Motor Neuron Disease Flashcards

1
Q

Motor neuron disease is a n________ disease, causing ___ and ___ signs

A

neurodegenerative
UMN and LMN

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2
Q

What is the main motor tract called?

A

Corticospinal tract

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3
Q

True or false: an UMN lesion causes hypotonia

A

False
UMN causes hypertonia, rigidity and spasticity

A LMN lesion causes hypotonia (flaccid and muscle wasting)

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4
Q

True or false: an UMN lesion causes hyperreflexia

A

True

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5
Q

What are fasciculations?

A

Muscle twitching due to an overactive nerve resulting in involuntary muscle movement

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6
Q

Are fasciculations present in an UMN or LMN lesion?

A

LMN

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7
Q

True or false: an UMN lesion gives a positive Babinski sign

A

True

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8
Q

What is Babinski sign?

A

Sole of foot is firmly stroked causing big toe to move upward and the other toes fan out
Normal reflex in infants (up to 2 y/o)
In adults, indicates problem with brain or spinal cord.

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9
Q

In an UMN lesion are the the flexor or extensor muscles in the arm more powerful?

A

Flexors

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10
Q

In an UMN lesion are the the flexor or extensor muscles in the leg more powerful?

A

Extensors

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11
Q

True or false: A LMN lesion can result in hyporeflexia, no fasciculations, positive Babinski sign and less power?

A

False
In a LMN lesion, get: hyporeflexia, fasciculations, negative Babinski sign and generally less power

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12
Q

If you see mixed UPN and LMN signs what could it be?

A

Motor neuron disease

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13
Q

What are risk factors for MND?

A

Male
Family history
SOD-1 mutation
Age

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14
Q

True or false: MND can cause paralysis of ocular muscles

A

False
Never affects eye muscles unlike in MS and MG (myasthenia gravis)

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15
Q

Does MND affect sensory function?

A

No. Sensory function can be affected in MS and polyneuropathies

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16
Q

After the idea of movement has begun in the association cortexes and pre-motor cortex and the UMNs have been activated in the motor cortex, where does the impulse travel next?

A

Down corticospinal tract with modulations at the cerebellum for fine tuning and basal ganglia to continue impulse.

17
Q

What are 2 classifications of MND?

A

ALS (more common) and progressive bulbar palsy

18
Q

What does ALS stand for?

A

Amyotrophic Lateral Sclerosis

19
Q

Does ALS affect UMN, LMN or both?

A

Both

20
Q

Does Progressive Muscular Atrophy occur in UMNs, LMNs or both?

A

LMN only

Is the degeneration of LMNs, generalised and progressive loss of muscle function

21
Q

What is PLS (primary lateral sclerosis)?

A

Type of motor neuron disease. Weakness in voluntary muscle movement due to nerve cells degenerating in motor regions.

22
Q

What is affected and in what order with primary lateral sclerosis?

A

Movements are slow and effortful first in legs, then body, trunk, arms, hands and bulbar muscles

23
Q

Does Primary Lateral Sclerosis occur in UMNs, LMNs or both?

A

UMNs only

24
Q

What is progressive bulbar palsy (PBP)?

A

Worst prognosis
Impaired function of lower cranial nerves that arise from brainstem
CN 9-12 affected
Difficulty chewing and swallowing, reduced gag reflex, nasal regurgitation, slurred speech, aspiration of secretions, dysphonia (hoarseness) and dysarthria

25
Q

There is an increased chance of _____ failure with progressive bulbar disease

A

respiratory

26
Q

What signs aren’t present in MND to help differentiate it from other neuro conditions?

A

No eye signs
No sensory signs
Cerebellar function fine
No Parkinson’s signs

27
Q

How is MND diagnosed?

A

Mainly clinical
EMG (electromyography) shows fibrillation potentials due to degeneration of muscles with LMN dysfunction

28
Q

How is MND managed?

A

Riluzole (anti-glutaminergic)
Supportive physio and breathing support

29
Q

What are complications of MND?

A

Respiratory failure
Aspiration pneumonia
Swallowing failure

30
Q

What is PMA (progressive muscular atrophy)?

A

A type of MND whereby there is degeneration of lower motor neurons only causing progressive loss of muscle function

31
Q

What are some symptoms of Progressive Muscular Atrophy?

A

Muscle weakness and wasting in legs, arms, body and hands
Flail arm syndrome
Fasciculations
Difficulty breathing