{"id":785151,"date":"2017-03-07T16:32:32","date_gmt":"2017-03-07T21:32:32","guid":{"rendered":"https:\/\/neurovirtual.com\/?p=785151"},"modified":"2017-03-07T16:32:32","modified_gmt":"2017-03-07T21:32:32","slug":"when-mesial-focal-dysplasia-mimics-lennox-gastaut-syndrome","status":"publish","type":"post","link":"https:\/\/neurovirtual.com\/latam\/when-mesial-focal-dysplasia-mimics-lennox-gastaut-syndrome\/","title":{"rendered":"Lennox-Gastaut syndrome al synchrony as an EEG pitfall"},"content":{"rendered":"<p style=\"text-align: center;\"><b>SECONDARY <\/b><b>BILATERWHEN MESIAL FOCAL DYSPLASIA\u00a0MIMICS\u00a0<\/b><b>LENNOX-GASTAUT SYNDROME\u00a0<\/b><b>AL <\/b><b>SYNCHRONY AS <\/b><b>AN EEG PITFALL<\/b><\/p>\n<p style=\"text-align: center;\">\u00a0Figueiredo NSV (1); De Marchi LR (1); Zetehaku AC (1); Gomes ACD (1); Girotto PN (1);\u00a0\u00a0Guilhoto LMFF (1); Guaranha MSB (1); Carrete Jr. H (1); Centeno RS (1); Yacubian EMT (1).<\/p>\n<p><strong>Introduction<\/strong><\/p>\n<ul>\n<li>Lennox-Gastaut syndrome (LGS) is a challenging diagnosis in epileptology as could be misunderstood with different etiologies as focal cortical dysplasia (FCD) (Bourgeois et al., 2014).<\/li>\n<li>If patient has many seizure types is more probably to be wrong diagnosed (Camfield &amp; Camfield, 2007).<\/li>\n<li>Moreover, mesial surface lesions (MSL) could result in false localizations leading to EEG misinterpretation.<\/li>\n<li>In turn, patients with mesial lesions could have secondary bilateral synchrony (SBS) in EEG as showed by T\u00fckel and Jasper since the fifties (T\u00fckel &amp; Jasper, 1952). It is described as 2-4Hz high-amplitude, synchronous, almost symmetric <i>diffuse slow sharp waves<\/i> (DSSW).<\/li>\n<\/ul>\n<p><strong>Objectives<\/strong><\/p>\n<p>To highlight SBS as an EEG\u2019s pitfall describing a case of FCD mimicking LGS.<\/p>\n<p><strong>Case Report<\/strong><\/p>\n<ul>\n<li>A 9yo boy from Acre with previous LGS diagnosis performed pre-surgical evaluation for callosotomy in our epilepsy center.<\/li>\n<li>His mother had malaria during pregnancy needing medications nonetheless without complications (term delivery).<\/li>\n<li>His neurodevelopment was normal until 3yo when left focal motor seizures started and quickly evolved to tonic-clonic, tonic and drop seizures plus atypical absences.<\/li>\n<li>Consequently, moderate neuro-psychomotor impairment occurred as mild left hemiparesis.<\/li>\n<li>Serial EEGs showed LGS\u2019s hallmarks: 2.5Hz DSSW and generalized fast rhythm.<\/li>\n<li>However, awake background activity was normal, an <i>unexpected finding<\/i> in LGS due to be slow and poorly organized since its onset.<\/li>\n<\/ul>\n<p><span style=\"font-size: 10pt;\"><b>Figure 1: <\/b>EEG records findings \u2013 pro and against LGS diagnosis.\u00a0<\/span><\/p>\n<p><span style=\"font-size: 10pt;\"><b><a href=\"https:\/\/elastic-goodall.132-148-76-14.plesk.page\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng.png\"><img fetchpriority=\"high\" decoding=\"async\" class=\"wp-image-785153 size-medium aligncenter\" src=\"https:\/\/elastic-goodall.132-148-76-14.plesk.page\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng-300x197.png\" alt=\"\" width=\"300\" height=\"197\" srcset=\"https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng-300x197.png 300w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng-1024x673.png 1024w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng-768x504.png 768w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng-1536x1009.png 1536w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem1_eng.png 1920w\" sizes=\"(max-width: 300px) 100vw, 300px\" \/><\/a>legend: (A, B) <\/b>In LGS\u2019s EEG pattern is expected a triad with 2.5Hz DSSW, generalized fast rhythm and poorly organized background. <b>(C) <\/b>However, in this reported case a curious finding was his normal background when awake not fitting with LGS diagnosis.