Neurodegenerative Disorders Quiz
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Questions and Answers

Which disease is characterized by gait ataxia, hand clumsiness, dysarthria, pes cavus, and kyphoscoliosis, with high incidence of cardiac disease and diabetes?

  • Amyotrophic lateral sclerosis (ALS)
  • Friedreich ataxia (correct)
  • Spinocerebellar ataxia
  • Ataxia telangiectasia
  • In which of the following diseases does cognitive changes such as memory disturbances, behavior, and language issues lead to dementia and may manifest as Alzheimer's disease or frontotemporal dementia?

  • Ataxia telangiectasia
  • Friedreich ataxia (correct)
  • Amyotrophic lateral sclerosis (ALS)
  • Spinocerebellar ataxia
  • Which disease affects the cerebellar cortex, spinal cord, and other brain regions variably, leading to ataxia?

  • Ataxia telangiectasia
  • Friedreich ataxia
  • Spinocerebellar ataxia (correct)
  • Amyotrophic lateral sclerosis (ALS)
  • Which disease is characterized by difficulty swallowing, respiratory issues, and muscle weakness due to involvement of the motor system?

    <p>Amyotrophic lateral sclerosis (ALS)</p> Signup and view all the answers

    Which genetic abnormality involving GAA trinucleotide repeat expansion is associated with Frataxin protein and regulation of mitochondrial iron?

    <p>Friedreich ataxia</p> Signup and view all the answers

    What disease is caused by CAG repeat expansions and involves intranuclear inclusions, among other mutations?

    <p>Huntington disease</p> Signup and view all the answers

    What is the primary cause of mitochondrial dysfunction in Friedreich's ataxia?

    <p>Decreased frataxin expression</p> Signup and view all the answers

    Which of the following is a characteristic feature of amyotrophic lateral sclerosis (ALS)?

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

    Which gene mutation is most commonly associated with familial forms of amyotrophic lateral sclerosis (ALS)?

    <p>SOD1</p> Signup and view all the answers

    Which of the following is a characteristic feature of Friedreich's ataxia?

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

    Which of the following is a common feature of both amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD)?

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

    Which of the following is a characteristic feature of ataxia telangiectasia?

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

    Which of the following is a characteristic feature of amyotrophic lateral sclerosis (ALS)?

    <p>Loss of anterior horn neurons and reactive gliosis</p> Signup and view all the answers

    Which of the following ataxias is associated with a deficiency in frataxin protein?

    <p>Friedreich ataxia</p> Signup and view all the answers

    In amyotrophic lateral sclerosis (ALS), which of the following is typically spared?

    <p>Extraocular muscles</p> Signup and view all the answers

    Which of the following is a characteristic feature of ataxia telangiectasia?

    <p>Deficiency in the ATM protein</p> Signup and view all the answers

    Which of the following is a common cause of cerebellar ataxia due to thiamine deficiency?

    <p>Chronic alcoholism</p> Signup and view all the answers

    In spinocerebellar ataxias, which of the following is commonly observed?

    <p>Degeneration of the spinocerebellar tracts</p> Signup and view all the answers

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