What is the prognosis of Sturge-Weber syndrome (SWS)? In adults

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

Few data are available on adults with SWS. Sujansky and Conradi studied the outcomes in 52 adults older than 18 years who had SWS and were identified by the Sturge-Weber Foundation. [42] The age of onset of glaucoma ranged from 0-41 years, with a median of 5 years.

Seizures occurred in 83% of the patients, glaucoma in 60%, and a neurologic deficit—such as stroke, paralysis, spasticity, or weakness—in 65%. The age of onset of seizures ranged from 0-23 years, with a median of 6 months.

Seizure outcome was known in 41 patients in the study, with full control attained in 11 (27%) patients, a decrease in seizures achieved in (49%) 20 patients, and no improvement found in 10 (24%) patients. The morbid conditions associated with these seizures are listed in Table 2, below.

Headache occurred in 28 (62%) of 45 patients, with age of onset ranging from early childhood to age 38 years and with a median age of onset of 18 years. The headache frequency could be determined in 23 patients: daily in 9 patients (39%), 1-4 times per week in 4 (17%), 1-2 times per month in 6 (26%) patients, and rare in 4 patients (17%).

Headaches were associated with increased discoloration of facial PWS, auras, nausea/vomiting, dysarthria, dizziness, and feelings of facial pulsation.

Table 2. Developmental Morbidity Associated with Seizures in Adults with SWS (Open Table in a new window)

 

With Seizures (%)

Without Seizures (%)

Developmental delay

45

0

Emotional/behavioral problems

85

58

Need for special education

71

0

Employability

46

78


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