Podcast
Questions and Answers
What is the most likely diagnosis for a 31-year-old lady with weakness and numbness in her left leg and a history of painful blurred vision in her right eye?
What is the most likely diagnosis for a 31-year-old lady with weakness and numbness in her left leg and a history of painful blurred vision in her right eye?
- Stroke
- Amyotrophic Lateral Sclerosis
- Multiple Sclerosis (correct)
- Peripheral Neuropathy
Which of the following is an unusual sign of demyelination associated with Multiple Sclerosis?
Which of the following is an unusual sign of demyelination associated with Multiple Sclerosis?
- Lhermitte’s sign (correct)
- Trigeminal neuralgia
- Bladder dysfunction
- Gait disturbance
How would you categorize a case of Multiple Sclerosis that began with one clinical episode and later progressed?
How would you categorize a case of Multiple Sclerosis that began with one clinical episode and later progressed?
- Clinically isolated syndrome
- Relapsing-remitting MS (correct)
- Secondary progressive MS
- Primary progressive MS
Which investigation is primarily used to support the diagnosis of Multiple Sclerosis?
Which investigation is primarily used to support the diagnosis of Multiple Sclerosis?
What is the name given to the phenomenon where raised body temperature exacerbates symptoms in Multiple Sclerosis?
What is the name given to the phenomenon where raised body temperature exacerbates symptoms in Multiple Sclerosis?
What specific finding in CSF analysis supports a diagnosis of Multiple Sclerosis?
What specific finding in CSF analysis supports a diagnosis of Multiple Sclerosis?
Which symptom is commonly associated with optic neuritis in Multiple Sclerosis?
Which symptom is commonly associated with optic neuritis in Multiple Sclerosis?
Which of the following clinical features is seen in a neurological examination of a patient with Multiple Sclerosis?
Which of the following clinical features is seen in a neurological examination of a patient with Multiple Sclerosis?
What name is given to the diagnostic criteria for multiple sclerosis (MS)?
What name is given to the diagnostic criteria for multiple sclerosis (MS)?
What pathologic process is occurring in the brain during an MS relapse?
What pathologic process is occurring in the brain during an MS relapse?
How does demyelination affect axonal transport?
How does demyelination affect axonal transport?
Which cell type is responsible for myelination in the central nervous system (CNS)?
Which cell type is responsible for myelination in the central nervous system (CNS)?
What is one of the management goals for a patient with multiple sclerosis?
What is one of the management goals for a patient with multiple sclerosis?
Which type of treatment is typically used for acute management of MS?
Which type of treatment is typically used for acute management of MS?
What is a characteristic change in the brain due to the progressive phase of multiple sclerosis?
What is a characteristic change in the brain due to the progressive phase of multiple sclerosis?
Which symptom is commonly managed within a multidisciplinary team for MS patients?
Which symptom is commonly managed within a multidisciplinary team for MS patients?
What defines delirium?
What defines delirium?
Which symptom is NOT associated with the man's delirium?
Which symptom is NOT associated with the man's delirium?
What part of the brain is primarily affected in memory impairment?
What part of the brain is primarily affected in memory impairment?
What is a characteristic feature of dementia?
What is a characteristic feature of dementia?
What investigation is most important to exclude acute reversible causes of cognitive decline?
What investigation is most important to exclude acute reversible causes of cognitive decline?
Which of the following assessments may help identify affected cognitive domains?
Which of the following assessments may help identify affected cognitive domains?
What could memory loss in this case suggest aside from delirium?
What could memory loss in this case suggest aside from delirium?
Which of the following is NOT a step in investigating cognitive decline?
Which of the following is NOT a step in investigating cognitive decline?
What is the most reliable method to confirm a diagnosis of Alzheimer's disease?
What is the most reliable method to confirm a diagnosis of Alzheimer's disease?
Which biomarker is associated with Alzheimer's disease pathology?
Which biomarker is associated with Alzheimer's disease pathology?
What is a common symptom exhibited in the progression of Alzheimer's disease?
What is a common symptom exhibited in the progression of Alzheimer's disease?
What role does Tau protein play in Alzheimer's disease?
What role does Tau protein play in Alzheimer's disease?
Why is the distribution of amyloid plaques significant in Alzheimer's disease?
