Flunarizine and Aspirin for Transient Hemiparesis in Sturge–Weber Syndrome

Children with Sturge–Weber syndrome (SWS) frequently have stroke-like episodes characterized by transient neurological defects.[1] [2] We describe a 5-year-old girl with SWS with right facial and cerebral angiomatosis. At 17 months of age, she started having episodes of fluctuating left hemiparesis on waking, lasting 20 minutes, not preceded by any symptoms or seizure activity ([Video Part A]). During these episodes, she remained conscious but very irritable. Electroencephalography (EEG) recordings only showed diffuse rhythmic delta slow waves, suggesting ischemia resulting from hemodynamic changes ([Video Part B]). Low-dose aspirin prophylaxis[3] was started with initial benefit, but the paroxysmal episodes recurred 9 months later, quickly increasing in frequency and duration, even lasting up to 1 hour. Aspirin was therefore replaced with flunarizine because the paroxysmal episodes were reminiscent of hemiplegic migraine, which responds to flunarizine,[4] and a case of SWS benefiting from flunarizine has been reported.[5] The episodes, however, recurred after 2 weeks with the same features. Then a combination of two drugs with an anti-ischemic effect resulting from antiaggregation and vasodilation (aspirin and flunarizine) achieved a remission persisting for more than 2 years. This is in accordance with the current hypothesis of recurrent microthrombosis and microvascular stasis caused by angiomatosis in SWS.

Video 1

Hypotonia and weakness of left arm and leg with difficulty standing unassisted. There is also loss of sensitivity and hemineglect on the same side. The EEG shows diffuse delta-wave activity prominent on right frontocentral areas (Part A). Evidence of hypotonia and paresis of left arm concomitant with rhythmic EEG delta waves more diffuse and with higher amplitude on right side where they are associated with background disorganization. The normalization of the EEG activity precedes the recovery of the paresis. Temporal delay between disappearance of slow waves and clinical recovery may be due to a brief residual metabolic disorder following the hemodynamic defect (Part B). EEG, electroencephalography.

• References

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