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Posterior quadrant disconnection surgery for S turge‐ W eber syndrome
Author(s) -
Sugano Hidenori,
Nakanishi Hajime,
Nakajima Madoka,
Higo Takuma,
Iimura Yasushi,
Tanaka Kyoko,
Hosozawa Mariko,
Niijima Shinichi,
Arai Hajime
Publication year - 2014
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/epi.12547
Subject(s) - psychomotor learning , bayley scales of infant development , epilepsy , epilepsy surgery , medicine , quadrant (abdomen) , psychomotor disorder , anesthesia , surgery , psychiatry , cognition
Summary Objective Some patients with S turge‐ W eber syndrome ( SWS ) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. The majority of patients with SWS have leptomeningeal angioma located over the temporal, parietal, and occipital lobes. We applied posterior quadrant disconnection surgery for this type of SWS with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development. Methods Ten patients who were surgically treated using the posterior quadrantectomy ( PQT ) were enrolled in this study. Surgical outcome was analyzed as seizure‐free or not at 2 years after surgery. Psychomotor development was evaluated by the scores of mental developmental index ( MDI ) and psychomotor developmental index ( PDI ) in the Bayley Scales of Infant Development II preoperatively, and at 6 and 12 months after the PQT . Results Eight of 10 patients were seizure‐free. Patients without complete elimination of the angiomatous areas had residual seizures. Average MDI and PDI scores before the surgery were 64.8 and 71.6, respectively. Scores of MDI at 6 and 12 months after the PQT in seizure‐free patients were 80.5 and 84.5, respectively (p < 0.01). PDI scores at these postoperative intervals were 87.3 and 86.4, respectively (p < 0.05). Patients with residual seizures did not improve in either MDI or PDI . Significance The PQT achieved good seizure control and improved psychomotor development in patients with SWS . The complete deafferentation of angiomatous areas is required for seizure‐free results and psychomotor developmental improvement. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here .