Motor Neuron Disease

Question 1

Question

What is the most common form of Motor Neuron Disease?

Answer

Amyotrophic Lateral Sclerosis (ALS)
Hereditary Spastic Paraplegia (HSP)
Primary Lateral Sclerosis (PLS)

Question 2

Question

Which type of motor neuron disease exhibits both upper and lower motor neuron degeneration?

Answer

Hereditary Spastic Paraplegia (HSP)
Primary Lateral Sclerosis (PLS)
Amyotrophic Lateral Sclerosis (ALS)

Question 3

Question

Which type fo Motor Neuron Disease is also referred to as Lou Gehrig's Disease?

Answer

Hereditary Spastic Paraplegia (HSP)
Primary Lateral Sclerosis (PLS)
Amyotrophic Lateral Sclerosis (ALS)

Question 4

Question

Amyotrophic Lateral Sclerosis typically presents at what age?

Answer

Around 40
Around 20
Around 60
Teenage years

Question 5

Question

The average survival for patients diagnosed with ALS is [blank_start]2-4[blank_end] years, with most eventually dying of [blank_start]respiratory[blank_end] failure

Answer

respiratory
heart
cerebral
2-4
10-20
15-20

Question 6

Question

The most common symptoms of onset in ALS are spinal onset and bulbar onset. Match the type of onset to its symptoms: [blank_start]Spinal onset (65%)[blank_end] = Presents with progressive weakness in the arms and the legs [blank_start]Bulbar onset (25%)[blank_end] = Presents with progressive weakness in the muscles of speech, chewing & swallowing.

Answer

Bulbar onset (25%)
Spinal onset (65%)

Question 7

Question

Which of the following describe Babinski sign

Answer

Firm stroking of the sole of the foot causes plantarflexion of the foot
Firm stroking of the sole of the foot causes dorsiflexion of the foot
Firm stroking of the sole of the foot causes eversion of the foot
Firm stroking of the sole of the foot causes inversion of the foot

Question 8

Question

A clonic jaw reflex would suggest which type of ALS onset?

Answer

Bulbar onset
Spinal onset
Respiratory onset

Question 9

Question

The hands can be a prime indicator of ALS. Split hand syndrome seen in ALS describes the preferential wasting of the [blank_start]thenar[blank_end] muscle group.

Answer

thenar
hypothenar

Question 10

Question

Primary Lateral Sclerosis differs from Amyotrophic Lateral Sclerosis in that it does NOT show signs of [blank_start]lower[blank_end] motor neuron degeneration

Answer

lower
upper

Question 11

Question

Which condition has a better prognosis?

Answer

Amyotrophic Lateral Sclerosis
Primary Lateral Sclerosis

Question 12

Question

There is a strong link between ALS and which type of dementia?

Answer

Frontotemporal Dementia (Pick's Disease)
Vascular Dementia
Alzheimer's Disease
Dementia with Lewy Bodies

Question 13

Question

Babinski Sign suggests that a patient has experienced [blank_start]upper[blank_end] motor neuron degeneration

Answer

upper
lower

Question 14

Question

What are the two most common causes of death in ALS patients?

Answer

Respiratory failure
Pneumonia
Cardiac failure
Endocarditis
Lung cancer
Choking from aspiration

Question 15

Question

The pathological hallmark of ALS is the presence of [blank_start]inclusion[blank_end] bodies (abnormal aggregations of protein) in the cytoplasm of motor neurons

Answer

inclusion
subtrusian
lewy
tau
ubiquitin

Question 16

Question

How are reflexes affected by ALS?

Answer

Patients have hyperreflexia due to the UMN degeneration
Patients have hyporeflexia due to the LMN degeneration

Question 17

Question

[blank_start]Kennedy's[blank_end] Disease is an X-linked recessive condition of the motor neurons in the brainstem and spinal cord. Degeneration of these structures leads to weakness and muscle cramps. It is commonly misdiagnosed as ALS.

Answer

Kennedy's

Question 18

Question

Kennedy's Disease [blank_start]only affects males[blank_end]

Answer

only affects males
only affects females
affects males and females equally

Question 19

Question

What is the main treatment for the respiratory failure seen in ALS patients?

Answer

Invasive ventilation
Non-invasive ventilation

Question 20

Question

Which sodium channel antagonist can be given to patients with ALS, prolonging their life by 2-3 months? [blank_start]Riluzole[blank_end]

Answer

Riluzole

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Motor Neuron Disease

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	Created by Matthew Coulson over 5 years ago