Stimulant Use in Patients With Sturge-Weber Syndrome: Safety and Efficacy

Abstract

BACKGROUND Sturge Weber Syndrome (SWS) is characterized by a facial port-wine birthmark, vascular eye abnormalities, and a leptomeningeal angioma. Attention and behavioral issues are common in SWS; however, literature evidence for stimulant treatment is minimal. This study evaluates stimulant medication safety and efficacy in SWS patients. METHODS The research database of the Hunter Nelson Sturge-Weber Center (n = 210 subjects with SWS brain involvement) was reviewed for stimulant use. Twelve subjects (mean age 10.5 years, age range 4 to 21 years) on stimulants were seen between 2003 and 2012. A retrospective chart review obtained co-morbid diagnoses, stimulant type and dosage, medication side effects, vital signs, and medication efficacy. RESULTS All twelve subjects had brain involvement (unilateral - nine; bilateral – three). Additional co-morbidities included epilepsy (twelve), hemi-paresis (eight), headaches (eight), and vision deficits (seven). Eight subjects reported side effects, primarily appetite suppression (four) and headaches (three). There were no statistically significant changes in weight or blood pressure six months after medication initiation. Medication efficacy was subjectively reported in eleven subjects. 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 SWS patients. Stimulants were tolerated and effective in most subjects. Side effects were mostly minor and medication did not negatively impact growth or vital signs. Stimulant medication may be a safe and effective intervention for SWS children with attention issues/attention deficit hyperactivity disorder (ADHD). Further studies with larger sample sizes are needed.

A Multidisciplinary Consensus for Clinical Care and Research Needs for Sturge-Weber Syndrome

“…7 Attention deficit hyperactivity disorder (ADHD) occurs in about 40% of patients with SWS. 30 In another study, stimulants were subjectively assessed to be effective ADHD treatments in SWS with minimal side effects. 30 Neuropsychological testing beginning at age 3 or 4 years can be helpful in identifying high-risk children 31 so that proper interventions, including early initiation of an individualized education plan and behavioral therapy, can be implemented.…”

Sturge-Weber Syndrome

“…Patients with cognitive deficits and attention problems can benefit from specialized education services, behavioral psychology intervention, and stimulant use. Stimulants have been shown to positively impact attention problems in SWS patients [55]. To address hemiparesis, physical and occupational therapy can be incorporated at an early age to improve motion and fine motor skills; some work with constraint therapy has been done with these patients and has been shown to be helpful [56].…”

Headache in Sturge–Weber syndrome: A systematic review

“…Finally, 48 articles met all the inclusion criteria and were included in the systematic review. Among these, 37 were case reports or case series , seven were retrospective observational studies based on medical records (45)(46)(47)(48)(49)(50)(51) and four studies were conducted using observational questionnaires (52)(53)(54)(55). The flow diagram of the study selection process according to the PRISMA guidelines is shown in Figure 1.…”