Neurodegenerative Disorders Overview
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Which of the following best describes neurodegenerative disorders (NDDs)? A. Acute, reversible conditions caused by infections. B. Chronic, progressive disorders leading to neuronal dysfunction and death. C. Disorders caused exclusively by hereditary mutations. D. Conditions characterized by reversible neuronal damage without cell death.

  • Acute, reversible conditions caused by infections.
  • Chronic, progressive disorders leading to neuronal dysfunction and death. (correct)
  • Disorders caused exclusively by hereditary mutations.
  • Conditions characterized by reversible neuronal damage without cell death.

    • What is a common pathological hallmark of neurodegenerative disorders?

  • Hyperactivation of immune cells in the peripheral blood.
  • Accumulation of aggregated proteins that can induce harmful signaling. (correct)
  • Localized inflammation in skeletal muscles.
  • Excessive regeneration of neurons in affected regions.

    • Which of the following is NOT true about neurodegenerative disorders? A. They are chronic and progressive in nature. B. Sporadic forms are more common than hereditary forms. C. They exclusively affect motor neurons. D. Aggregated proteins can directly harm neurons.

  • They are chronic and progressive in nature.
  • Sporadic forms are more common than hereditary forms.
  • They exclusively affect motor neurons. (correct)
  • Aggregated proteins can directly harm neurons.

    • Which of the following pairs a neurodegenerative disorder with its most commonly affected brain region correctly? A. Alzheimer’s disease – Basal ganglia B. Parkinson’s disease – Substantia nigra C. Huntington’s disease – Hippocampus D. Amyotrophic lateral sclerosis – Cerebellum

    <p>Parkinson’s disease – Substantia nigra</p> Signup and view all the answers

    Which of these proteins is associated with Alzheimer’s disease?

    <p>Beta-amyloid</p> Signup and view all the answers

    Which brain region is first affected in Alzheimer’s disease (AD)?

    <p>Cerebral cortex, basal ganglia, hippocampus</p> Signup and view all the answers

    Which neurodegenerative disorder is characterized by weakness and paralysis due to motor neuron involvement?

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

    What is the first brain region affected in Parkinson's disease (PD)?

    <p>Basal ganglia and midbrain (substantia nigra)</p> Signup and view all the answers

    Creutzfeldt-Jakob disease (CJD) commonly affects which combination of brain regions?

    <p>Cerebral cortex, basal ganglia, cerebellum</p> Signup and view all the answers

    Which neurodegenerative disorder is most associated with uncontrolled movements due to striatal involvement?

    <p>Huntington’s Disease</p> Signup and view all the answers

    Dementia is a prominent clinical feature of which of the following disorders? (Select one)

    <p>Creutzfeldt-Jakob Disease and Alzheimer’s Disease</p> Signup and view all the answers

    Elective neuronal vulnerability in neurodegenerative disorders means:

    <p>Specific neuronal populations are affected first based on the disease.</p> Signup and view all the answers

    Which neurodegenerative disorder is the leading cause of dementia?

    <p>Alzheimer’s disease</p> Signup and view all the answers

    What is one hallmark of neurodegenerative diseases?

    <p>Many involve the accumulation of aggregating proteins.</p> Signup and view all the answers

    Which of the following is a modifiable risk factor for dementia, according to the Lancet Commission on Dementia prevention (2020)?

    <p>Physical inactivity</p> Signup and view all the answers

    Which of the following is NOT a hallmark of Alzheimer's disease (AD)?

    <p>Cortical thickening</p> Signup and view all the answers

    Neurofibrillary tangles (NFTs) in Alzheimer's disease consist of: A. Insoluble fibrils inside neurons made up of abnormal Tau protein B. Extracellular amyloid deposits C. Accumulations of Tau in the synaptic cleft D. Disrupted neurofilaments in the axons of neurons

    <p>Insoluble fibrils inside neurons made up of abnormal Tau protein</p> Signup and view all the answers

    What is the function of Tau protein in a normal neuron?

    <p>To help stabilize microtubules for intracellular transport</p> Signup and view all the answers

    What happens to the microtubule structure in Alzheimer's disease?

    <p>It collapses due to abnormally phosphorylated Tau protein (p-Tau).</p> Signup and view all the answers

    Which of the following best describes brain atrophy in Alzheimer’s disease?

    <p>It is characterized by cortical thinning, especially in the hippocampus and cerebral cortex.</p> Signup and view all the answers

    Which of the following neurodegenerative disorders is NOT associated with Tau protein deposition?

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

    What is the primary characteristic of Tauopathies?

    <p>Deposition of abnormal Tau protein in the brain</p> Signup and view all the answers

    Which of the following disorders is associated with β-amyloid plaques?

    <p>Alzheimer's disease</p> Signup and view all the answers

    What is a characteristic feature of β-amyloid plaques in Alzheimer's disease?

