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Neurology > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

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?

A

Clinically

19
Q

What can you do to confirm the diagnosis of MND?

A

EMG

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?

A

Riluzole

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
Q

What do people with MND normally die from?

A

Respiratory or bulbar failure

26
Q

What form of nutritional support is preferred in those presenting with MND?

A

PEG

27
Q

What would point towards a diganosis of MND rather than anything else?

A

asymmetric limb weakness
mixture of LMN and UMN signs
wasting of the small hand muscles/tibialis anterior
fasciculations
the absence of sensory signs

28
Q

How long does riluzole prolong life?

A

3 months

29
Q

How long does non-invasive ventilation prolong life?

A

7 months

30
Q

What form of dementia is associated with MND?

A

Frontotemporal dementia

Neurology (63 decks)

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