Motor Neuron Disease

Description

Motor Neuron Diseases lecture given by Esther Sammler in Week 5
Matthew Coulson
Quiz by Matthew Coulson, updated more than 1 year ago
Matthew Coulson
Created by Matthew Coulson about 6 years ago
632
1

Resource summary

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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