flunarizine and Sturge-Weber-Syndrome

Children with Sturge-Weber syndrome can experience severe headache with or without transient hemiparesis. Flunarizine, a calcium antagonist, has been used for migraine. The experience with flunarizine for headache in a cohort of children at a national center for Sturge-Weber syndrome is reviewed, reporting its efficacy and adverse effect in this population.. We collected data from health care professionals' documentation on headache (severity, frequency, duration) before and on flunarizine in 20 children with Sturge-Weber syndrome. Adverse effects reported during flunarizine treatment were collated. The Wilcoxon signed rank test was used to determine the significance of pre- versus post-treatment effect.. Flunarizine was used for headache alone (13) or mixed migrainous episodes and vascular events (7). The median duration of treatment was 145 days (range 43 to 1864 days). Flunarizine reduced headache severity (z = -3.354, P = 0.001), monthly frequency (z = -2.585, P = 0.01), and duration (z = -2.549, P = 0.01). Flunarizine was discontinued owing to intolerable adverse effects in a minority (2). Sedation and weight gain were the most common side effects. There were no reports of behavior change or extrapyramidal features.. The most effective management for headaches in patients with Sturge-Weber syndrome has not been established. This retrospective observational study found benefit of flunarizine prophylaxis on headache severity, frequency, and duration in children with Sturge-Weber syndrome without severe side effects. Flunarizine is not licensed for use in the United Kingdom, but these data support its off-license specialist use for headache prophylaxis in Sturge-Weber syndrome.

Topics: Adolescent; Calcium Channel Blockers; Child; Child, Preschool; Female; Flunarizine; Headache; Humans; Male; Paresis; Retrospective Studies; Sensation Disorders; Sturge-Weber Syndrome; Treatment Outcome

Topics: Anti-Inflammatory Agents, Non-Steroidal; Aspirin; Child, Preschool; Electroencephalography; Female; Flunarizine; Humans; Migraine Disorders; Paresis; Sturge-Weber Syndrome; Vasodilator Agents

A 3-year-old boy was reported, who suffered from attacks of hemiconvulsion and hemiparesis. Carotid angiography showed anomalous cerebral venous drainage with a decrease in the number of superficial cortical veins, and dilatation of medullary veins and deep veins. Brain scanning demonstrated abnormality of regional cerebral blood flow and delay of both uptake and drainage of radionuclide. These are similar to the findings known in Sturge-Weber disease, so that a leptomeningeal angiomatosis was strongly suggested. Flunarizine which has a cerebral vasodilating effect improved his clinical symptoms significantly, and his attack disappeared completely.

Topics: Angiomatosis; Arachnoid; Child, Preschool; Flunarizine; Humans; Male; Meningeal Neoplasms; Pia Mater; Sturge-Weber Syndrome