Pathology CPC Year 2: Neurodegeneration Quiz
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Questions and Answers

What is the most likely diagnosis for a patient showing progressive decline and dependency on others?

  • Alzheimer disease (correct)
  • Frontotemporal dementia
  • Lewy body dementia
  • Vascular dementia

    • How can Alzheimer's disease be confirmed postmortem?

  • Genetic testing
  • Brain imaging
  • Autopsy brain examination (correct)
  • Blood test

    • What are key features of the pathology associated with Alzheimer's disease?

  • White matter hyperintensities and axonal regeneration
  • Neurofibrillary tangles and neurodegeneration
  • Vascular lesions and myelination defects
  • Plaques and tangles (correct)

    • What role does Tau play in the axon?

    <p>Stabilizes microtubules</p> Signup and view all the answers

    What is the significance of Aβ42 in Alzheimer's pathology?

    <p>Forms plaques and contributes to neurodegeneration</p> Signup and view all the answers

    Which method is used to detect amyloid plaques in living patients?

    <p>18F-FDG and Pittsburgh Compound B (PiB) imaging</p> Signup and view all the answers

    Why is the examination of brain tissue postmortem critical in Alzheimer's research?

    <p>To confirm diagnosis and understand pathology</p> Signup and view all the answers

    What clinical feature does NOT typically indicate Alzheimer's disease?

    <p>Recognition of familiar faces</p> Signup and view all the answers

    What name is given to the diagnostic criteria for multiple sclerosis?

    <p>McDonald Criteria</p> Signup and view all the answers

    What is the most likely diagnosis for the 31-year-old lady presenting with weakness and numbness in her left leg?

    <p>Multiple sclerosis</p> Signup and view all the answers

    What pathologic process occurs in the brain during an active MS plaque?

    <p>Demyelination</p> Signup and view all the answers

    Which of the following is a common presenting symptom of multiple sclerosis?

    <p>Visual loss</p> Signup and view all the answers

    Which cell type is responsible for myelination in the central nervous system?

    <p>Oligodendrocyte</p> Signup and view all the answers

    What type of multiple sclerosis does the patient’s clinical syndrome most likely represent?

    <p>Relapsing-remitting MS</p> Signup and view all the answers

    What is one of the management goals for patients with multiple sclerosis?

    <p>Improve quality of life</p> Signup and view all the answers

    Which test is considered crucial in supporting the clinical diagnosis of MS?

    <p>Magnetic resonance imaging (MRI) of the brain and spine</p> Signup and view all the answers

    What effect does demyelination have on axonal transport?

    <p>Impairment of saltatory conduction</p> Signup and view all the answers

    What unusual sign can be observed in patients with demyelination?

    <p>Uhtoff's phenomenon</p> Signup and view all the answers

    Which treatment is commonly used in acute management of multiple sclerosis?

    <p>Steroids</p> Signup and view all the answers

    How is the presence of oligoclonal bands relevant to diagnosing MS?

    <p>Supports the diagnosis of inflammatory disease</p> Signup and view all the answers

    What is characterized by axonal loss leading to spinal cord thinning in multiple sclerosis?

    <p>Progressive degenerative phase</p> Signup and view all the answers

    What types of medications are included in long-term management strategies for MS?

    <p>Disease-modifying agents</p> Signup and view all the answers

    Which of the following symptoms is associated with brain stem involvement in MS?

    <p>Double vision</p> Signup and view all the answers

    What is delirium primarily characterized by?

    <p>Reversible changes in attention and cognition</p> Signup and view all the answers

    Which symptom did the 70-year-old man NOT exhibit during his episode of delirium?

    <p>Short term memory loss</p> Signup and view all the answers

    What is a key distinction between delirium and dementia?

    <p>Dementia shows a progressive decline in cognitive function, while delirium does not.</p> Signup and view all the answers

    Which of the following is included in the investigations for cognitive decline?

    <p>Psychological evaluation</p> Signup and view all the answers

    What underlying condition was ruled out in the man’s case before considering cognitive decline?

    <p>Thyroid dysfunction</p> Signup and view all the answers

    Which imaging technique is useful for assessing brain atrophy and excluding strokes?

    <p>CT or MRI</p> Signup and view all the answers

    What anatomical part of the brain is primarily associated with short term memory impairment?

    <p>Hippocampus</p> Signup and view all the answers

    What could be a potential reason for the man’s family attributing ‘forgetfulness’ to aging?

    <p>Belief that cognitive decline is a natural part of aging</p> Signup and view all the answers

    Which characteristic feature is associated with Alzheimer's disease pathology?

    <p>Presence of amyloid plaques</p> Signup and view all the answers

    What is the role of Tau protein in neurodegeneration?

