Cerebral disease: degenerative, congenital, anomalous

Question 1

What is the prevalence of degenerative and congenital cerebral disease

Answer 1

These disorders are uncommon and are typically seen in young animals

Question 2

What are the two pathomechanisms leading to hydrocephalus

Answer 2

Hydrocephalus may be congenital or acquired secondary to lesions that obstruct the flow of CSF (e.g., tumors, FIP)

Question 3

What is the typical presentation of animals with congenital hydrocephalus

Answer 3

Animals with congenital hydrocephalus typically have:
- a domed shaped skull
- possibly an open fontanelle
- bilateral ventrolateral strabismus (divergent strabismus) due to malformation of the skull

Affected animals may be neurologically normal or have depressed mentation, cortical blindness and seizures

Question 4

What would be a possible treatment for hydrocephalus

Answer 4

Treatment is often unsuccessful but may consist of:
- corticosteroids to reduce production of CSF
- surgical shunting
- seizures can be treated with phenobarbitone

Question 5

What are the main facts about lysosomal storage diseases

Answer 5

These diseases are fortunately rare

Clinical signs are caused by the accumulation of substances that are usually metabolized by the deficient enzymes
- they are progressive
- they include ataxia, dementia, seizures and blindness
- they generally occur before the animal is 12 months of age

Most of these disorders have an autosomal recessive mode of inheritance and result in a deficiency or malfunction of key enzymes within the lysosomal catabolic pathway

Question 6

What are the subgroups used to oragnize storage diseases

Answer 6

Storage diseases are highly diverse and have been organized into subgroups based on the deranged metabolic pathway

These subgroups include:
- glycoproteinoses
- oligosaccaridoses
- sphingolipidoses
- mucopolysaccharidoses
- proteinoses

Question 7

What are the features common to all storage diseases

Answer 7

Features common to all storage diseases include:
- equal distribution between males and females
- a slowly progressive clinical course (normal at birth and in the first months of life)
- +/- a history of neonatal deaths within the litter