<\/span><\/p>\n<ul>\n<li>Video-EEG revealed stereotyped and predominantly <i>focal seizures<\/i> beginning at <i>right <\/i><i>fronto<\/i><i>-central <\/i>(RFC) regions that quickly <i>diffuse bilaterally<\/i> as DSSW. In addition, all mentioned seizures were clinically recorded.<\/li>\n<\/ul>\n<p><span style=\"font-size: 10pt;\"><b>Figure 2: <\/b>One electrographic seizure recorded during video-EEG with a focal beginning.<\/span><\/p>\n<p><a href=\"https:\/\/elastic-goodall.132-148-76-14.plesk.page\/latam\/wp-content\/uploads\/2017\/03\/Imagem2.png\"><img decoding=\"async\" class=\"size-medium wp-image-785154 aligncenter\" src=\"https:\/\/elastic-goodall.132-148-76-14.plesk.page\/latam\/wp-content\/uploads\/2017\/03\/Imagem2-300x171.png\" alt=\"Imagem2\" width=\"300\" height=\"171\" srcset=\"https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem2-300x171.png 300w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem2-1024x583.png 1024w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem2-768x437.png 768w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem2-1536x874.png 1536w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem2.png 1920w\" sizes=\"(max-width: 300px) 100vw, 300px\" \/><\/a><\/p>\n<p><span style=\"font-size: 10pt;\"><b>Legend: (A, B) <\/b>Although DSSW morphology (asterisks), the recorded electrographic seizure begun at RFC region (arrow and circle), putting lights on a probably focal lesion.<\/span><\/p>\n<p>EEG-guided curvilinear 3T MRI showed FCD at <i>right superior mesial frontal gyrus<\/i> extending to <i>anterior cingulate <\/i><i>gyrus<\/i> (ACG).<\/p>\n<p><span style=\"font-size: 10pt;\"><b>Figure 3: <\/b>The role of neuroimaging guided by EEG localizing the lesion.<\/span><\/p>\n<p><span style=\"font-size: 10pt;\"><b><a href=\"https:\/\/elastic-goodall.132-148-76-14.plesk.page\/latam\/wp-content\/uploads\/2017\/03\/Imagem3.png\"><img decoding=\"async\" class=\"size-medium wp-image-785155 aligncenter\" src=\"https:\/\/elastic-goodall.132-148-76-14.plesk.page\/latam\/wp-content\/uploads\/2017\/03\/Imagem3-300x300.png\" alt=\"Imagem3\" width=\"300\" height=\"300\" srcset=\"https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem3-300x300.png 300w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem3-1024x1021.png 1024w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem3-150x150.png 150w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem3-768x766.png 768w, https:\/\/neurovirtual.com\/latam\/wp-content\/uploads\/2017\/03\/Imagem3.png 1386w\" sizes=\"(max-width: 300px) 100vw, 300px\" \/><\/a>Legend: <\/b>Once EEG recordings showed a focal beginning at RFC region, a 3T MRI localized the lesion (arrows) in right frontal parasagittal region in sagittal T1 <b>(A)<\/b> and coronal T2 sequences <b>(B)<\/b>, such as axial DIR <b>(C)<\/b> and IR <b>(D)<\/b>. The transmantel sign is shown as a head arrow in <b>(B)<\/b>.<\/span><\/p>\n<p><span style=\"font-size: 10pt;\">[DIR: double inversion-recovery;\u00a0 IR: inversion-recovery].<\/span><\/p>\n<ul>\n<li>The patient underwent lesionectomy with electrocorticography; pathology confirmed FCD type I.