Legius syndrome

Legius syndrome
Other namesNeurofibromatosis 1-like syndrome
This condition is inherited in an autosomal dominant manner.
Symptomscafé au lait spots; +/- learning disabilities
Usual onsetat birth
CausesMutations in the SPRED1 gene
Diagnostic methodClinical findings, Genetic test
Differential diagnosisneurofibromatosis type I
TreatmentPhysical therapy, Speech therapy
Prognosisgood
Frequencyrare (estimated at 1:46,000-1:75,000)

Legius syndrome (LS) is an autosomal dominant condition characterized by cafe au lait spots. It was first described in 2007 and is often mistaken for neurofibromatosis type I. It is caused by mutations in the SPRED1 gene. It is also known as neurofibromatosis type 1-like syndrome.