Dementia and Degenerative Disorders Overview
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Questions and Answers

Which feature is NOT associated with Parkinson's disease?

  • Pill-rolling tremor at rest
  • Shuffling gait
  • Chorea (correct)
  • Cogwheel rigidity
  • What is the genetic basis of Huntington's disease?

  • Autosomal dominant inheritance with CAG repeats (correct)
  • Monogenic inheritance on chromosome 14
  • X-linked inheritance pattern
  • Mitochondrial inheritance
  • What symptom combination is characteristic of Normal Pressure Hydrocephalus?

  • Urinary incontinence, catatonia, and dementia
  • Urinary incontinence, gait instability, and dementia (correct)
  • Seizures, loss of cognitive function, and hallucinations
  • Dementia, tremors, and rigidity
  • Which of the following statements about Lewy bodies is correct?

    <p>They are characteristic of Parkinson's and Lewy body dementia</p> Signup and view all the answers

    What effect does a lumbar puncture have on patients with Normal Pressure Hydrocephalus?

    <p>Improves symptoms</p> Signup and view all the answers

    Which type of neurons are primarily degenerated in Huntington's disease?

    <p>GABAergic neurons</p> Signup and view all the answers

    Which characteristic is typical for spongiform encephalopathy?

    <p>Intracellular spongy degeneration due to prion proteins</p> Signup and view all the answers

    What is a common cause of death among individuals with Huntington's disease?

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

    What is the primary feature of Alzheimer's disease that differentiates it from early cognitive decline?

    <p>Progressive disorientation and chronic memory loss</p> Signup and view all the answers

    Which allele is associated with an increased risk for Alzheimer's disease?

    <p>ε4 allele of apolipoprotein E</p> Signup and view all the answers

    Which of the following is a common cause of death in patients with Alzheimer's disease?

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

    What marks the morphological changes associated with Alzheimer's disease?

    <p>Cerebral atrophy with widening of sulci and dilation of ventricles</p> Signup and view all the answers

    Which of the following correctly describes neurofibrillary tangles in Alzheimer's disease?

    <p>Intracellular aggregates of hyperphosphorylated tau protein</p> Signup and view all the answers

    Which statement about Pick disease is correct?

    <p>Round aggregates of tau protein are found in cortical neurons.</p> Signup and view all the answers

    What clinical feature distinguishes vascular dementia from other types of dementia?

    <p>Multifocal infarction due to vascular issues</p> Signup and view all the answers

    What is the presumed method of diagnosis for Alzheimer's disease?

    <p>Clinical diagnosis after excluding other causes</p> Signup and view all the answers

    Study Notes

    Dementia and Degenerative Disorders

    • Characterized by neuron loss in gray matter, often due to protein accumulation damaging neurons.

    Alzheimer Disease (AD)

    • Degenerative cortical disease, most common dementia type.
    • Clinical features include: slow-onset memory loss (short-term to long-term), disorientation, loss of learned motor skills, language, behavioral/personality changes, and death from infection.
    • Usually sporadic in elderly with risk increasing with age.
    • ApoE alleles (particularly ε4) increase risk.
    • Early-onset AD occurs in familial cases with presenilin 1 and 2 mutations or Down syndrome.
    • Morphological features:
      • Cerebral atrophy with broadened sulci and widened ventricles.
      • Neuritic plaques: extracellular amyloid-beta (Aβ) aggregates from APP.
      • Neurofibrillary tangles: intracellular hyperphosphorylated tau protein aggregates.
    • Diagnosis: clinical and pathological correlations; confirmed by autopsy.

    Vascular Dementia

    • Multifocal infarction and injury due to hypertension, atherosclerosis, or vasculitis.
    • Second most common dementia cause.

    Pick Disease

    • Degenerative frontal and temporal cortex disease.
    • Characterized by Pick bodies (tau protein aggregates).
    • Early behavioral and language symptoms, progressing to dementia.

    Parkinson Disease

    • Degenerative loss of dopaminergic neurons in substantia nigra.
    • Clinical features ('TRAP'): tremor, rigidity, akinesia/bradykinesia, and postural instability.
    • Histological: loss of pigmented neurons and Lewy bodies.
    • Dementia is a late-stage common feature.

    Huntington Disease

    • Degeneration of GABAergic neurons in caudate nucleus.
    • Autosomal dominant disorder with expanded trinucleotide repeats (CAG).
    • Progressive chorea (involuntary movements), dementia, and depression.

    Normal Pressure Hydrocephalus

    • Increased CSF causing dilated ventricles.
    • Can cause dementia in adults, often idiopathic.
    • Wet, wobbly, and wacky symptoms (urinary incontinence, gait instability, and dementia).

    Spongiform Encephalopathy

    • Degenerative disease caused by prion protein.
    • Conversion of normal prion protein (PrPc) to misfolded prion protein (PrPSc).
    • Pathologic protein is not degradable leading to neuron and glial damage and vacuole formation.
    • Creutzfeldt-Jakob Disease (CJD) is most common, potentially sporadic or transmitted from infected human tissue.
    • Variant CJD is linked to bovine spongiform encephalopathy (BSE).

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    Description

    Explore the key aspects of dementia and degenerative disorders, focusing on Alzheimer's disease and vascular dementia. This quiz covers clinical features, risk factors, morphological changes, and diagnostic methods associated with these conditions. Enhance your understanding of how these disorders affect cognitive function and overall health.

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