Why is the distribution of amyloid plaques significant in Alzheimer's disease?
What feature distinguishes Alzheimer's disease pathology from that of other dementias?
What feature distinguishes Alzheimer's disease pathology from that of other dementias?
Which of the following is a potential consequence of Tau depletion in nerve cells?
Which of the following is a potential consequence of Tau depletion in nerve cells?
What is the primary reason for conducting a postmortem examination in cases of Alzheimer's disease?
What is the primary reason for conducting a postmortem examination in cases of Alzheimer's disease?
What are the key components of the McDonald Criteria for diagnosing MS?
What are the key components of the McDonald Criteria for diagnosing MS?
Which combination of symptoms is indicative of demyelination in Multiple Sclerosis?
Which combination of symptoms is indicative of demyelination in Multiple Sclerosis?
Which pathologic changes are primarily observed during an active MS plaque?
Which pathologic changes are primarily observed during an active MS plaque?
How does demyelination specifically disrupt axonal conduction in the CNS?
How does demyelination specifically disrupt axonal conduction in the CNS?
What is a common differential diagnosis for a patient presenting with symptoms similar to multiple sclerosis?
What is a common differential diagnosis for a patient presenting with symptoms similar to multiple sclerosis?
What is the primary role of oligodendrocytes in the central nervous system?
What is the primary role of oligodendrocytes in the central nervous system?
Which one of the following investigations is most crucial in confirming the diagnosis of Multiple Sclerosis?
Which one of the following investigations is most crucial in confirming the diagnosis of Multiple Sclerosis?
What is the likely mechanism behind Uhtoff’s phenomenon in Multiple Sclerosis patients?
What is the likely mechanism behind Uhtoff’s phenomenon in Multiple Sclerosis patients?
What are the overall management goals for treating Multiple Sclerosis?
What are the overall management goals for treating Multiple Sclerosis?
In the context of Multiple Sclerosis, what does the term 'clinically isolated syndrome' refer to?
In the context of Multiple Sclerosis, what does the term 'clinically isolated syndrome' refer to?
Which treatment approach is typically NOT used for long-term management of MS?
Which treatment approach is typically NOT used for long-term management of MS?
Which symptom is least likely to be associated with Multiple Sclerosis?
Which symptom is least likely to be associated with Multiple Sclerosis?
What causes the characteristic brain atrophy associated with the progressive phase of Multiple Sclerosis?
What causes the characteristic brain atrophy associated with the progressive phase of Multiple Sclerosis?
Which symptom can be effectively managed by a multidisciplinary team in MS patients?
Which symptom can be effectively managed by a multidisciplinary team in MS patients?
What role do oligoclonal bands play in the diagnosis of Multiple Sclerosis?
What role do oligoclonal bands play in the diagnosis of Multiple Sclerosis?
Which clinical presentation is associated with Lhermitte’s sign in patients with Multiple Sclerosis?
Which clinical presentation is associated with Lhermitte’s sign in patients with Multiple Sclerosis?
Which of the following features distinguishes delirium from dementia?
Which of the following features distinguishes delirium from dementia?
What component of the cognitive assessment can help determine the affected cognitive domains in a patient?
What component of the cognitive assessment can help determine the affected cognitive domains in a patient?
Which anatomical structures in the brain are primarily associated with short-term memory impairment?
Which anatomical structures in the brain are primarily associated with short-term memory impairment?
Which of the following is NOT considered a progressive cognitive decline?
Which of the following is NOT considered a progressive cognitive decline?
In the investigation of cognitive decline, which test is least likely to indicate a reversible cause?
In the investigation of cognitive decline, which test is least likely to indicate a reversible cause?
Which of the following describes a characteristic symptom of dementia as opposed to delirium?
Which of the following describes a characteristic symptom of dementia as opposed to delirium?
Which investigation is often done last when assessing a patient for cognitive decline?
Which investigation is often done last when assessing a patient for cognitive decline?
What cognitive change in personality and memory is often initially attributed to aging by family members?
What cognitive change in personality and memory is often initially attributed to aging by family members?
What is the primary method to confirm a diagnosis of Alzheimer's disease postmortem?
