What is the prevalence of Sturge-Weber syndrome (SWS) in the US?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
  • Print
Answer

According to the National Organization of Rare Disorders, Sturge-Weber Syndrome (SWS) occurs in one of every estimated 20,000 to 50,000 live births. [31]  The inheritance is sporadic, and no regional differences in incidence have been identified. The incidences of the major clinical manifestations of SWS are listed in Table 1.

Table 1. Clinical Manifestations of Sturge-Weber Syndrome (Open Table in a new window)

Clinical Manifestation

Incidence Rate

Risk of SWS with facial PWS

8%

SWS without facial nevus

13%

Bilateral cerebral involvement

15%

Seizures

72-93%

Hemiparesis

25-56%

Hemianopia

44%

Headaches

44-62%

Developmental delay and mental retardation

50-75%

Glaucoma

30-71%

Choroidal hemangioma

40%


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!