Pathology CPC CNS 2: Neurodegeneration Quiz

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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?

Stroke

Amyotrophic Lateral Sclerosis

Multiple Sclerosis (correct)

Peripheral Neuropathy

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?

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?

MRI of Brain and Spine with contrast Signup and view all the answers

What is the name given to the phenomenon where raised body temperature exacerbates symptoms in Multiple Sclerosis?

Uhtoff's phenomenon Signup and view all the answers

What specific finding in CSF analysis supports a diagnosis of Multiple Sclerosis?

Oligoclonal bands Signup and view all the answers

Which symptom is commonly associated with optic neuritis in Multiple Sclerosis?

Visual loss Signup and view all the answers

Which of the following clinical features is seen in a neurological examination of a patient with Multiple Sclerosis?

Brisk reflexes in the affected limb Signup and view all the answers

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

McDonald Criteria Signup and view all the answers

What pathologic process is occurring in the brain during an MS relapse?

Demyelination Signup and view all the answers

How does demyelination affect axonal transport?

Impedes saltatory conduction Signup and view all the answers

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

Oligodendrocyte Signup and view all the answers

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

Shorten relapse duration Signup and view all the answers

Which type of treatment is typically used for acute management of MS?

Steroids Signup and view all the answers

What is a characteristic change in the brain due to the progressive phase of multiple sclerosis?

Brain atrophy Signup and view all the answers

Which symptom is commonly managed within a multidisciplinary team for MS patients?

Fatigue management Signup and view all the answers

What defines delirium?

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

Which symptom is NOT associated with the man's delirium?

Memory loss about the event Signup and view all the answers

What part of the brain is primarily affected in memory impairment?

The temporal lobe Signup and view all the answers

What is a characteristic feature of dementia?

Progressive decline in cognitive function Signup and view all the answers

What investigation is most important to exclude acute reversible causes of cognitive decline?

Urinalysis and urine culture Signup and view all the answers

Which of the following assessments may help identify affected cognitive domains?

Montreal Cognitive Assessment Signup and view all the answers

What could memory loss in this case suggest aside from delirium?

Possible dementia Signup and view all the answers

Which of the following is NOT a step in investigating cognitive decline?

Conducting routine physical exams only Signup and view all the answers

What is the most reliable method to confirm a diagnosis of Alzheimer's disease?

Autopsy (postmortem) brain examination Signup and view all the answers

Which biomarker is associated with Alzheimer's disease pathology?

Presence of beta-amyloid plaques Signup and view all the answers

What is a common symptom exhibited in the progression of Alzheimer's disease?

Total dependence on others Signup and view all the answers

What role does Tau protein play in Alzheimer's disease?

Stabilizes microtubules in neurons Signup and view all the answers

Why is the distribution of amyloid plaques significant in Alzheimer's disease?

They correlate with the level of cognitive decline. Signup and view all the answers

What feature distinguishes Alzheimer's disease pathology from that of other dementias?

Specific pattern of amyloid and tau accumulation Signup and view all the answers

Which of the following is a potential consequence of Tau depletion in nerve cells?

Failure of axonal elongation Signup and view all the answers

What is the primary reason for conducting a postmortem examination in cases of Alzheimer's disease?

To provide definitive diagnosis confirmation Signup and view all the answers

What are the key components of the McDonald Criteria for diagnosing MS?

Dissemination of CNS lesions in space and time through clinical and MRI findings Signup and view all the answers

Which combination of symptoms is indicative of demyelination in Multiple Sclerosis?

Weakness in lower limbs and facial pain Signup and view all the answers

Which pathologic changes are primarily observed during an active MS plaque?

Demyelination with perivascular and parenchymal inflammation Signup and view all the answers

How does demyelination specifically disrupt axonal conduction in the CNS?

By impairing saltatory conduction Signup and view all the answers

What is a common differential diagnosis for a patient presenting with symptoms similar to multiple sclerosis?

Guillain-Barré syndrome Signup and view all the answers

What is the primary role of oligodendrocytes in the central nervous system?

Forming myelin sheaths around multiple axons Signup and view all the answers

Which one of the following investigations is most crucial in confirming the diagnosis of Multiple Sclerosis?

Magnetic resonance imaging (MRI) with contrast Signup and view all the answers

What is the likely mechanism behind Uhtoff’s phenomenon in Multiple Sclerosis patients?

Demyelination causing overheating effects on nerve conduction Signup and view all the answers

What are the overall management goals for treating Multiple Sclerosis?

Shorten relapses and prolong remissions while improving quality of life Signup and view all the answers

In the context of Multiple Sclerosis, what does the term 'clinically isolated syndrome' refer to?