What is the primary method to confirm a diagnosis of Alzheimer's disease postmortem?
Which of the following features is indicative of Alzheimer's disease pathology?
Which of the following features is indicative of Alzheimer's disease pathology?
Which biomarker alteration is most commonly associated with Alzheimer's disease?
Which biomarker alteration is most commonly associated with Alzheimer's disease?
What change in amyloid beta levels might indicate the progression of Alzheimer's disease?
What change in amyloid beta levels might indicate the progression of Alzheimer's disease?
Which clinical feature is most likely observed in advanced stages of Alzheimer's disease?
Which clinical feature is most likely observed in advanced stages of Alzheimer's disease?
What is a primary role of Tau protein in the context of Alzheimer's disease?
What is a primary role of Tau protein in the context of Alzheimer's disease?
In Alzheimer's disease research, what significance does the Pittsburgh Compound B (PiB) hold?
In Alzheimer's disease research, what significance does the Pittsburgh Compound B (PiB) hold?
What is one of the main macroscopic features observed during the diagnosis of Alzheimer's disease?
What is one of the main macroscopic features observed during the diagnosis of Alzheimer's disease?
Flashcards
Clinical Isolated Syndrome (CIS)
Clinical Isolated Syndrome (CIS)
A single episode of neurological dysfunction suggestive of MS, with subsequent MRI findings of demyelination, but no further attacks or symptoms.
Relapsing-remitting MS (RRMS)
Relapsing-remitting MS (RRMS)
The most common type of MS, characterized by relapses (periods of new or worsening symptoms) followed by periods of remission (when symptoms improve or disappear).
MRI Brain and Spine with contrast
MRI Brain and Spine with contrast
An imaging technique used to visualize the brain and spinal cord, identifying areas of inflammation and demyelination (characteristic features of MS).
CSF analysis
CSF analysis
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Oligoclonal Bands (OCBs)
Oligoclonal Bands (OCBs)
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Visual Evoked Potentials (VEPs)
Visual Evoked Potentials (VEPs)
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Lhermitte's sign
Lhermitte's sign
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Uhthoff's phenomenon
Uhthoff's phenomenon
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McDonald Criteria
McDonald Criteria
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MS relapse (active MS plaque)
MS relapse (active MS plaque)
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Demyelination
Demyelination
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Axonal Transport
Axonal Transport
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Oligodendrocyte
Oligodendrocyte
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Saltatory Conduction
Saltatory Conduction
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MS Treatment (acute)
MS Treatment (acute)
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MS Treatment (long-term)
MS Treatment (long-term)
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Delirium
Delirium
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Delirium Symptoms
Delirium Symptoms
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Dementia
Dementia
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Detecting Dementia
Detecting Dementia
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Cognitive Assessment
Cognitive Assessment
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Brain Imaging for Dementia
Brain Imaging for Dementia
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Memory Impairment
Memory Impairment
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Short-Term Memory
Short-Term Memory
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Alzheimer's Disease (AD)
Alzheimer's Disease (AD)
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CSF Analysis in AD
CSF Analysis in AD
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18F-FDG PET Scan
18F-FDG PET Scan
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Pittsburg Compound B (PiB) PET Scan
Pittsburg Compound B (PiB) PET Scan
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Autopsy Brain Examination
Autopsy Brain Examination
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Amyloid Plaques
Amyloid Plaques
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Tau Protein
Tau Protein
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Neurodegeneration
Neurodegeneration
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What is the most likely diagnosis in this case?
What is the most likely diagnosis in this case?
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What is Clinically Isolated Syndrome (CIS)?
What is Clinically Isolated Syndrome (CIS)?
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What are the McDonald Criteria?
What are the McDonald Criteria?
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What are Uhthoff's Phenomenon and Lhermitte's Sign?
What are Uhthoff's Phenomenon and Lhermitte's Sign?
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What are 3 investigations that support a diagnosis of MS?
What are 3 investigations that support a diagnosis of MS?
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What is Demyelination?
What is Demyelination?
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What is Oligoclonal Bands (OCBs)?
What is Oligoclonal Bands (OCBs)?
