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.

Introduction

Major clinical features of Sturge-Weber syndrome (SWS) include seizures, stroke-like episodes, and glaucoma because of vascular malformations involving the skin, brain, and eyes. Several patients also have cognitive issues as well as difficulties with attention and behavior. Attention deficit hyperactivity disorder (ADHD) was observed in 42% of SWS patients described in one 40-year chart review.¹ Chapieski et al. reported 22% of SWS patients with ADHD in a group of 79 patients.² Although Turin et al. reported a 90% rate of attention problems in 10 children with SWS, none of those subjects met Diagnostic and Statistical Manual of Mental Disorders- IV text revision criteria for ADHD after psychiatric evaluation.³ A recent survey from our center noted 10% of patients with self-reported ADHD.⁴ Clinicians' reluctance to use stimulant medications in SWS patients likely involves concerns for worsening seizure activity and possible neurovascular interactions. Current research is focused on the use of low dose aspirin as a potential neurological disease treatment option as well.⁵

Individuals with SWS commonly develop epilepsy and stroke-like episodes. Interestingly, ADHD is quite common in epilepsy; one population based study reported a 27.2% prevalence in pediatric epilepsy patients.⁶ Dunn et al. also found a gender ratio of 1:1 in comparison to a 2:1 male/female ratio in the general ADHD population as well as a higher overall prevalence of inattentive type ADHD.⁷ In addition, one study showed higher rates of ADHD in children with a history of stroke than in the general population.⁸ SWS patients are typically treated with a combination of medication titration and behavioral therapy to safely optimize seizure control, behavioral issues, and school performance. In addition, multiple studies have shown that stimulants do not increase seizures in epilepsy.9, 10

Although stimulant use is common in the typical child with a diagnosis of ADHD, medication safety and efficacy in children with neurovascular disease has not been well established. This study is the first to review stimulant use in a cohort of patients with SWS. The objective of the study is to describe efficacy and side effects of stimulant medications in a retrospective cohort of SWS patients.