    <p>They cause a local inflammatory tissue response.</p> Signup and view all the answers

    β-amyloid is a cleavage product of which larger protein?

    <p>Amyloid precursor protein (APP)</p> Signup and view all the answers

    What is the role of Amyloid Precursor Protein (APP) in the brain under normal conditions?

    <p>It plays a role in neural growth and repair.</p> Signup and view all the answers

    Which of the following genetic mutations is associated with frontotemporal dementia with Parkinsonism (FTDP-17)?

    <p>Mutations on chromosome 17</p> Signup and view all the answers

    Which of the following describes the normal processing of Amyloid Precursor Protein (APP)?

    <p>It is cleaved by α-secretase and γ-secretase, producing non-amyloidogenic fragments.</p> Signup and view all the answers

    In Alzheimer's disease, the abnormal processing of APP leads to the production of which form of β-amyloid?

    <p>Ab1-42</p> Signup and view all the answers

    Which secretase is responsible for cleaving APP in the extracellular segment, leading to the formation of the amyloidogenic form of β-amyloid in Alzheimer’s disease?

    <p>β-secretase</p> Signup and view all the answers

    What is the primary consequence of the production of the amyloidogenic form of β-amyloid (Aβ1-42) in Alzheimer's disease?

    <p>It leads to the formation of amyloid plaques, causing neuroinflammation.</p> Signup and view all the answers

    Which fragment is NOT a product of normal APP processing?

    <p>Ab1-42</p> Signup and view all the answers

    In Alzheimer's disease, β-secretase cleaves APP in which region of the protein?

    <p>Extracellular segment</p> Signup and view all the answers

    Which segment of APP is cleaved by γ-secretase in Alzheimer's disease to generate β-amyloid?

    <p>Transmembrane segment</p> Signup and view all the answers

    Which enzyme is responsible for cleaving APP in the normal processing pathway?

    <p>α-secretase</p> Signup and view all the answers

    Familial Alzheimer's Disease (FAD) typically presents with an onset of symptoms at what age?

    <p>&lt;60 years</p> Signup and view all the answers

    Which of the following mutations or genetic abnormalities is associated with Familial Alzheimer's Disease (FAD)?

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

    Familial Alzheimer's Disease (FAD) is inherited in which manner?

    <p>Autosomal dominant</p> Signup and view all the answers

    Children of a parent with Familial Alzheimer's Disease (FAD) have what chance of inheriting the condition?

    <p>50%</p> Signup and view all the answers

    The most common mutations in Familial Alzheimer's Disease (FAD) are found in which gene?

    <p>PSEN1 gene</p> Signup and view all the answers

    In Down’s syndrome, there is an increased risk of developing Familial Alzheimer's Disease (FAD) due to which genetic factor?

    <p>Extra copy of the APP gene</p> Signup and view all the answers

    PSEN1 and PSEN2 are genes that encode proteins that are essential for the function of which enzyme involved in Alzheimer’s pathology?

    <p>γ-secretase</p> Signup and view all the answers

    Which mutation is the most common cause of Familial Alzheimer's Disease (FAD)?

    <p>PSEN1</p> Signup and view all the answers

    Alzheimer's Disease (AD) accounts for what percentage of all dementia cases?

    <p>~70%</p> Signup and view all the answers

    Which of the following is considered the most important risk factor for the development of Alzheimer’s Disease (AD)?

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

    Alzheimer's Disease (AD) usually develops after which age?

    <blockquote> <p>65 years</p> </blockquote> Signup and view all the answers

    Which gene is associated with an increased risk of Alzheimer's Disease (AD)?

    <p>Apolipoprotein E (ApoE)</p> Signup and view all the answers

    Which Apolipoprotein E (ApoE) allele is most strongly associated with an increased risk of developing Alzheimer's Disease (AD)?

    <p>ApoE4</p> Signup and view all the answers

    Apolipoprotein E (ApoE) plays a role in which biological process that is linked to Alzheimer's Disease (AD)?

    <p>Lipid metabolism and neuronal repair</p> Signup and view all the answers

    Which of the following is true regarding Apolipoprotein E (ApoE) and Alzheimer’s Disease (AD)? A. ApoE4 allele decreases the risk of AD B. ApoE3 allele is associated with a neutral risk of AD C. ApoE2 allele increases the risk of AD D. ApoE4 allele is protective against AD

    <p>ApoE3 allele is associated with a neutral risk of AD</p> Signup and view all the answers

    Which of the following genetic mutations are commonly associated with Familial Alzheimer’s Disease (FAD)?

    <p>PSEN1, PSEN2, and APP</p> Signup and view all the answers

    Where is the APOE gene located?