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Explain the importance of MRI Brain and Spine with contrast in diagnosis of MS
Explain the importance of MRI Brain and Spine with contrast in diagnosis of MS
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Active MS Plaque
Active MS Plaque
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Acute MS Treatment
Acute MS Treatment
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Long-Term MS Treatment
Long-Term MS Treatment
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Progressive Phase of MS
Progressive Phase of MS
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Cognitive Impairment
Cognitive Impairment
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MMSE (Mini-Mental State Examination)
MMSE (Mini-Mental State Examination)
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Progressive Cognitive Decline
Progressive Cognitive Decline
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Neurodegenerative Disease
Neurodegenerative Disease
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Study Notes
CNS 2 Neurodegeneration/Demyelination
- Course: Pathology CPC
- Year: 2
- Lecturer: Dr. Michael Farrell
- Date: November 22nd, 2023
Clinical Scenario
- Patient: 31-year-old female
- Symptoms: 4-day history of weakness and numbness in left leg, episode of painful blurred vision in right eye one year prior (resolved after a week)
- Medical History: No other past medical history; not on regular medications
- Examination: ↑ tone in left leg, power 3/5, brisk reflexes in left leg, ↓ pin prick and proprioception, pale right optic nerve
Questions
- What is the most likely diagnosis in this case?
- How else can this condition present?
- How would you categorise the patient's clinical syndrome of MS?
- Name 3 investigations that will support your clinical diagnosis?
- What name is given to the diagnostic criteria for MS?
- What pathologic process is going on in the brain during a MS relapse (i.e. active MS plaque)?
- How does demyelination affect axonal transport?
- What cell is responsible for myelination in the CNS?
- What types of treatment might be used for this patient?
- What are the management goals?
- What is the most likely clinical diagnosis?
- Over the next year he shows progressive decline, fails to recognise close family member, becomes withdrawn, totally dependent on others until death. How might a diagnosis be confirmed?
- A pathologic diagnosis of Alzheimer Type Pathology is made. What are the macroscopic and microscopic features?
- Why is this type of postmortem examination so important?
Answers
-
Multiple Sclerosis (MS): A clinical diagnosis supplemented by inflammation in the brain and/or spinal cord, which is dissociated in time and place.
-
Clinical presentation of MS: Visual loss, gait and lower limb weakness, facial pain, double vision, gait/balance disturbance, vertigo, bladder dysfunction, Lhermitte's sign (tingling or shock-like feeling when flexing the neck), Uhthoff's phenomenon (exacerbation of symptoms with raised body temperature).
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MS Classifications: Clinically isolated syndrome, Relapsing-remitting MS (most common initial type), secondary progressive MS, primary progressive MS
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MS Investigations: MRI brain & spine with contrast, CSF analysis and oligoclonal bands (OCBs), visual evoked potentials, delayed P100 potentials.
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MS Diagnostic Criteria: McDonald Criteria
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Active MS Plaque Pathophysiology: Demyelination, perivascular inflammation, parenchymal inflammation, presence of lymphocytes and macrophages.
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Demyelination & Axonal Transport: Impairment of saltatory conduction and axonal transport
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CNS Myelination Cell: Oligodendrocytes
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MS Treatment: Steroids (sometimes plasma exchange) for acute attacks; Disease-modifying agents (injectable, oral, and infusion medications) for long-term.
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MS Management Goals: Shorten relapse duration, prolong remissions, prevent progression, and improve quality of life.
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Most likely clinical diagnosis for progressively declining 70-year-old male: Alzheimer Disease.
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Confirming Alzheimer diagnosis: Autopsy (postmortem) brain examination.
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Macroscopic and microscopic features of Alzheimer's pathology: Amyloid plaques (ßA4), neurofibrillary tangles (tau protein).
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Importance of postmortem examinations in Alzheimer's and other neurodegenerative disorders: Overlap exists between clinical phenotypes and pathologies; clinical diagnosis may not always match pathologic diagnosis, crucial for family diagnosis and inheritance likelihood, contributes to understanding disease and response to treatments.
Learning Objectives
- Correctly interpret clinical histories
- Provide appropriate investigations for suspected MS
- Describe demyelination's pathology and physiology.
- Distinguish delirium and dementia differences.
- Describe cortical neurodegenerative disease investigations.
- Describe the macroscopic and microscopic brain features in Alzheimer's disease.
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