title: Stimulant use in patients with sturge-weber syndrome: safety and efficacy.
abstract: BACKGROUND
  Sturge-Weber syndrome is characterized by a facial port-wine birthmark, vascular eye abnormalities, and a leptomeningeal angioma. Attention and behavioral issues are common in Sturge-Weber syndrome. However, literature evidence for stimulant treatment is minimal. This study evaluates stimulant medication safety and efficacy in individuals with Sturge-Weber syndrome.
  
  
  METHODS
  The research database of the Hunter Nelson Sturge-Weber Center (n = 210 subjects in the database) was reviewed for stimulant use. Twelve patients (mean age 10.5 years, age range 4 to 21 years) on stimulants were seen between 2003 and 2012. A retrospective chart review obtained comorbid diagnoses, stimulant type and dosage, medication side effects, vital signs, and medication efficacy.
  
  
  RESULTS
  All 12 patients had brain involvement (unilateral, nine; bilateral, three). Additional comorbidities included epilepsy (twelve), hemiparesis (eight), headaches (eight), and vision deficits (six). Eight patients reported side effects, primarily appetite suppression (four) and headaches (three). There were no statistically significant changes in weight or blood pressure 6 months after medication initiation. Medication efficacy was subjectively reported in 11 patients. Seven patients remained on stimulants at their most recent follow-up visit.
  
  
  CONCLUSIONS
  This study preliminarily evaluates stimulant medication use in a small group of Sturge-Weber syndrome patients. Stimulants were tolerated and effective in most subjects. Side effects were mostly minor and medication did not negatively affect growth or vital signs. Stimulant medication may be a safe and effective intervention for Sturge-Weber syndrome children with attention issues/attention deficit hyperactivity disorder. Further studies with larger sample sizes are needed.
authors.affiliation: Department of Neurodevelopmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland; Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland; Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland.
authors.forename: Eboni I
authors.initials: EI
authors.lastname: Lance
authors.affiliation: Department of Neurodevelopmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland.
authors.forename: Kira E
authors.initials: KE
authors.lastname: Lanier
authors.affiliation: Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland.
authors.forename: T Andrew
authors.initials: TA
authors.lastname: Zabel
authors.affiliation: Department of Neurodevelopmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland; Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland; Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland. Electronic address: comi@kennedykrieger.org.
authors.forename: Anne M
authors.initials: AM
authors.lastname: Comi
mesh_terms: D000293:Adolescent; D001921:Brain / Q000187:drug effects* / Q000473:pathology; D000697:Central Nervous System Stimulants / Q000494:pharmacology / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual / Q000706:statistics & numerical data; D064420:Drug-Related Side Effects and Adverse Reactions / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D013341:Sturge-Weber Syndrome / Q000188:drug therapy* / Q000473:pathology; D055815:Young Adult
chemical_list: D000697:Central Nervous System Stimulants
keywords: ADHD; Sturge Weber syndrome; neurocutaneous syndromes; stimulant medication