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Year Two - Neurology System > Motor Neurone Disease > Flashcards

Motor Neurone Disease Flashcards

1
Q

What is motor neurone disease (MND)?

A

It refers to a group of neurodegenerative diseases, in which there is progressive degeneration of both upper and lower motor neurones

The sensory neurones are spared

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2
Q

What are the four classifications of motor neurone disease?

A

Amyotrophic Lateral Sclerosis (ALS)

Progressive Bulbar Palsy (PBP)

Progressive Muscular Atrophy (PMA)

Primary Lateral Sclerosis (PLS)

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3
Q

What is the most common classification of motor neurone disease?

A

ALS

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4
Q

What is ALS?

A

It is a condition in which there is degeneration of the of the motor cortex neuronal cells, resulting in upper motor lesion features, and anterior horn cells, resulting in lower motor lesion features

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5
Q

What are the five clinical features associated with ALS?

A

Muscle wasting

Muscle weakness

Muscle stiffness

Muscle cramps

Rippling of the muscles

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6
Q

What is the characteristic features of ALS?

A

The presentation of lower motor neurone features in the arms and upper motor neurone features in the legs

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7
Q

In familial cases of ALS, which chromosome does the gene mutation lie on? What does this gene code for?

A

Chromosome 21

Superoxide dismutase

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8
Q

What is the second most common classification of motor neurone disease?

A

PBP

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9
Q

What is PBP?

A

It is a condition in which there is degeneration of both upper and lower motor neurones in the bulbar area of the brainstem

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10
Q

What is the function of motor neurones in the bulbar area of the brainstem?

A

They innervate the tongue muscles, pharynx and larynx

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11
Q

What are the three clinical features associated with PBP?

A

Tongue palsy

Speech slurring

Swallowing difficulties

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12
Q

What motor neurone disease classification carries the worst prognosis?

A

PBP

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13
Q

What is PMA?

A

It is characterised by lower motor neurone degeneration only

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14
Q

What clinical feature is associated with PMA?

A

Muscle weakness, which tends to affect distal muscles before proximal

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15
Q

What motor neurone disease classification carries the best prognosis?

A

PMA

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16
Q

What is PLS?

A

It is characterised by upper motor neurone degeneration only

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17
Q

What are the three clinical features of PLS?

A

Lower limb weakness

Lower limb stiffness

Hyperreflexia

18
Q

What are the six risk factors of motor neurone disease?

A

Older Age > 60

Male Gender

Family History

Smoking

Heavy Metals Exposure

Pesticide Exposure

19
Q

Describe the typical presentation of motor neurone disease

A

It results in an insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech

20
Q

What are the four common presentations of motor neurone disease?

A

Clumsiness

Tripping over

Object dropping

Dysphasia

21
Q

What are the four lower motor neurone features of motor neurone disease?

A

Muscle Wasting

Muscle Tone Reduction

Fasciculations

Hyporeflexia

22
Q

What is the key lower motor neurone feature of motor neurone disease?

A

Fasciculations

23
Q

In motor neurone disease, which muscles tend to experience wasting first?

A

Upper limb muscles

24
Q

What two muscles most commonly experience muscle wasting in motor neurone disease?

A

Small hand muscles

Tibilias anterior

25
Q

What are the three upper motor neurone features of motor neurone disease?

A

Muscle Spasticity

Hyperreflexia

Extensor Plantar Reflex

26
Q

What two clinical features don’t indicate motor neurone disease?

A

External ocular muscle wasting

Cerebellar features

27
Q

In most cases, how is motor neurone diagnosed?

A

Clinically

28
Q

What is a clinical diagnosis of motor neurone disease based on?

A

There is evidence of lower and upper motor neurone features in at least three regions of the body, with no associated sensory clinical features

29
Q

What three investigations can be used to confirm a diagnosis of motor neurone disease?

A

Electromyography (EMG)

Nerve Conduction Studies

MRI Scan

30
Q

What is electromyography (EMG)?

A

It measures muscle response or electrical activity in response to a nerve’s stimulation of the muscle

31
Q

What are three features of motor neurone disease on EMG?

A

Reduced Number of Action Potentials

Increased Action Potential Amplitude

Widespread Denervation

32
Q

What EMG feature is required to confirm diagnosis of motor neurone disease?

A

The features should be demonstrated in all four limbs, the muscles above the neck and the tongue

33
Q

What are nerve conduction studies?

A

They measure how fast an electrical impulse is transmitted through the nerve

34
Q

How are nerve conduction studies used to diagnose motor neurone disease?

A

Motor neurone disease results in normal motor conduction, and is therefore used to exclude a neuropathy

35
Q

How are MRI scans used to diagnose motor neurone disease?

A

Motor neurone disease results in normal results, and is therefore used to exclude cervical cord compression and myelopathy

36
Q

What pharmacological management option is used in motor neurone disease?

A

Benzothiazoles

37
Q

What benzothiazole is used to manage motor neurone disease?

A

Riluzole

38
Q

What is the function of Riluzole?

A

It is an NDMA antagonist which inhibits the release of glutamate

39
Q

How does Riluzole treat motor neurone disease?

A

It can be administered to slow down the progression of the disease and extend survival by a few months, in ALS

40
Q

What are the five supportive management options for motor neurone disease?

A

Non-Invasive Ventilation (NIV)

PEG Feeding

Physiotherapy Input

Occupational Therapy Input

Speech & Language Therapy Input

41
Q

What is a complication associated with motor neurone disease?

A

Frontotemporal Dementia

42
Q

What are the two common death causes of motor neurone disease?

A

Respiratory failure

Pneumonia

Year Two - Neurology System (25 decks)

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