis anterior fasciculations the absence of sensory signs
28
How long does riluzole prolong life?
3 months
29
How long does non-invasive ventilation prolong life?
7 months
30
What form of dementia is associated with MND?
Frontotemporal dementia