    <p>Stabilizes microtubules</p> Signup and view all the answers

    What confirms the diagnosis of Alzheimer's disease at a definitive level?

    <p>Postmortem brain examination</p> Signup and view all the answers

    Which biochemical marker is primarily elevated in Alzheimer's disease?

    <p>Tau protein</p> Signup and view all the answers

    What is the significance of amyloid beta 42 in Alzheimer's disease?

    <p>Forms harmful plaques in the brain</p> Signup and view all the answers

    Which of the following symptoms is least likely to be associated with multiple sclerosis?

    <p>Sudden seizures</p> Signup and view all the answers

    How does the progression of Alzheimer's disease generally manifest in patients?

    <p>Gradual decline in social interactions</p> Signup and view all the answers

    What is the significance of oligoclonal bands in cerebrospinal fluid analysis for MS diagnosis?

    <p>They confirm the presence of autoimmune activity</p> Signup and view all the answers

    What postmortem finding would be characteristic of Alzheimer’s disease?

    <p>Neurofibrillary tangles in the hippocampus</p> Signup and view all the answers

    What best describes the role of 18F-FDG in the context of diagnosing Alzheimer's disease?

    <p>It helps measure cerebral glucose metabolism</p> Signup and view all the answers

    In the context of multiple sclerosis, which type of progression reflects a steady worsening of symptoms without distinct relapses?

    <p>Primary progressive MS</p> Signup and view all the answers

    Which of the following diagnostic tools is most definitive for observing demyelination in patients suspected of having MS?

    <p>MRI Brain &amp; Spine with contrast</p> Signup and view all the answers

    With regard to multiple sclerosis, what is Lhermitte’s sign commonly associated with?

    <p>Neck flexion causing electric shock-like sensations</p> Signup and view all the answers

    What phenomenon describes an exacerbation of symptoms in MS patients due to increased body temperature?

    <p>Uhtoff's phenomenon</p> Signup and view all the answers

    How is the clinical presentation of transverse myelitis primarily characterized in MS?

    <p>Causes gait and lower limb weakness</p> Signup and view all the answers

    For a patient with multiple sclerosis, which finding would most likely be observed during a sensory examination?

    <p>Decreased pin prick and proprioception in affected areas</p> Signup and view all the answers

    What is the main goal of using disease-modifying agents in multiple sclerosis therapy?

    <p>To shorten relapses and prolong remissions</p> Signup and view all the answers

    Which type of treatment is typically employed for acute episodes in multiple sclerosis?

    <p>Steroids or sometimes plasma exchange</p> Signup and view all the answers

    What is the primary pathological feature observed in an active MS plaque?

    <p>Demyelination with inflammation</p> Signup and view all the answers

    Which criteria must be met according to the McDonald Criteria for diagnosing multiple sclerosis?

    <p>Evidence of CNS lesions in both space and time</p> Signup and view all the answers

    What role do oligodendrocytes play in the central nervous system?

    <p>They myelinate multiple axons</p> Signup and view all the answers

    What is a common consequence of demyelination in terms of nerve signal conduction?

    <p>Impaired saltatory conduction</p> Signup and view all the answers

    Which of the following symptoms is not associated with the active phase of multiple sclerosis?

    <p>Complete resolution of all prior symptoms</p> Signup and view all the answers

    What constitutes a significant change in the progressive phase of multiple sclerosis?

    <p>Transition to neurodegenerative symptoms</p> Signup and view all the answers

    Which of the following best describes delirium?

    <p>An acute disorder of attention and global cognition that is reversible.</p> Signup and view all the answers

    What cognitive area is characterized by a progressive decline in dementia?

    <p>Multiple cognitive areas.</p> Signup and view all the answers

    What initial step should be taken when investigating cognitive decline?

    <p>Exclude acute reversible causes such as infections.</p> Signup and view all the answers

    Which of the following symptoms might suggest a diagnosis of dementia rather than acute delirium?

    <p>Poor recall of significant events.</p> Signup and view all the answers

    What specific test is commonly done to assess cognitive function in suspected dementia cases?

    <p>Montreal Cognitive Assessment.</p> Signup and view all the answers

    What anatomical structure is primarily associated with short-term memory impairment?

    <p>Hippocampus.</p> Signup and view all the answers

    Which brain imaging technique would be most beneficial for excluding strokes when evaluating cognitive decline?

    <p>Magnetic Resonance Imaging (MRI).</p> Signup and view all the answers

    What clinical features might a family describe when discussing the onset of dementia-like symptoms?

