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) (D)</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 (B)</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 (A)</p> Signup and view all the answers

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

<p>Decreased frataxin expression (B)</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 (D)</p> Signup and view all the answers

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

<p>SOD1 (B)</p> Signup and view all the answers

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

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

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

<p>All of the above (D)</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 (B)</p> Signup and view all the answers

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

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

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

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

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

<p>Deficiency in the ATM protein (C)</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 (B)</p> Signup and view all the answers

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

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

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