Motor Neuron Disease

Descripción

Motor Neuron Diseases lecture given by Esther Sammler in Week 5
Matthew Coulson
Test por Matthew Coulson, actualizado hace más de 1 año
Matthew Coulson
Creado por Matthew Coulson hace más de 5 años
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Resumen del Recurso

Pregunta 1

Pregunta
What is the most common form of Motor Neuron Disease?
Respuesta
  • Amyotrophic Lateral Sclerosis (ALS)
  • Hereditary Spastic Paraplegia (HSP)
  • Primary Lateral Sclerosis (PLS)

Pregunta 2

Pregunta
Which type of motor neuron disease exhibits both upper and lower motor neuron degeneration?
Respuesta
  • Hereditary Spastic Paraplegia (HSP)
  • Primary Lateral Sclerosis (PLS)
  • Amyotrophic Lateral Sclerosis (ALS)

Pregunta 3

Pregunta
Which type fo Motor Neuron Disease is also referred to as Lou Gehrig's Disease?
Respuesta
  • Hereditary Spastic Paraplegia (HSP)
  • Primary Lateral Sclerosis (PLS)
  • Amyotrophic Lateral Sclerosis (ALS)

Pregunta 4

Pregunta
Amyotrophic Lateral Sclerosis typically presents at what age?
Respuesta
  • Around 40
  • Around 20
  • Around 60
  • Teenage years

Pregunta 5

Pregunta
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
Respuesta
  • respiratory
  • heart
  • cerebral
  • 2-4
  • 10-20
  • 15-20

Pregunta 6

Pregunta
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.
Respuesta
  • Bulbar onset (25%)
  • Spinal onset (65%)

Pregunta 7

Pregunta
Which of the following describe Babinski sign
Respuesta
  • 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

Pregunta 8

Pregunta
A clonic jaw reflex would suggest which type of ALS onset?
Respuesta
  • Bulbar onset
  • Spinal onset
  • Respiratory onset

Pregunta 9

Pregunta
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.
Respuesta
  • thenar
  • hypothenar

Pregunta 10

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

Pregunta 11

Pregunta
Which condition has a better prognosis?
Respuesta
  • Amyotrophic Lateral Sclerosis
  • Primary Lateral Sclerosis

Pregunta 12

Pregunta
There is a strong link between ALS and which type of dementia?
Respuesta
  • Frontotemporal Dementia (Pick's Disease)
  • Vascular Dementia
  • Alzheimer's Disease
  • Dementia with Lewy Bodies

Pregunta 13

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

Pregunta 14

Pregunta
What are the two most common causes of death in ALS patients?
Respuesta
  • Respiratory failure
  • Pneumonia
  • Cardiac failure
  • Endocarditis
  • Lung cancer
  • Choking from aspiration

Pregunta 15

Pregunta
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
Respuesta
  • inclusion
  • subtrusian
  • lewy
  • tau
  • ubiquitin

Pregunta 16

Pregunta
How are reflexes affected by ALS?
Respuesta
  • Patients have hyperreflexia due to the UMN degeneration
  • Patients have hyporeflexia due to the LMN degeneration

Pregunta 17

Pregunta
[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.
Respuesta
  • Kennedy's

Pregunta 18

Pregunta
Kennedy's Disease [blank_start]only affects males[blank_end]
Respuesta
  • only affects males
  • only affects females
  • affects males and females equally

Pregunta 19

Pregunta
What is the main treatment for the respiratory failure seen in ALS patients?
Respuesta
  • Invasive ventilation
  • Non-invasive ventilation

Pregunta 20

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