    <p>Gradual change in personality and forgetfulness over time.</p> Signup and view all the answers

    Study Notes

    CNS 2 Neurodegeneration/ Demyelination

    • Class: Year 2
    • Course: Pathology CPC
    • Lecturer: Dr. Michael Farrell, Beaumont Hospital
    • Date: November 22nd, 2023

    Clinical Scenario

    • A 31-year-old woman presented with a 4-day history of weakness and numbness in her left leg
    • A year prior, she had an episode of painful blurred vision in her right eye that resolved within a week.
    • No other significant medical history or regular medications.
    • Physical exam revealed increased tone in the left leg, a power of 3/5, brisk reflexes in the left leg, and decreased pin prick and proprioception sensation.
    • Her right optic nerve was pale.

    Questions

    • What is the most likely diagnosis in this case?
    • How else can this condition present?

    Answers: Common presenting symptoms of MS

    • Visual loss
    • Gait and lower limb weakness
    • Facial pain
    • Double vision
    • Gait/balance disturbance
    • Vertigo
    • Bladder dysfunction
    • Optic neuritis
    • Transverse myelitis
    • Trigeminal neuralgia (Pons)
    • Brain stem-MLF
    • Cerebellum/spinal cord
    • Brain stem
    • Spinal cord

    Unusual Signs of Demyelination

    • Lhermitte's sign: Neck flexion resulting in rapid tingling or electric shock-like sensations that travel down the spine and into the limbs.
    • Uhthoff's phenomenon: Raised body temperature can cause an exacerbation of symptoms.

    Questions

    • How would you categorize the patient's clinical syndrome of MS?

    Answers

    • Clinically isolated syndrome
    • Relapsing-remitting MS (majority of cases at onset)
    • Secondary progressive MS
    • Primary progressive MS

    Questions

    • Name 3 investigations that will support your clinical diagnosis

    Answers

    • MRI brain & spine with contrast
    • CSF analysis and SPEP: Oligoclonal bands (OCBs) should be unmatched.
    • Visual evoked potentials, Delayed P100 potentials

    MRI Brain + Contrast - MS

    • Images illustrating inflammatory disease in the brain, potentially a sign of MS.

    Questions

    • What name is given to the diagnostic criteria for MS?

    Answer

    • McDonald criteria

    Diagnosis - McDonald Criteria

    • Objective demonstration of dissemination of CNS lesions in both space and time based on either clinical findings alone or in combination with MRI findings.
    • Specific details of clinical presentation and additional data needed for diagnosis are outlined.

    Questions

    • What pathologic process is going on in the brain during a MS relapse (active MS plaque)?

    Answer

    • Demyelination: Reactive astrocytes, lymphocytes, and microglia and/or macrophages (foamy macrophages) in the perivascular and/or parenchymal environment.

    Demyelination

    • Short segment of remyelination with a thin myelin sheath
    • Demyelinated axon
    • Lymphocytes, microglia, foamy macrophages, and reactive astrocytes

    Questions

    • How does demyelination affect axonal transport?

    Answer

    • Impaired saltatory conduction

    Demyelination

    • Progressive decline, impaired saltatory conduction

    Questions

    • What cell is responsible for myelination in the CNS?

    Answer

    • Oligodendrocyte

    Myelin & the Oligodendrocyte

    • CNS: One oligodendrocyte myelinates several internodes
    • PNS: One Schwann cell myelinates only one internode

    Questions

    • What types of treatment might be used for this patient?
    • What are the management goals?

    Answer

    • Acute: Steroids (sometimes plasma exchange)
    • Long-term: Disease-modifying agents (injectable, oral, infusion medications)
    • Management Goals: Shorten relapses, prolong remissions, prevent progression, improve quality of life
    • Multidisciplinary team, dedicated clinics, address fatigue, spasticity, urogenital problems

    Learning Objectives 1

    • Range of symptoms of multiple sclerosis
    • Clinical classification of MS
    • Investigation of MS (Multiple sclerosis progresses to a progressive degenerative phase characterized by axonal loss (leading to spinal cord thinning) and brain atrophy due to white matter loss).

    Learning Objectives 2

    • Distinguish between delirium and dementia
    • How to investigate above
    • List clinical features of neurodegenerative disease affecting different anatomic sites
    • Discuss how to make a diagnosis of a neurodegenerative disease
    • List pathologic features of a neurodegenerative disease

    Next Case

    • A 70-year-old man presented with fever, disorientation, altered personality, and volatile behavior for two days.
    • Urinalysis showed nitrites, leukocytes, and ketones.
    • Urinary culture showed pure growth of E. coli. He was treated with appropriate antibiotics and recovered.
    • After returning home, he had no memory of the hospital visit and couldn’t recall what hospital he was in.

