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Questions and Answers
What is a common characteristic of leukodystrophies?
What is a common characteristic of leukodystrophies?
- Typically present in older adults
- Insidious and progressive loss of cerebral function (correct)
- Associated with asymmetric changes on imaging studies
- Sudden loss of cerebral function
What is the main difference between leukodystrophies and demyelinating diseases?
What is the main difference between leukodystrophies and demyelinating diseases?
- Leukodystrophies typically present at older ages
- Demyelinating diseases are associated with symmetric changes on imaging studies
- Leukodystrophies are caused by single enzyme defects
- Leukodystrophies are associated with diffuse and symmetric changes on imaging studies (correct)
What is the genetic pattern of inheritance for Krabbe disease?
What is the genetic pattern of inheritance for Krabbe disease?
- Mitochondrial inheritance
- Autosomal dominant
- X-linked recessive
- Autosomal recessive (correct)
What is the enzyme deficiency in Krabbe disease?
What is the enzyme deficiency in Krabbe disease?
What is the substrate for the deficient enzyme in Krabbe disease?
What is the substrate for the deficient enzyme in Krabbe disease?
What is the product of the catabolism of galactocerebroside in normal individuals?
What is the product of the catabolism of galactocerebroside in normal individuals?
What is the primary function of the myelin sheath?
What is the primary function of the myelin sheath?
What is the term for diseases characterized by damage to the myelin sheath?
What is the term for diseases characterized by damage to the myelin sheath?
What is the significance of myelin in the central nervous system?
What is the significance of myelin in the central nervous system?
What is the main characteristic of leukodystrophies on imaging studies?
What is the main characteristic of leukodystrophies on imaging studies?
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