JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES) ›› 2017, Vol. 55 ›› Issue (4): 91-95.doi: 10.6040/j.issn.1671-7554.0.2016.666

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Sturge-Weber syndrome in patients with intractable epilepsy: a clinicopathologic study of 7 cases

LI Yan, GUO Jun, WANG Rongqi, LIU Yongling   

  1. Department of Pathology, Haidian Hospital;
    Haidian District of No.3 Hospital of Peking University, Beijing 100080, China
  • Received:2016-06-05 Online:2017-04-10 Published:2017-04-10

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Abstract: Objective To investigate the clinicopathologic features of the brain tissues from the patients with refractory epilepsy related Sturge-Weber syndrome(SWS). Methods The clinical and pathologic data were reviewed in 7 SWS patients who underwent epilepsy operation. Seven patients including 4 males and 3 females had been diagnosed SWS. The age of patients for surgery was 2-20 years old. The age of patients when seizure onset was 1-12 months. Six cases accepted multilobar resettion, and 1 case accepted functional hemispherectomy. Results The pathological diagnoses were meningioangiomatosis, remarkable calcification in the brains gray matter and small blood vessels, focal cortical dysplasia(FCD)Ⅲc. Seizure outcome after the epilepsy operation revealed 3 patients had an Engel grade I, 2 patients had an Engel grade Ⅱ, 1 patient had an Engel grade Ⅲ, and 1 patient had an Engel grade Ⅳ. Conclusion SWS patients had earlier onset, longer course and characteristic pathological change. Children with typical symptom should accept operation as soon as possible. Atypical patients also had good prognosis after operation.

Key words: Sturge-Weber syndrome, Neurosurgery, Malformations of cortical development, Leptomeningeal angiomatosis, Epilepsy

CLC Number: 

  • R741.02
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