{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T04:24:26Z","timestamp":1774326266904,"version":"3.50.1"},"reference-count":37,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2013,4,15]],"date-time":"2013-04-15T00:00:00Z","timestamp":1365984000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>Interest in the association of epilepsy and pseudobulbar palsy was rekindled since the identification through magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>) of bilateral perisylvian polymicrogyria (<jats:styled-content style=\"fixed-case\">PMG<\/jats:styled-content>). Seizures are often intractable, but resective epilepsy surgery has not been recommended. However, a similar clinical picture can be encountered in patients with bilateral perisylvian destructive lesions, which fit the description of ulegyria (<jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content>). We report a series of patients with epilepsy and pseudobulbar palsy due to bilateral perisylvian <jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content>), show that hippocampal sclerosis (<jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>) is often associated and highlight the fact that in this entity, unlike in malformative bilateral perisylvian <jats:styled-content style=\"fixed-case\">PMG<\/jats:styled-content>, seizures may be surgically treated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The motor, cognitive, epileptologic, and imaging features of 12 patients with perisylvian <jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content> followed at three institutions are described. For patients with refractory seizures, we detail extracranial and intracranial electrographic recordings, surgical strategies, histopathologic analyses of the resected tissue, and outcome of surgical treatment. Descriptive statistics were used for quantitative and categorical variables. Student's <jats:italic>t<\/jats:italic>\u2010test was used to compare means, and a p\u00a0&lt;\u00a00.05 was considered significant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Key Findings<\/jats:title><jats:p>Pseudobulbar palsy and mental retardation were present in all patients with symmetrical <jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content>. Five had refractory seizures. There was no relationship between the severity of the pseudobulbar palsy or of the mental retardation and the degree of seizure control with medication. The five patients in whom seizures were refractory to medication had significantly earlier age of onset and longer duration of epilepsy (p\u00a0&lt;\u00a00.05). Dual pathology with associated unilateral <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content> was present in four. One patient with dual pathology had a temporolimbic electroclinical picture and had an anterior temporal lobectomy (<jats:styled-content style=\"fixed-case\">ATL<\/jats:styled-content>) based upon noninvasive evaluation. The other four had ictal semiology suggesting involvement of both temporolimbic and perisylvian cortex. Intracranial electroencephalography (<jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content>) showed concomitant seizure onset in the anterior temporal region and in the ipsilateral <jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content> in three of the four with dual pathology and in the ulegyric cortex in the one without <jats:styled-content style=\"fixed-case\">HS<\/jats:styled-content>. Resection guided by a combination of semiology, <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>, and extra and intracranial <jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content> led to complete seizure control in two and almost complete seizure control (Engel class <jats:styled-content style=\"fixed-case\">II<\/jats:styled-content>) in two other patients. The only surgical failure was an isolated <jats:styled-content style=\"fixed-case\">ATL<\/jats:styled-content> in a patient with dual pathology, and concomitant seizure onset in both lesions according to semiology and intracranial <jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Our findings suggest that <jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content> mimics the clinical features of bilateral perisylvian <jats:styled-content style=\"fixed-case\">PMG<\/jats:styled-content>. In patients with refractory seizures, recognition of this entity should lead to consideration of resective surgery despite the bilateral <jats:styled-content style=\"fixed-case\">ULG<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.12160","type":"journal-article","created":{"date-parts":[[2013,4,15]],"date-time":"2013-04-15T18:21:55Z","timestamp":1366050115000},"page":"1360-1367","source":"Crossref","is-referenced-by-count":11,"title":["Bilateral perisylvian ulegyria: An under\u2010recognized, surgically remediable epileptic syndrome"],"prefix":"10.1111","volume":"54","author":[{"given":"Lucas P.","family":"Schilling","sequence":"first","affiliation":[{"name":"Porto Alegre Epilepsy Surgery Program Hospital S\u00e3o Lucas PUCRS Porto Alegre Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Renata R.","family":"Kieling","sequence":"additional","affiliation":[{"name":"Porto Alegre Epilepsy Surgery Program Hospital S\u00e3o Lucas PUCRS Porto Alegre 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