Neurodegenerative Disorders Lecture: Loss of Neurons and Cognitive Abnormalities
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Questions and Answers

What is the typical progression of Lewy body dementia?

  • Progresses over 10 to 15 years, eventually producing severe motor slowing or near immobility (correct)
  • Progresses over 5 to 10 years, eventually producing severe motor slowing or near immobility
  • Progresses over 15 to 20 years, eventually producing severe motor slowing or near immobility
  • Progresses rapidly, producing severe motor slowing or near immobility within 5 years

What is the relationship between Lewy body dementia and Parkinson's disease?

  • Lewy body dementia occurs when the cortex is involved, in addition to the basal ganglia as in Parkinson's disease (correct)
  • Lewy body dementia and Parkinson's disease are completely unrelated conditions
  • Lewy body dementia and Parkinson's disease have no overlapping symptoms
  • Lewy body dementia is a subtype of Parkinson's disease

What is the primary cause of death in patients with Lewy body dementia?

  • Kidney failure
  • Aspiration pneumonia or trauma from falls caused by postural instability (correct)
  • Respiratory failure
  • Heart failure

How does the effectiveness of L-DOPA treatment change over the course of Lewy body dementia?

<p>L-DOPA is initially effective, but becomes less effective with disease progression (C)</p> Signup and view all the answers

What is the difference between fine and coarse tremors in Parkinson's disease?

<p>Fine tremors are seen in hyperthyroidism or hypoglycemia, while coarse tremors are seen in Parkinson's disease (C)</p> Signup and view all the answers

How does the slowed movement (bradykinesia) affect a Parkinson's patient's gait?

<p>Patients take shorter steps and have difficulty initiating movement (B)</p> Signup and view all the answers

What characterizes neurodegenerative diseases involving the cortex?

<p>Memory loss, behavioral changes, and language difficulties (A)</p> Signup and view all the answers

Which disease is characterized by difficulty in swallowing and respiratory problems due to muscle weakness?

<p>Amyotrophic Lateral Sclerosis (B)</p> Signup and view all the answers

What is the main cause of Parkinson's Disease?

<p>Loss of dopaminergic neurons from the substantia nigra (D)</p> Signup and view all the answers

Which disease presents as a hypokinetic movement disorder?

<p>Parkinson's Disease (C)</p> Signup and view all the answers

What neurological symptom is associated with diseases affecting the cerebellum?

<p>Ataxia (C)</p> Signup and view all the answers

Which diseases involve cognitive abnormalities like memory loss and behavior changes?

<p>Frontotemporal Dementia and Pick Disease (B)</p> Signup and view all the answers

What is the relationship between the number of CAG repeats and the age of onset of the disease?

<p>More CAG repeats lead to an earlier age of onset. (D)</p> Signup and view all the answers

What is the consequence of further expansions of CAG repeats during spermatogenesis?

<p>Early onset disease in the next generation. (D)</p> Signup and view all the answers

Which brain region shows striking atrophy in Huntington's disease?

<p>Caudate nucleus and putamen (A)</p> Signup and view all the answers

What is the main type of neurons that are especially sensitive and disappear early in Huntington's disease affected regions?

<p>Spiny neurons releasing GABA, enkephalin, dynorphin, and substance P (B)</p> Signup and view all the answers

What is a significant characteristic of the intranuclear inclusions seen in Huntington's disease?

<p>They contain aggregates of ubiquitinated huntingtin protein. (A)</p> Signup and view all the answers

How do larger numbers of CAG repeats influence disease onset in Huntington's?

<p>Lead to earlier onset due to proteolysis and aggregation of the mutant protein. (D)</p> Signup and view all the answers

What is the second most common neurodegenerative disorder after Alzheimer's disease?

<p>Parkinsonism (B)</p> Signup and view all the answers

What is the primary characteristic of the resting tremor in Parkinsonism?

<p>It is a coarse tremor that disappears with movement (B)</p> Signup and view all the answers

What is the primary cause of Parkinsonism?

<p>Damage to dopaminergic neurons projecting from the substantia nigra to the striatum (C)</p> Signup and view all the answers

What is the key diagnostic feature of Parkinsonism?

<p>Lewy bodies containing $\alpha$-synuclein (B)</p> Signup and view all the answers

What is the underlying mechanism that leads to neuronal loss in Parkinsonism?

<p>All of the above (D)</p> Signup and view all the answers

How does Parkinsonism differ from Parkinson's disease?

<p>Parkinsonism is a clinical syndrome, while Parkinson's disease is a specific neurodegenerative disorder (B)</p> Signup and view all the answers

Based on the information provided, which condition is the patient most likely suffering from?

<p>Lewy body dementia (A)</p> Signup and view all the answers

What is the protein accumulated in this patient's condition?

<p>Alpha-synuclein (C)</p> Signup and view all the answers

Which of the following is NOT a characteristic feature of Lewy body dementia?

<p>Intranuclear inclusions (A)</p> Signup and view all the answers

In which of the following conditions are intranuclear inclusions observed?

<p>Huntington's disease (C)</p> Signup and view all the answers

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