Abstract
Aims Children with Sturge-Weber syndrome (SWS), a neurocutaneous disorder affecting cerebral perfusion, can experience severe headache with or without transient hemiparesis. Flunarizine, a calcium antagonist, has been used in children for migraine prophylaxis. This retrospective observational review describes the use of flunarizine and its side effect profile in 20 children with Sturge-Weber syndrome. We investigated the efficacy of flunarizine in reducing headache severity, frequency and duration. Methods 20 children with SWS on flunarizine treatment were studied retrospectively using a combination of health care professionals’ case note documentation. Headache severity, frequency and duration were recorded before commencement of flunarizine and at the latest clinic follow up after commencement. The Wilcoxen Sign Test was used to determine significance of the effect. Symptoms reported during flunarizine treatment were also recorded and compared with its established side effect profile. Results Flunarizine was used in 13 children for headache alone, and 7 children for mixed migrainous episodes and vascular events. Flunarizine significantly reduced headache severity (z-value 3.354, p=0.001), the monthly frequency of headache (z-value 2.585, p=0.01), and headache duration (z-value 2.549, p=0.011). Sedation and weight gain were the most prevalent observed side effects. There were no reports of behaviour change or extra-pyramidal movement disorders. 2 children discontinued flunarizine due to intolerable side effect. Conclusions Flunarizine prophylaxis reduced the severity, frequency and duration of headache in children with SWS. Weight gain and sedation were observed in this cohort, but discontinuation of flunarizine was rare. Further studies are required to consolidate information on the safety of flunarizine, in order to move towards licensing its use in the UK. However, this review supports the off license use of flunarizine prophylaxis for headache in children with SWS.
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