First episode of neurological symptoms lasting more than 24 hours without evidence of another cause Signup and view all the answers

Which treatment approach is typically NOT used for long-term management of MS?

Antibiotics to prevent infections Signup and view all the answers

Which symptom is least likely to be associated with Multiple Sclerosis?

Chronic cough Signup and view all the answers

What causes the characteristic brain atrophy associated with the progressive phase of Multiple Sclerosis?

Ongoing axonal loss and subsequent loss of white matter Signup and view all the answers

Which symptom can be effectively managed by a multidisciplinary team in MS patients?

Fatigue and urogenital problems Signup and view all the answers

What role do oligoclonal bands play in the diagnosis of Multiple Sclerosis?

Reflect an autoimmune process within the central nervous system Signup and view all the answers

Which clinical presentation is associated with Lhermitte’s sign in patients with Multiple Sclerosis?

Electric shock-like sensations down the spine during neck flexion Signup and view all the answers

Which of the following features distinguishes delirium from dementia?

Delirium has a rapid onset. Signup and view all the answers

What component of the cognitive assessment can help determine the affected cognitive domains in a patient?

Montreal Cognitive Assessment. Signup and view all the answers

Which anatomical structures in the brain are primarily associated with short-term memory impairment?

Hippocampus and surrounding cortices. Signup and view all the answers

Which of the following is NOT considered a progressive cognitive decline?

Major Depressive Disorder. Signup and view all the answers

In the investigation of cognitive decline, which test is least likely to indicate a reversible cause?

Mini-Mental State Examination. Signup and view all the answers

Which of the following describes a characteristic symptom of dementia as opposed to delirium?

Progressive decline of function over time. Signup and view all the answers

Which investigation is often done last when assessing a patient for cognitive decline?

Brain imaging (CT or MRI). Signup and view all the answers

What cognitive change in personality and memory is often initially attributed to aging by family members?

Short-term memory loss. Signup and view all the answers

What is the primary method to confirm a diagnosis of Alzheimer's disease postmortem?

Autopsy brain examination Signup and view all the answers

Which of the following features is indicative of Alzheimer's disease pathology?

Presence of neurofibrillary tangles Signup and view all the answers

Which biomarker alteration is most commonly associated with Alzheimer's disease?

Altered phosphorylated Tau levels Signup and view all the answers

What change in amyloid beta levels might indicate the progression of Alzheimer's disease?

A decrease in Aβ42 and an increase in Aβ40 Signup and view all the answers

Which clinical feature is most likely observed in advanced stages of Alzheimer's disease?

Complete loss of recognition of family members Signup and view all the answers

What is a primary role of Tau protein in the context of Alzheimer's disease?

Stabilizing microtubules in neurons Signup and view all the answers

In Alzheimer's disease research, what significance does the Pittsburgh Compound B (PiB) hold?

It labels amyloid plaques for imaging Signup and view all the answers

What is one of the main macroscopic features observed during the diagnosis of Alzheimer's disease?

Cortical atrophy Signup and view all the answers

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).
MS Classifications: Clinically isolated syndrome, Relapsing-remitting MS (most common initial type), secondary progressive MS, primary progressive MS
MS Investigations: MRI brain & spine with contrast, CSF analysis and oligoclonal bands (OCBs), visual evoked potentials, delayed P100 potentials.
MS Diagnostic Criteria: McDonald Criteria
Active MS Plaque Pathophysiology: Demyelination, perivascular inflammation, parenchymal inflammation, presence of lymphocytes and macrophages.
Demyelination & Axonal Transport: Impairment of saltatory conduction and axonal transport
CNS Myelination Cell: Oligodendrocytes
MS Treatment: Steroids (sometimes plasma exchange) for acute attacks; Disease-modifying agents (injectable, oral, and infusion medications) for long-term.
MS Management Goals: Shorten relapse duration, prolong remissions, prevent progression, and improve quality of life.
Most likely clinical diagnosis for progressively declining 70-year-old male: Alzheimer Disease.
Confirming Alzheimer diagnosis: Autopsy (postmortem) brain examination.
Macroscopic and microscopic features of Alzheimer's pathology: Amyloid plaques (ßA4), neurofibrillary tangles (tau protein).
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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Description

Test your knowledge on neurodegeneration and demyelination with this quiz focused on clinical scenarios in pathology. Explore multiple aspects of multiple sclerosis (MS), including diagnosis, presentations, investigations, and the underlying pathophysiology. This quiz is designed for second-year medical students and covers key topics presented in the Pathology CPC course.