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SL22044 Neuroscience > ALS > Flashcards

ALS Flashcards

1
Q

What does MND refer to?

A

A heterogenous group of conditions characterised by degeneration of lower motor neurons and/or upper motor neurons

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2
Q

Where are the upper motor neurons?

A

Cortex and brainstem

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3
Q

Where are the lower motor neurons?

A

SC

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4
Q

Which upper motor neurons can be affected by MND?

A

Ones that synapse with lower motor neurons

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5
Q

Which diseases involve upper motor neuron defects?

A

Primary lateral sclerosis, pseudobulbar palsy

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6
Q

Which diseases involve lower motor neuron diseases?

A

Progressive muscular atrophy, spinal muscular atrophy, progressive bulbar palsy, Kennedy disease

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7
Q

What is a common symptom between all MNDs involving lower motor neurons?

A

Muscular atrophy/loss of muscle function

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8
Q

Which disease is associated with upper and lower motor neuron dysfunction?

A

Amyotrophic lateral sclerosis

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9
Q

When was ALS first described in a publication?

A

1869

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10
Q

ALS stands for?

A

Amyotrophic lateral sclerosis

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11
Q

Amyotrophic meaning?

A

No muscle nourishment

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12
Q

Sclerosis meaning?

A

Scarring of muscle

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13
Q

Main cause of death as a result of ALS?

A

Respiratory failure

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14
Q

Signs that upper motor neurons are affected negatively?

A

Hyperreflexia, spasticity, slowing of movements

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15
Q

What is hyperreflexia?

A

Reflexes are frequent and happen in an uncontrolled way

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16
Q

Muscle spasticity?

A

Muscles become thinner and lose the stiffness

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17
Q

Signs that lower motor neurons are affected?

A

Weakness, muscle atrophy, fasciculations

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18
Q

What is fasiculations?

A

Uncontrolled, rapid movements

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19
Q

Why is drooling seen in ALS?

A

Upper motor neurons (brainstem etc) is affected–> muscles that control saliva cannot be controlled correctly

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20
Q

Established risk factors for ALS?

A

Family history and age

21
Q

What % of ALS patients have inherited a mutation associated with the disease from their parents?

A

10%

22
Q

Lifestyle choices which may be associated w/ ALS?

A

Manual labour, smoking, excessive exercise, exposure to toxic chemicals

23
Q

Why could heavy exercise contribute to ALS?

A

Overuse of muscles which could lead to muscle injuries and damage of neuons

24
Q

What happens as a result of cortical motor cells dissapearing?

A

Retrograde axonal loss and gliosis in the corticospinal tract

25
Q

What does gliosis cause?

A

White matter changes

26
Q

Effect of ALS on SC?

A

The SC becomes atrophic, ventral roots become thin and there is a loss of myelinated fibres in motor nerves

27
Q

First step in pathogenic mechanisms of ALS?

A

Glutamate excitotoxicity–> if glutamate stays in the synapse for too long it is toxic. Usually astrocytes take excess glutamate up but in ALS there is too much glutamate

28
Q

What does excess glutamate cause in the post N?

A

Excessive Ca2+

29
Q

What does excessive Ca2+ in the post n cause?

A

Mitochondrial dysfunction

30
Q

Effect of mitochondrial dysfunction?

A

ATP production is affected, also superoxides are produces (oxidative stress)

31
Q

What happens as a result of oxidative stress and mitochondrial dysfunction?

A

Aggregation of SOD1 and TPD proteins which are responsible for decreasing oxidative stress

32
Q

What causes neuroinflammation?

A

Release of inflammatory mediators by microglia

33
Q

How can neuroinflammation affect RNA metabolism?

A

Mutation of gene expression which can dysregulated RNA metabolism

34
Q

How does ALS cause axonal transport defects?

A

Neurofilaments that are meant to be present along the axon begin to accumulate–> too much of them

35
Q

Which tools are used to diagnose ALS?

A

El Escorial Criteria–> set of diagnostic guidelines

36
Q

Why is electromyography key in diagnosing MND?

A

It detects electrical activity in muscles and can reveal abnormalities indicative of motor neuron involvement e.g. denervation patterns and spontaneous muscle activity

37
Q

Four steps in El Escorial criteria?

A

Definite ALS, Probable ALS, Probable ALS, lab results supported, possible ALS

38
Q

Possible ALS criteria?

A

Presence of upper motor neuron and lower motor neuron signs in one region or upper motor neuron signs in 2/3 regions

39
Q

Probable ALS, lab results supported criteria?

A

Presence of upper motor neuron and lower motor neuron signs in one region with evidence by EMG of lower motor neuron involvement in another region

40
Q

Probable ALS criteria?

A

Presence of upper and lower motor neuron signs in at least two regions with UMN sign rostral to LMN sign

41
Q

Definite ALS criteria?

A

Presence of upper motor neuron and lower motor neuron signs in three anatomical regions

42
Q

How is a myelogram carried out?

A

A dye is injected into the spinal canal–> dyes areas and then does a CT scan. Look to see where the dye has deposited

43
Q

Which exams can be used to diagnose ALS?

A

MRI, Myelogram, muscle and/or nerve biopsy

44
Q

What do the three separate mechanisms of riluzole all do?

A

Reduce glutamate induced exciotixicity

45
Q

How does riluzole work?

A

It inhibits glutamic acid release, noncompetitive block of NDMA receptor mediated response, direct action on the voltage dependent sodium channel

46
Q

Drawbacks of riluzole?

A

Only extends survival rate by 2-3 months, 50% of patients experience side effects

47
Q

Side effects of riluzole?

A

gastrointestinal disturbances (nausea, vomiting, diarrhea), fatigue, and dizziness

48
Q

Serious risks of riluzole?

A

Neutropenia, liver dysfunction

49
Q

Formulation issues w/ riluzole?

A

Patients cannow swallow

SL22044 Neuroscience (71 decks)

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