Malformations of cortical development

Question 1

What caused this?

Answer 1

periventricular nodular heterotopia (PVNH)

Question 2

How to differentiate Periventricular nodular heterotopia (5)

Answer 2

Question 3

What are these images showing

Answer 3

Focal cortical dysplasia:
Left: Thickened cortex (T2 FLAIR)
Right: concordant region of hypometabolism

Question 4

Patient with FCDs on MRI. What factors may suggest a genetic cause (and which gene)

Answer 4

X-linked FNLA (Filamin A)
More commonly Female
More commonly bilateral

Question 5

FCD classification:
- Why do we do it?
- Type I
- Type II:
- Type III

Answer 5

• More severe pathology has better prognosis (possibly due to being seen on MRI more easily
• Type I
  
  • “mycrodysgenesis
  • May not be seen on MRI
• Type II:
  
  • Most severe
  • Type IIb (baloon cells)
• Type III
  
  • dual pathology
  • associated with other lesions (such as mesial temporal sclerosis