Corpus callosum plays the important role in bilateral synchronous expression of focal discharges of ESES. Sparing dominant hemisphere form continuous spike and slow waves during sleep accounts for normal cognitive scores. Early detection and treatment of ESES have a great impact on cognitive and language scores and final prognosis

Asadi-Pooya, Ali Akbar

Badv, Reza Shervin

Kowkabi, Safoura

Malamiri, Reza Azizi

Mohammadi, Mahmoud

English

Jefferson Digital Commons

Thomas Jefferson UniversityJefferson Digital CommonsDepartment of Neurology Faculty Papers Department of Neurology2-10-2019Hemi-ESES associated with agenesis of the corpuscallosum and normal cognition.Mahmoud MohammadiTehran University of Medical SciencesSafoura KowkabiTehran University of Medical SciencesAli Akbar Asadi-PooyaThomas Jefferson University; Shiraz University of Medical Sciences, Aliakbar.Asadi-Pooya@jefferson.eduReza Azizi MalamiriAhvaz Jundishapur University of Medical SciencesReza Shervin BadvTehran University of Medical SciencesLet us know how access to this document benefits youFollow this and additional works at: https://jdc.jefferson.edu/neurologyfpPart of the Neurology CommonsThis Article is brought to you for free and open access by the Jefferson Digital Commons. The Jefferson Digital Commons is a service of ThomasJefferson University's Center for Teaching and Learning (CTL). The Commons is a showcase for Jefferson books and journals, peer-reviewed scholarlypublications, unique historical collections from the University archives, and teaching tools. The Jefferson Digital Commons allows researchers andinterested readers anywhere in the world to learn about and keep up to date with Jefferson scholarship. This article has been accepted for inclusion inDepartment of Neurology Faculty Papers by an authorized administrator of the Jefferson Digital Commons. For more information, please contact:JeffersonDigitalCommons@jefferson.edu.Recommended CitationMohammadi, Mahmoud; Kowkabi, Safoura; Asadi-Pooya, Ali Akbar; Malamiri, Reza Azizi; andBadv, Reza Shervin, "Hemi-ESES associated with agenesis of the corpus callosum and normalcognition." (2019). Department of Neurology Faculty Papers. Paper 180.https://jdc.jefferson.edu/neurologyfp/180Case ReportHemi-ESES associated with agenesis of the corpus callosum andnormal cognitionMahmoudMohammadi a, Safoura kowkabi a,⁎, Ali A. Asadi-Pooya b,c, Reza Azizi Malamiri d, Reza Shervin Badv aa Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iranb Jefferson Comprehensive Epilepsy Center, Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USAc Department of Neurology, Shiraz University of Medical Sciences, Shiraz, Irand Department of Pediatric Neurology, Golestan Medical, Educational and Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Irana r t i c l e i n f oArticle history:Received 29 October 2018Received in revised form 16 January 2019Accepted 17 January 2019Available online 10 February 20191. IntroductionContinuous spike wave during sleep (CSWS) is an epilepticencephalopathymainly deﬁned by cognitive decline, various seizuretypes, and a speciﬁc electroencephalographic pattern, called electri-cal status epilepticus during sleep (ESES) [1,2]. ESES is characterizedby abundant and diffuse spike-and-wave complexes during slow-wave sleep. The percentage of these spike-and-waves during sleepis called the spike–wave index (SWI) [3]. To deﬁne ESES, the SWIshould be present in at least 85% of NREM sleep; however, the cutoffpoint has been deﬁned in different studies to be between 25 to85% of NREM sleep duration [2,4]. CSWS is a rare and age-relatedepileptic encephalopathy with a prevalence of 0.5% in childrenwith epilepsy [2].The pathophysiology of this syndrome is unknown; however,recent investigations suggest that continuous spike and wave duringsleep may interfere with sleep-related physiologic functions and therestorative process of slow-wave sleep due to the lack of sleep down-scaling (overnight decrease in slow-wave slope), entailing cognitiveand memory dysfunction [5,6]. In almost all patients with CSWS,seizures stop and the ESES pattern disappears during or afterpuberty; in only 10–40% of these