    Questions

    • What do you understand by the term delirium?
    • List the symptoms of this man's delirium

    Answer

    • Delirium = acute, reversible cognitive disorder
    • Symptoms = disorientation, altered personality, and volatile behavior.

    Questions

    • What is there in the man's story that might indicate he has more than just delirium?

    Answer

    • Inability to recall the hospital event and the location.

    Answer

    • Possible dementia (progressive cognitive decline in at least two cognitive areas)

    Investigation of Progressive Cognitive Decline

    • Exclude acute reversible causes (infection)
    • Detailed history (including family history) and cognitive assessment (MMSE, Montreal cognitive assessment).
    • Exclude treatable conditions (e.g., metabolic causes, thyroid function tests, vitamin B12 levels, U&E, LFTs, autoantibody workup).
    • Brain imaging (CT, MRI, or PET) to assess for stroke or atrophy distribution.
    • Brain biopsy (rarely) to identify treatable causes (e.g., vasculitis, lymphoma).

    History From Family Members

    • Family members reported a change in personality and increasing forgetfulness over the previous 18 months.

    Questions

    • What anatomical parts of his brain are affected by memory impairment?

    Answer

    • Areas associated with memory impairment in the brain: Parietal lobe, frontal lobe, medial temporal lobe (hippocampus, amygdala, limbic system), and temporal neocortex. The frontal lobe manages judgment, abstract reasoning, strategic planning, and emotional restraint. The temporal neocortex is associated with receptive dysphasia and automatisms; the occipital lobe is associated with failure of visual sensory systems.

    More Detailed Examination

    • Examination revealed moderate short-term memory impairment.

    Questions

    • What is the most likely clinical diagnosis?
    • How may the diagnosis be confirmed?

    Answer

    • Alzheimer's disease
    • A definitive diagnosis may be difficult to achieve definitively in life but can be confirmed post-mortem via brain examination, analysis, and or study of tissues.

    Question

    • Over the next year he progressively declines, fails to recognize close family members, becomes withdrawn, dependent on others, until death.
    • How might a diagnosis be confirmed?

    Answer

    • Autopsy (postmortem) and brain examination. This is the only way to definitively confirm the diagnosis of Alzheimer's disease or other conditions.

    Question

    • A pathologic diagnosis of Alzheimer type pathology is made.
    • What are the macroscopic and microscopic features?

    Answer

    • Macroscopic features: Atrophy of the brain, especially in the medial temporal lobe, frontal lobes
    • Microscopic features: Amyloid plaques and neurofibrillary tangles.

    Alzheimer's Disease Gravestones

    • Aloiz Alzheimer
    • Auguste Deter
    • Plaques & Tangles
    • AD - clinical entity, AD - pathologic process with a prodrome

    β A4 Amyloid Plaques in Cerebral Cortex

    • Microscopic image of 𝛽A4 amyloid plaques in cerebral cortex.

    AD Plaque Distribution

    • Diagram of amyloid plaques in the brain, particularly concentrated in the frontal and temporal cortex and hippocampus.

    Vascular Amyloid

    • Micrographs of vascular amyloid deposits

    Alzheimer's Disease - Tau

    • Figures demonstrating pathologic process of tau in the brain, severity and percentage of affected cases correlated with age.

    Tau Function Transgenics

    • Tau -/- nerve cells: Neuritic sprouting but no axon elongation.
    • Tau +/+ non-neural cells: Elaborate long, thin process with compacted MTs

    Neurodegeneration - Amyloid-Tau

    • Illustration of neurodegeneration with amyloid and tau.

    Questions

    • Why is this type of postmortem examination so important?

    Answer

    • Overlap between clinical phenotypes and pathologies.
    • Clinical diagnosis of Alzheimer's disease isn't always matched by pathology.
    • Important for family diagnosis.
    • Aids in understanding the disease and response to treatments.

    Learning Outcomes

    • Correctly interpret a clinical history of a patient with MS
    • Provide appropriate key investigations for a patient suspected of having MS.
    • Describe the pathology and physiologic effects of demyelination & why they cause neurological symptoms
    • Distinguish and demonstrate clinical differences between delirium and dementia
    • Describe key investigations for cortical neurodegenerative disease
    • Describe macroscopic and microscopic features seen in the brain of a patient with Alzheimer's disease.

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    Related Documents

    RCSI CNS 2 Neurodegeneration/Demyelination PDF

    Description

    Test your knowledge on neurodegeneration and demyelination with this quiz from the Year 2 Pathology CPC course. Analyze a clinical scenario involving a patient with possible multiple sclerosis and answer key questions about presentation and symptoms. This quiz will enhance your understanding of important concepts in neuro pathology.

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