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Chapter 28: The Central Nervous System

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Questions and Answers

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?

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?

Mitochondrial inheritance

Autosomal dominant

X-linked recessive

Autosomal recessive (correct)

What is the enzyme deficiency in Krabbe disease?

Galactocerebroside β-galactosidase Signup and view all the answers

What is the substrate for the deficient enzyme in Krabbe disease?

Galactocerebroside Signup and view all the answers

What is the product of the catabolism of galactocerebroside in normal individuals?

Galactose and ceramide Signup and view all the answers

What is the primary function of the myelin sheath?

Insulation and conduction of nerve impulses Signup and view all the answers

What is the term for diseases characterized by damage to the myelin sheath?

Demyelinating diseases Signup and view all the answers

What is the significance of myelin in the central nervous system?

Myelin is necessary for normal brain function Signup and view all the answers

What is the main characteristic of leukodystrophies on imaging studies?

Diffuse and symmetric changes Signup and view all the answers