    <p>Chromosome 19</p> Signup and view all the answers

    Which of the following APOE alleles is most strongly associated with a lower risk of Alzheimer's Disease (AD)?

    <p>APOE ε2</p> Signup and view all the answers

    Which APOE allele is the most common and has a neutral correlation with the risk of Alzheimer's Disease?

    <p>APOE ε3</p> Signup and view all the answers

    Having one or two copies of the APOE ε4 allele increases the risk of developing Alzheimer's Disease (AD). What is the primary function of APOE?

    <p>Cholesterol transport</p> Signup and view all the answers

    Which APOE allele is associated with the highest risk of developing Alzheimer's Disease?

    <p>APOE ε4</p> Signup and view all the answers

    Which genetic factor is most strongly correlated with an increased risk of developing Alzheimer's Disease (AD)?

    <p>Duplications of the APOE ε4 allele</p> Signup and view all the answers

    How many alleles of APOE exist, and which of the following is not one of them?

    <p>APOE ε1</p> Signup and view all the answers

    Which of the following mutations or genetic changes increase the risk of developing Alzheimer's Disease (AD)?

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

    Which allele of APOE is considered the rarest and is associated with a lower risk of Alzheimer’s Disease?

    <p>APOE ε2</p> Signup and view all the answers

    Which of the following is typically seen in the mild stage of Alzheimer's Disease (AD)?

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

    During the moderate stage of Alzheimer's Disease (AD), which symptom is most commonly observed?

    <p>Difficulty with personal care tasks like dressing</p> Signup and view all the answers

    Which of the following is a severe symptom in Alzheimer's Disease (AD)? A. Trouble with routine tasks B. Anxiety, agitation, and paranoia C. Loss of speech and appetite D. Personality changes

    <p>Loss of speech and appetite</p> Signup and view all the answers

    What is typically observed during the prodromal (early) stage of Alzheimer's Disease (AD)?

    <p>Difficulty with routine tasks and getting lost in familiar places</p> Signup and view all the answers

    Which of the following is a common symptom seen in the moderate stage of Alzheimer's Disease (AD)?

    <p>Insomnia or sleep disturbances</p> Signup and view all the answers

    What symptom is typically observed in the severe stage of Alzheimer's Disease (AD)?

    <p>Complete dependence on caregiver</p> Signup and view all the answers

    In the mild stage of Alzheimer's Disease (AD), individuals often experience which of the following symptoms? (Select one)

    <p>Personality changes and confusion</p> Signup and view all the answers

    In Alzheimer's Disease, which of the following symptoms might appear in the severe stage? (Select all that apply)

    <p>Loss of bladder and bowel control</p> Signup and view all the answers

    Which of the following is a prodromal (early) symptom of Alzheimer's Disease (AD)?

    <p>Getting lost in familiar places</p> Signup and view all the answers

    Which of the following is not a reason why early diagnosis of Alzheimer’s Disease (AD) is important?

    <p>Clinical symptoms improve over time with early diagnosis</p> Signup and view all the answers

    In Alzheimer's Disease, which biomarker change can be identified before clinical symptoms appear?

    <p>Changes in Tau and β-amyloid levels</p> Signup and view all the answers

    The preclinical phase of Alzheimer's Disease can last for how many years before the clinical phase begins?

    <p>15–30 years</p> Signup and view all the answers

    Which of the following is a common method used to detect early biomarkers for Alzheimer's Disease?

    <p>Genetic screening</p> Signup and view all the answers

    Which imaging technique can be used to identify early biomarkers of Alzheimer's Disease?

    <p>PET scans and MRI</p> Signup and view all the answers

    Which phase of Alzheimer's Disease is characterized by identifiable changes in biomarkers such as Aβ42 and p-Tau?

    <p>Preclinical phase</p> Signup and view all the answers

    Which of the following is not typically involved in the diagnosis of Alzheimer's Disease?

    <p>Genetic testing for all patients</p> Signup and view all the answers

    What is the primary focus of acetylcholinesterase (AChE) inhibitors in the pharmacological treatment of Alzheimer's Disease?

    <p>Address loss of cholinergic neurotransmission</p> Signup and view all the answers

    Which of the following drugs is an example of an acetylcholinesterase (AChE) inhibitor used to treat Alzheimer's Disease?

    <p>Both B and C</p> Signup and view all the answers

    What is the primary goal of NMDA receptor antagonists like Memantine in treating Alzheimer's Disease?

    <p>To block excitotoxicity caused by glutamate overactivation</p> Signup and view all the answers

    Which of the following is true about the benefits of acetylcholinesterase inhibitors in Alzheimer's Disease treatment?

    <p>They improve cognition, behavior, and functional ability in about 20% of patients</p> Signup and view all the answers

    Which diagnostic method is used to detect the presence of amyloid plaques and neurofibrillary tangles post-mortem in Alzheimer's Disease?

