Schwannomatosis & Legius Syndrome

Other conditions related to Neurofibromatosis include, Schwannomatosis and Legius Syndrome.

Schwannomatosis

This is a very rare condition. It shares some features of NF2 but not hearing loss.

Doctors working in the field of Neurofibromatosis (NF) now regard schwannomatosis as the third main type of NF. The clinical problems that can occur overlap with Neurofibromatosis type 2 (NF2). Both conditions are associated with the growth of a type of benign tumour called a schwannoma.

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Legius Syndrome or SPRED1

Legius syndrome is named after the Belgian doctor Professor Eric Legius who first identified it as a distinct condition. It is also called SPRED1 because that is the location of the genetic “spelling mistake” that gives rise to this condition.

Legius syndrome has some similarities with Neurofibromatosis type 1 but as molecular genetic testing has developed, it became apparent that this is a separate and different condition.

NF1 is located on chromosome 17. Legius syndrome is located on chromosome 15.

Legius syndrome is a dominant condition which means there is a 50% chance of an affected parent passing it on to each of their children.

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