subcortical heterotopia

Subcortical heterotopia is a form of nodular grey matter heterotopia. They can be divided into:

  • nodular form: extend from the ventricle into the white matter
  • curvilinear form: extend from the cortex into the underlying white matter
  • mixed form

Terminology

The term laminar heterotopia is sometimes used ambiguously to refer to some of these patients . It is important to note that these patients should not be confused with lissencephaly type I-subcortical band heterotopia spectrum which is distinct on both imaging and at an underlying genetic level.

Clinical presentation

Epilepsy is very common and typically presents in childhood . It is usually associated with variable developmental delay .

Radiographic features

Subcortical heterotopias are continuous with the overlying cortex or underlying ventricle  and are associated with severe abnormalities of the involved hemisphere, characterized by :

  • diffuse reduction in size of the hemisphere
  • distorted ventricles
  • diminished and abnormal white matter
  • thinned overlying cortex with shallow sulci
  • distorted basal ganglia

These features are helpful in distinguishing this entity from subependymal grey matter heterotopia and lissencephaly type I-subcortical band heterotopia spectrum.

Siehe auch: