10 Questions
What is a common characteristic of leukodystrophies?
Insidious and progressive loss of cerebral function
What is the main difference between leukodystrophies and demyelinating diseases?
Leukodystrophies are associated with diffuse and symmetric changes on imaging studies
What is the genetic pattern of inheritance for Krabbe disease?
Autosomal recessive
What is the enzyme deficiency in Krabbe disease?
Galactocerebroside β-galactosidase
What is the substrate for the deficient enzyme in Krabbe disease?
Galactocerebroside
What is the product of the catabolism of galactocerebroside in normal individuals?
Galactose and ceramide
What is the primary function of the myelin sheath?
Insulation and conduction of nerve impulses
What is the term for diseases characterized by damage to the myelin sheath?
Demyelinating diseases
What is the significance of myelin in the central nervous system?
Myelin is necessary for normal brain function
What is the main characteristic of leukodystrophies on imaging studies?
Diffuse and symmetric changes
Test your knowledge of the Central Nervous System, including prion diseases, spongiform changes, and protein deposits in the brain. Assess your understanding of the cerebral cortex and cerebellar cortex. Evaluate your knowledge of Creutzfeldt-Jakob disease and its effects.
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