Linear Nevus syndrome

Question 1

What are the clinical manifestations of Linear Nevus syndrome?

Answer 1

1. Midline nevus over forehead and nose, initially faint then hyperkeratotic and then yellow-brown
2. 2/3 cortical dysplasia
3. glial harmatomas
4. low-grade gliomas
5. hemimegalencephaly
6. enlargement of lateral ventricles
7. Epilepsy 75%
8. Intellectual disability 60%
9. Hemiparesis
10. Homonymous hemianopia

Question 2

What is the inheritance of Linear Nevus syndrome?

Answer 2

Sporadic