Phakomatoses are a group of neurocutaneous disorders characterised by involvement of structures that arise from the embryonic ectoderm (thus central nervous system, skin and eyes). Other organs may also be involved

Radiographic features

Please refer articles on individual lesions  for respective specific imaging features.

1. 
   meningioma
   
2. 
   schwannoma - usually inferior vestibular division of CN8
   
3. 
   ependymoma - usually spinal intramedullary (not intracranial/intraventricular)
   

Tuberous sclerosis (TS) (also known as tuberous sclerosis complex or Bourneville disease) is a neurocutaneous disorder (phakomatosis) characterized by development of multiple benign tumours of the embryonic ectoderm, e.g. skin, eyes, nervous system.

Epidemiology

Tuberous sclerosis has an incidence of 1 in 6000 to 12,000, with most being sporadic (see below) ⁶.

Clinical presentation

Tuberous sclerosis was classically described as presenting in childhood with a triad of :

1. 
   seizures: absent in 1/4 of individuals
   
2.