children, however, cognition andlanguage becomes normal after the ESES pattern has resolved.Therefore, early detection and treatment have a great impact on theﬁnal prognosis [1,6].ESES is often a generalized pattern, but some studies have reportedcases with hemi-ESES in children with polymicrogyria, porencephaliccysts, hydrocephalus, and thalamic lesions [7–9].The corpus callosum is the most prominent anatomic commissuralstructure connecting and integrating the activities of both cerebralhemispheres. Moreover, the disruption of callosal ﬁbers preventsseizure propagation and evolution to a generalized seizure [10].We herein describe a child with normal cognition and nocturnalseizures, after a thorough workup, we found hemi-ESES and absolutelyno corpus callous in the patient.2. Case reportOur patientwas a 6-year-old girl referred for nocturnal seizures. Herparents were ﬁrst cousins, and she was delivered by cesarean sectionfollowing an unremarkable pregnancy. Her development was normal,and she was right-handed. At age 3, she developed an event duringsleep, later deﬁned as seizure. The seizure began as a left perioral twitchwith subsequent left hand clonicmovements and left lateral gaze lastingfor about 2 min, without any signiﬁcant postictal confusion. A thoroughneurological examination, biochemistry workup, and a standard EEGwere performed, but no signs of abnormality were seen. A year follow-ing the ﬁrst seizure, she developed twomore seizures, whichwere sim-ilar to the ﬁrst one. After these seizures, sodium valproate was started,and a brain MRI was performed. Her MRI showed complete absence ofthe corpus callosum along with colpocephaly (Fig. 1). Her one-hourvideo EEG showed frequent right centrotemporal spike-and-slowwaves. She experienced no seizures over the next two years. Later on,however, she developed another semiology of nocturnal focal seizuresmanifest as left hand and face clonic jerks. After these seizuresreoccurred, she referred to our center for long-termvideo-EEGmonitor-ing (LTM).After admission at our center, we discontinued her medicationand waited for three days prior to conducting LTM. After four daysof monitoring, her EEG showed very few right centrotemporalspike-and-slow waves during the awake state. We observed a prom-inent accentuation of these spike-and-slow waves during slowsleep. Her SWI showed a hemi-ESES pattern over the right hemi-sphere with 50–85% spike-and-slow wave complexes during NREMsleep (Fig. 2). During NREM, the architecture of sleep was normalon the left side, yet severely disturbed on the right side. After ad-ministering high doses of benzodiazepine (diazepam orally1 mg/kg stat at night), the hemi-ESES pattern was completely re-solved. We conducted the Wechsler IQ test (WPPSI) just beforeEpilepsy & Behavior Case Reports 11 (2019) 96–98⁎ Corresponding author at: Shiraz University of Medical Sciences, Shiraz, Iran.E-mail address: kowkabis@gmail.com (S. kowkabi).https://doi.org/10.1016/j.ebcr.2019.01.0052213-3232/© 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).Contents lists available at ScienceDirectEpilepsy & Behavior Case Reportsj ourna l homepage: www.e lsev ie r .com/ locate /ebcrand one day following the administration of benzodiazepine. Her IQscore was surprisingly in a normal range, where the verbal IQ scorewas 92; average score for verbal tests was 44 and for each subtest:Information = 8, Vocabulary = 10, Similarities = 10, Arithmetic= 10, Comprehension = 6), performance IQ score was 101;averagescore for performance tests was 51 and for each subtest: Animalhouse = 10, Picture completion = 10, Mazes = 10, Geometricdesign = 12, Block design = 9) and total IQ score was 97 with anaverage total score of 95. As far as intelligence score is concerned,there was no signiﬁcant difference between her verbal andperformance score, nor was any difference observed before andafter taking diazepam.3. DiscussionThe ESES pattern has been reported in patients with focal cerebrallesions including unilateral thalamic lesions [8]. Kelman A. et al. sug-gested that this ﬁnding is elucidated by electrographic bilateral syn-chrony propagated via corpus callosum on EEG. Other studies haveshown