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Z Year3 Neuro Conditions > Motor Neuron Disease > Flashcards

Motor Neuron Disease Flashcards

1
Q

What is motor neuron disease?

A

cluster of neurodegenerative disease affect

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2
Q

How common is MND?

A

6/100,000

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3
Q

What is epid of MND?

A
  • Male more than female (3:2)

- Usually over 40

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4
Q

What is MND characterised by?

A

loss of neurons in:

  1. motor cortex
  2. cranial nerve nuclei
  3. anterior horn cells
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5
Q

How can you distinguish MND from MS and polyneuropathies?

A

upper and lower motor neurons can be affected but NO sensory loss or sphincter disturbances: distinguish between MS and polyneuropathies

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6
Q

How can you distinguish between myasthenia gravis and MND?

A

MND never affects eye movement, so not myasthenia

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7
Q

What are the 4 clinical patterns of MND and their prevelances?

A
  1. ALS (amyotrophic lateral sclerosis): 80%
  2. Progressive bulbar palsy: 10-20%
  3. Progressive muscular atrophy (<10%)
  4. Primary lateral sclerosis: rare
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8
Q

Where is the loss of neurons and signs in ALS?

A
  1. Loss of motor neurons in motor cortex and anterior horn of cord
  2. Combined UMN and LMN signs
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9
Q

When does ALS have worse prognosis?

A
  • bulbar onset
  • high age
  • low Forced vital capacity
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10
Q

What does progressive bulbar palsy affect?

A

Only affects cranial nerves IX-XII

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11
Q

Where is the loss of neurons and signs in progressive muscular atrophy?

A
  1. Anterior horn cell lesion
  2. LMN signs ONLY
  3. Affects distal muscle groups before proximal
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12
Q

What is the prognosis of progressive muscular atrophy?

A

better than ALS

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13
Q

Where is the loss of cells and signs in primary lateral sclerosis?

A
  1. Loss of Betz cells in motor cortex
  2. Mainly UMN cells
  3. Marked spastic leg weakness and pseudobulbar palsy
  4. No cognitive decline
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14
Q

What are RF for MND?

A
  1. Genetic predispotion
  2. FHx
  3. Age
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15
Q

What are symptoms and signs of MND?

A
  1. Stumbling spastic gait
  2. Foot drop ± proximal myopathy
  3. Weak grip
  4. Shoulder abduction (hard to hair wash)
  5. Aspiration pneumonia
  6. SOB
  7. Dysphagia
  8. Progressive muscle weakness
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16
Q

What are UMN signs in MND?

A
  1. Spasticity
  2. Brisk reflexes
  3. Increased plantars
  4. Hyper-reflexia
  5. Clonus
  6. Positive Babinski’s signs
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17
Q

What are LMN signs in MND?

A
  1. Wasting and fasiculation of tongue, abdomen, back, thigh
  2. Hypotonia
  3. Hyporeflexia
  4. Muscle atrophy
18
Q

What are the bulbar signs of MND?

A
  • speech and swallowing affected
    1. Flaccid and fasciulating tongue
    2. Jaw jerk is normal or absent
    3. Speech is quiet hoarse of nasal
19
Q

What are possible DDx for MND?

A
  1. Cervical spondylosis with myelopathy and radiculopathy
  2. Multifocal motor neuropathy
  3. Inclusion body myositis
  4. Monomelic amyotrophy 5. MG
20
Q

What criteria is used for ALS?

A

El Escorial Diagnostic criteria for ALS

21
Q

What is definite ALS?

A

UMN and LMN signs in 3 regions

22
Q

What is probable ALS?

A

LMN and UMN signs in 2 regions

23
Q

What is probable ALS with lab support?

A

LMN and UMN signs in 1 region or UMN signs in more than 1 region and EMG shows acute denervation in 2 or more limb

24
Q

What is possible ALS?

A

UMN and LMN signs in 2 regions

25
Q

What is suspected ALS?

A

UMN or LMN signs only in 1 or more regions

26
Q

What investigations may you do in MND and why?

A
  1. Brain/cord MRI to exclude structural causes
  2. LP exclude inflammatory causes
  3. Neurophysiology can detect subclinical denervation and hep exclude mimicking motor neuropathies
27
Q

What medication is used in MND to improve survival?

A

Riluzole 50mg twice daily

28
Q

How does Riluzole work?

A

inhibitor of glutamate released and NMDA receptor antagonist: improve survival

29
Q

When else may you use medication in MND?

A

do control symptoms

30
Q

What are examples of other medications used for MND?

A
  1. Can use carbocisteine and supportive respiratory management: for excess mucus
  2. Can used NIPPV: for resp symptoms
  3. Can used Muscle relaxants: for spasticity
31
Q

What are possible complications for MND?

A
  1. Respiratory failure
  2. Nutritional deficit
  3. Aspiration pneumonia
  4. Riluzole-related hepatotoxicity
  5. Riluzole-related neutropenia
32
Q

What can motor neuron disease also be called?

A
  1. Amyotrophic lateral sclerosis (ALS)
  2. Lou Gehrig’s disease
  3. Charcot’s disease
33
Q

What is the patho of MND?

A
  1. Build up of unbiquinated proteins in cytoplasm

2. Death of corticospinal tracts

34
Q

How does MND present?

A

Presents with upper and lower MN symptoms

35
Q

What is spared in MND?

A
  1. oculomotor
  2. sensory
    3, autonomic function
36
Q

What are areas of wasting in MND?

A
  1. tounge base

2. thenar muscles

37
Q

What does EMG show in MND?

A

fibrillation and fasiculations

38
Q

What are fasiculations?

A

damaged motor units produce spontaneous action potentials, resulting in a visible twitch

39
Q

What are fibrillations?

A

spontaneous twitching of individual muscle fibres; recorded during needle electromyography examination

40
Q

What test is usually positive in MND?

A

97% of MND patients have ubiquitin inclusions positive for TDP-43

41
Q

Are there sensory symptoms in MND?

A

NO - and no autonimic e.g. constipation

Z Year3 Neuro Conditions (34 decks)

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