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Osce > Motor neuron disease > Flashcards

Motor neuron disease Flashcards

1
Q

What is motor neuron diseases?

A

A cluster of major degenerative diseases characterised by selective loss of neutrons in the motor cortex, cranial nerve nuclei and anterior horn cells.

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2
Q

What type of nerves are affected?

A

Upper and lower motor neurone, but no sensory loss of sphincter disturbance. It never affects eye movements

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3
Q

What are the four types of MND?

A

ALS, progressive bulbar palsy, progressive muscular atrophy and primary lateral sclerosis.

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4
Q

What nerves are affects in ALS?

A

Loss of motor neurone in motor cortex and the anterior horn of the cord.

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5
Q

What are the symptoms of ALS?

A

Weakness and UMN signs and LMN wasting/fasciculation. Progressive tetra paresis or paraparesis with wasting and fasciculation.

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6
Q

What makes a worse prognosis of ALS?

A

Bulbar onset, old, thenar wasting.

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7
Q

Which are the most common types of MND?

A

ALS (50%), The others are 10/20%

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8
Q

What nerves are affected by a bulbar palsy?

A

Only affects cranial nerves 9-12 in the medulla. LMN lesion of the tongue and muscles of talking and swallowing,

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9
Q

What are the signs of a bulbar palsy?

A

Flaccid, fasciculating tongue, jaw jerk is normal or absent, speech is quiet, horse or nasal. Dysphagia with wasting.

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10
Q

What nerves are affected by progressive muscular atrophy?

A

Anterior horn cell lesion only, therefore no UMN signs.

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11
Q

Which muscle groups are affected first by progressive muscular atrophy?

A

Affects distal muscle groups before proximal. Presents with weakness, wasting and fasciculations in the hands and arms.

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12
Q

Does progressive muscular atrophy or ALS have a better prognosis?

A

Progressive muscular atrophy

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13
Q

What nerves are affected in primary lateral sclerosis?

A

Loss of Betz cells in motor cortex, thus mainly UMN signs.

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14
Q

What are the symptoms of primary lateral sclerosis?

A

Marked spastic leg weakness and psuedobulbar palsy. No cognitive decline. Progressive tetraparesis

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15
Q

How do you diagnose MND?

A

There is no diagnostic test, it is on clinical features

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16
Q

How common is MND?

A

6 in 100,000

17
Q

Is it more common in men or women?

A

Men:women, 3:2

18
Q

What is the median age of onset?

A

60 years

19
Q

What is the prognosis?

A

Often fatal in 2-4 years

20
Q

Who is in the team to help treatment?

A

A multidisciplinary approach is best: neurologist, palliative nurse, hospice, physiology, speech therapist, OT, dietician, social services

21
Q

What medication can you give to prolong life?

A

Riluzole prolongs life by approx. 3 months, it is costly

22
Q

What are the side effects of Riluzole?

A

Liver damage (check every 3 months), vomiting, headache, vertigo, increased pulse

23
Q

What medications can you give to help the drooling?

A

Amitriptyline, doctors think this is good but patients tend to disagree.

24
Q

What medications can you give for spasticity?

A

Baclofen

25
Q

What can you do to help the dysphagia?

A

Blend food, or possible NG tube

26
Q

What medications can you give to help the joint pain and distress?

A

Analgesic ladder

27
Q

What can you do to prevent respiratory failure?

A

Non-invasive ventilation at home may give valuable palliation.

28
Q

Is it hereditary?

A

Most cases are sporadic with no family history.

29
Q

What does motor neurone disease not affect?

A

Eye movements, the senses (sight, touch, sound) or the bladder or bowel

30
Q

Will I get cognitive changes?

A

Around 35% of people with MND experience mild cognitive change, which can cause issues in executive functions such as planning, decision-making and language

31
Q

What blood test can you get with MND?

A

It can increase your creatinine kinase, but this is not specific

32
Q

What nerve tests are there?

A

Nerve conduction tests and an EMG

33
Q

Are there any packs I can have in case of emergencies?

A

MND associate have a just in case kit that has two kits, one for carer’s and one for doctors in case anything bad happens

34
Q

What is non-invasive ventilation?

A

a portable machine supports your own breathing by providing extra air through a mask

35
Q

Is there any preventative measures I can take against any other diseases?

A

You are advised to get a flu jab each year.

36
Q

Is there any end of life care decision I should make?

A

It is easiest to make these decisions whilst you can still speak and communicate properly, it is advised to put someone as your power of attorney.

Osce (20 decks)

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