the ESES pattern both in patients without a brain lesion, and inthose with epilepsy associated with different types of focal cortical le-sions. Diffuse cortical spike-and-slow waves could be explained byspreading from a focal onset to the hemispheres bilaterally via the cor-pus callosum [8].In our patient, the presence of hemi-ESES is explained by the con-genital absence of the corpus callosum inhibiting the propagation ofspike and slow waves during sleep from the right side of the brain tothe left, thus limiting bilateral synchronous expression of ESES. It is as-sumed that sparing the left dominant hemisphere from continuousspike-and-slow waves during sleep in our patient accounts for the nor-mal cognitive and language scores in a right-handed patient with agen-esis of the corpus callosum. Although sleep has a potent activation rolefor expresssing interictal epileptiform discharges, for example in pa-tients with frontal lobe epilepsy, it differs from ESES with a pattern ofcentrotemporal spikes with respect to: 1) dipole orientation that has atangential dipole with a negative pole in the central sulcus and a posi-tive pole on the anterior head region in centrotemporal spikes, and2) the few seizures involving the hand and facemotor area in the typicalESES pattern seen with centrotemporal spikes.4. ConclusionOur ﬁndings emphasize the focal nature of ESES and role of thecorpus callosum in bilateral synchronous expression of hemispheric ep-ileptiform discharges.Fig. 1. Sagittal brain MRI of index case. MRI depicts complete agenesis of corpuscallosum.Fig. 2. EEG features of index case. EEG shows a hemi-ESES pattern over the right hemisphere with 50–85% spike and slow wave complex during NREM sleep.97M. Mohammadi et al. / Epilepsy & Behavior Case Reports 11 (2019) 96–98AcknowledgmentsThe authors would like to thank Miss Bakhshi for her invaluablecooperation.Declarations of interestNone.This research did not receive any speciﬁc grant from fundingagencies in the public, commercial, or not-for-proﬁt sectors.References[1] Striano P, Capovilla G. Epileptic encephalopathy with continuous spikes and wavesduring sleep. Curr Neurol Neurosci Rep 2013;13(7):360.[2] Pavlidis E, Rubboli G, Nikanorova M, Kölmel MS, Gardella E. Encephalopathy withstatus epilepticus during sleep (ESES) induced by oxcarbazepine in idiopathic focalepilepsy in childhood. Funct Neurol 2015;30(2):139.[3] Tassinari CA, Rubboli G, Volpi L, Meletti S, d'Orsi G, Franca M, et al. Encephalopathywith electrical status epilepticus during slow sleep or ESES syndrome including theacquired aphasia. Clin Neurophysiol 2000;111:S94–S102.[4] Scheltens-de Boer M. Guidelines for EEG in encephalopathy related to ESES/CSWS inchildren. Epilepsia 2009;50:13–7.[5] Bölsterli BK, Schmitt B, Bast Th, Critelli H, Heinzle J, Jenni OG, et al. Impaired slowwave sleep downscaling in encephalopathy with status epilepticus during sleep(ESES). Clin Neurophysiol 2011;122:1779–87.[6] Maltoni L, PosarA, Parmeggiani A. Long-term follow-upof cognitive functions inpatientswith continuous spike–waves during sleep (CSWS). Epilepsy Behav 2016;60:211–7.[7] Caraballo RH, Fortini S, Flesler S, Pasteris MC, Caramuta L, Portuondo E.Encephalopathy with status epilepticus during sleep: unusual EEG patterns. Seizure2015;25:117–25.[8] Kelemen A, Barsi P, Gyorsok Z, Sarac J, Szűcs A, Halász P. Thalamic lesion andepilepsy with generalized seizures, ESES and spike-wave paroxysms—report ofthree cases. Seizure 2006;15(6):454–8.[9] Buzatu M, Bulteau C, Altuzarra C, Dulac O, Van Bogaert. Corticosteroids as treatmentof epileptic syndromes with continuous spike-waves during slow-wave sleep.Epilepsia 2009;50:68–72.[10] Asadi-Pooya AA, Sharan A, Nei M, Sperling MR. Corpus callosotomy. Epilepsy Behav2008;13(2):271–8.98 M. Mohammadi et al. / Epilepsy & Behavior Case Reports 11 (2019) 96–98

Hemi-ESES associated with agenesis of the corpus callosum and normal cognition.

https://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1186&amp;context=neurologyfp

Hemi-ESES associated with agenesis of the corpus callosum and normal cognition.

Abstract

Similar works

Full text

Available Versions

Jefferson Digital Commons