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

    What is the response rate for Memantine, an NMDA receptor antagonist, in improving cognition, social behavior, and functional ability in Alzheimer's patients?

    <p>60-70%</p> Signup and view all the answers

    Which of the following is typically used in the diagnosis of Alzheimer's Disease to exclude other conditions?

    <p>Standard laboratory tests</p> Signup and view all the answers

    Which of the following best describes prion diseases?

    <p>They are transmissible, untreatable, fatal brain diseases</p> Signup and view all the answers

    What is the characteristic feature of prion diseases?

    <p>Accumulation of misfolded prion protein</p> Signup and view all the answers

    Which of the following is the most common prion disease in humans?

    <p>Creutzfeldt-Jakob Disease (CJD)</p> Signup and view all the answers

    Which of the following is true regarding the prognosis of prion diseases? A. They are treatable with antiviral medications B. They are progressive, and there is currently no cure C. They only affect animals and not humans D. They can be fully reversed with early treatment

    <p>They are progressive, and there is currently no cure</p> Signup and view all the answers

    What is the normal prion protein (PrPc) known for? (Select one)

    <p>Having an unknown physiological function</p> Signup and view all the answers

    The abnormal, misfolded form of the prion protein (PrPSc) is:

    <p>Infectious, insoluble, and cannot be eradicated by sterilization</p> Signup and view all the answers

    What is the mechanism by which prions cause disease?

    <p>They cause a chain reaction of misfolding other PrP proteins</p> Signup and view all the answers

    Which of the following is true about Creutzfeldt-Jakob Disease (CJD)? A. It is a common form of childhood dementia B. It is a rare form of adult dementia with a rapidly progressive course C. It has a known and effective treatment D. It is primarily caused by environmental factors

    <p>It is a rare form of adult dementia with a rapidly progressive course</p> Signup and view all the answers

    Which of the following is not a typical symptom of Creutzfeldt-Jakob Disease (CJD)? A. Jerky movements and limb stiffness B. Loss of memory and mood changes C. Development of spongiform plaques in the brain D. Progressive hearing loss

    <p>Progressive hearing loss</p> Signup and view all the answers

    What characteristic feature is seen in the brains of individuals with Creutzfeldt-Jakob Disease (CJD)?

    <p>Spongiform degeneration with holes</p> Signup and view all the answers

    Which of the following is the most common outcome for people diagnosed with Creutzfeldt-Jakob Disease (CJD)? A. Complete recovery within a few months B. Death within 6 months of symptom onset C. Gradual symptom progression over many years D. Spontaneous remission of symptoms

    <p>Death within 6 months of symptom onset</p> Signup and view all the answers

    What is the cause of death in most cases of Creutzfeldt-Jakob Disease (CJD)?

    <p>Sepsis due to pneumonia</p> Signup and view all the answers

    Creutzfeldt-Jakob Disease (CJD) is considered:

    <p>A transmissible, untreatable, and fatal disease</p> Signup and view all the answers

    What is the main cause of Creutzfeldt-Jakob Disease (CJD)?

    <p>Misfolded prion protein (PrPSc)</p> Signup and view all the answers

    Huntington's Disease (HD) is caused by a mutation in which protein?

    <p>Huntingtin (Htt) protein</p> Signup and view all the answers

    In Huntington's Disease (HD), what is the primary result of the mutation in the Huntingtin (Htt) protein?

    <p>Aggregation of polyglutamine repeats</p> Signup and view all the answers

    What is the key feature of Huntington's Disease (HD) related to movement?

    <p>Chorea (abnormal, involuntary movements)</p> Signup and view all the answers

    How is the age of onset of Huntington's Disease (HD) related to the genetic mutation?

    <p>Later onset is associated with longer CAG repeats</p> Signup and view all the answers

    In Huntington's Disease (HD), the CAG repeat mutation in the Huntingtin gene leads to which of the following?

    <p>Chain of glutamines (polyglutamine repeats)</p> Signup and view all the answers

    Amyotrophic Lateral Sclerosis (ALS) is also known as:

    <p>Lou Gehrig’s disease</p> Signup and view all the answers

    Which type of neurons are primarily affected in Amyotrophic Lateral Sclerosis (ALS)?

    <p>Upper and lower motor neurons</p> Signup and view all the answers

    Which of the following mutations is associated with familial Amyotrophic Lateral Sclerosis (ALS)?

    <p>Mutations in the SOD gene</p> Signup and view all the answers

    Which protein is frequently associated with the genetic mutations seen in Amyotrophic Lateral Sclerosis (ALS)?

    <p>TDP-43 (RNA processing protein)</p> Signup and view all the answers

    Amyotrophic Lateral Sclerosis (ALS) primarily leads to which type of dysfunction?

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

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