Motor Neuron Disease Flashcards

1
Q

Is MND treatable?

A

No.

It is a rapidly progressive neurodegenerative illness.

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2
Q

How does MND initially present?

A

Begins focally, before spreading out and becoming a generalised paresis.

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3
Q

Are most cases of MND sporadic or genetic?

A

Around 90% of cases are sporadic.

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4
Q

What are the 4 types of MND?

A

PLS
PMA
ALS
Bulbar onset MND

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5
Q

What is the most common form of MND?

A

ALS

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6
Q

What is the most common site of MND onset?

A

Limbs

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7
Q

Is bulbar onset MND more common in males or females?

A

Females

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8
Q

Which form of MND has the poorest prognosis?

A

ALS

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9
Q

What is affected in bulbar MND?

A

The muscles supplied by CNVII-CNXII.

Affects tongue muscles, facial muscles and pharyngeal muscles.

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10
Q

Is cognitive impairment a feature seen in MND?

A

Yes

MND has an association with frontotemporal dementia.

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11
Q

What are examples of UMN signs in MND?

A
Increased tone
Hyper-reflexia
Extensor plantar response
Spastic gait
Exaggerated jaw-jerk
Slowed movement
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12
Q

What are examples of LMN signs in MND?

A
Muscle wasting
Weakness
Fasciculations
Absent/reduced reflexes
Loss of tone
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13
Q

What does MND do to the metabolic rate?

A

Increases it. This is called hypercatabolism.

It is linked to the weight loss experienced in MND.

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14
Q

What is split-hand syndrome?

A

Where a region of the hand experiences disease, whilst another area of the same hand remains functional.

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15
Q

What is the name of the diagnostic criteria used for MND?

A

El Escorial criteria.

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16
Q

What is the name of the only drug treatment available for MND?

A

Riluzole

17
Q

Why do patients not like riluzole therapy?

A

Causes many liver/kidney issues.

Only lengthens life for 3 months, and this is during the period in which the patient is most unwell.

18
Q

What is sialorrhoea?

A

Excessive salivation.

Treat with hyoscine. If not enough then botox injection of the salivary glands is an option.

19
Q

What is the treatment for muscle spasms?

A

Gabapentin

20
Q

What is the treatment for muscle cramps?

A

Baclofen

21
Q

What is a major cause of death in MND?

A

Weakening of the respiratory muscles.

Treat with non-invasive ventilation.