How are strokelike events in Sturge-Weber syndrome (SWS) treated?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

Aspirin has been used for headaches and to prevent vascular disease, although it typically is used in patients who have had neurologic progression or recurrent vascular events. [99] Aspirin needs to be used with extreme caution in children because of the risk that Reye syndrome could develop, and the risks and benefits need to be carefully weighed.

Thomas-Sohl, Vaslow, and Maria have recommended 3-5 mg/kg/day of aspirin for stroke-like events. They also recommended varicella and yearly influenza immunizations because of the association of varicella and influenza infections with Reye syndrome. [1]

Maria et al reported a decreased incidence of strokelike events in 20 patients with SWS who received aspirin. Of 119 strokelike events, 31 occurred in patients treated with aspirin, whereas 88 of these events occurred in those not treated with aspirin. The authors suggested further investigation of aspirin treatment in SWS. [22]

Using data from a survey on aspirin use in 34 SWS patients, Bay et al reported a reduction of strokelike episodes from 1.1 to 0.3 per month and a reduction of seizures from 3 to 1 per month. The investigators found that 39% of patients had complications of predominantly bruising or gum/nose bleeding, although none were reported to have discontinued aspirin due to adverse effects. [100]

Lance et al, in a review of 58 patients with SWS on aspirin, found that 49 had no significant adverse effects, while the remaining 9 had allergic reactions or minimal to significant bleeding while on aspirin. Reasonable seizure control was seen in 91% of the patients, no or mild hemiparesis occurred in 57% of them, no vision impairment was found in 71% of patients, and no or mild cognitive impairment was seen in 80% of them. The authors suggested that these data support the use of low-dose aspirin for optimizing neurodevelopmental outcome in SWS. [101]


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