<\/li>\n<\/ul>\n<p><strong>Discussion<\/strong><\/p>\n<ul>\n<li>This case displays a pseudo-LGS with MSL at frontal\/temporal lobes leading to SBS.<\/li>\n<li>In Gastaut\u2019s series (1987), misdiagnosis of LGS in symptomatic partial epilepsies ranged from 37-51.5%, mainly when drop attacks were present.<\/li>\n<li>Furthermore, fast rhythms and tonic seizures could also be find misleading differentiation.<\/li>\n<li>Cortical areas on mesial surface, around corpus callosum such as ACG, set abnormal electrical activity that rapidly involves both hemispheres generating SBS with similar morphology to generalized seizures \u2013 an EEG\u2019s pitfall (Lennox &amp; Robinson, 1951).<\/li>\n<\/ul>\n<p><strong>Conclusions<\/strong><\/p>\n<p>Better outcomes depend on accurate history associated with detailed EEG analysis avoiding misinterpretations.<\/p>\n<p><span style=\"font-size: 10pt;\"><b>References<\/b><\/span><\/p>\n<p><span style=\"font-size: 10pt;\">1- BOURGEOIS BFD, DOUGLASS LM, SANKAR R. Lennox-Gastaut syndrome: A consensus approach to differential diagnosis. Epilepsia 2014;55(Suppl.4):S4-S9.<\/span><\/p>\n<p><span style=\"font-size: 10pt;\">2- CAMFIELD P, CAMFIELD C. Long-term prognosis for symptomatic (secondarily) generalized epilepsies: a population-based study. Epilepsia 2007;48:1128-1132.<\/span><\/p>\n<p><span style=\"font-size: 10pt;\">3- T\u00dcKEL K, JASPER H. The electroencephalogram in parasagittal lesions. Electroencephalogr Clin Neurophysiol 1952;4:481-494.<\/span><\/p>\n<p><span style=\"font-size: 10pt;\">4- GASTAUT H, ZIFKIN B, MAGGAUDA A, MARIANIL E. Symptomatic partial epilepsies with secondary bilateral synchrony: differentiation from symptomatic generalized epilepsies of the lennox-gastaut type. In: <i>Presurgical<\/i> <i>Evaluation of <\/i><i>Epileptics.<\/i> WIESER HG, ELGER CE, editors. Springer-Verlag, Berlin Heidelberg, 1987.<\/span><\/p>\n<p><span style=\"font-size: 10pt;\">5- LENNOX MA, ROBINSON F. Cingulate-cerebellar mechanisms in the physiological pathogenesis of epilepsy. EEG Clin Neurophysiol 1981;3:197-205.<\/span><\/p>\n","protected":false},"excerpt":{"rendered":"<p>SECONDARY BILATERWHEN MESIAL FOCAL DYSPLASIA\u00a0MIMICS\u00a0LENNOX-GASTAUT SYNDROME\u00a0AL SYNCHRONY AS AN EEG PITFALL \u00a0Figueiredo NSV (1); De Marchi LR (1); Zetehaku AC (1); Gomes ACD (1); Girotto PN (1);\u00a0\u00a0Guilhoto LMFF (1); Guaranha MSB (1); Carrete Jr. H (1); Centeno RS (1); Yacubian EMT (1). Introduction Lennox-Gastaut syndrome (LGS) is a challenging diagnosis in epileptology as could be [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":822936,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[14,15],"tags":[],"class_list":["post-785151","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-international","category-news"],"acf":[],"_links":{"self":[{"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/posts\/785151","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/comments?post=785151"}],"version-history":[{"count":0,"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/posts\/785151\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/media\/822936"}],"wp:attachment":[{"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/media?parent=785151"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/categories?post=785151"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/neurovirtual.com\/latam\/wp-json\/wp\/v2\/tags?post=785151"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}