Brain & Development xxx (2014) xxx–xxx www.elsevier.com/locate/braindev
Original article
Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy Harumi Yoshinaga ⇑, Katsuhiro Kobayashi, Takashi Shibata, Takushi Inoue, Makio Oka, Tomoyuki Akiyama Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan Received 21 August 2013; received in revised form 26 January 2014; accepted 26 January 2014
Abstract Purpose: Benign childhood epilepsy with centro-temporal spikes (BECTS) and Panayiotopoulos syndrome (PS) have different pathophysiologies and show different types of seizures, yet they overlap in some important respects. In an attempt to understand the ways in which they differ from each other and overlap each other, we performed a detailed investigation on patients who had both characteristic types of seizure manifestations, namely, sylvian seizures and emetic seizures. Subjects and methods: We recruited consecutive subjects from the EEG database of outpatients who had visited our hospital between 2008 and 2010 and who had been diagnosed with BECTS or PS. As a result, 45 patients with BECTS and 50 patients with PS were selected from the database. Viewing the clinical records of these 95 patients, five patients were selected who had experienced both sylvian seizures and emetic seizures. Next, the clinical features and EEG findings of these five patients were retrospectively observed at the date of investigation: October 1, 2011. Results: We found that all the patients showed rolandic spikes when they had sylvian seizures, and occipital spikes or multifocal spikes when they had emetic seizures. We also report in detail on one patient who showed two different types of ictal EEG patterns: one of which started in the occipital area and the other of which was located in the rolandic area. Conclusion: Based on these findings, we conclude that widespread cortical hyperexcitability that includes the occipital area is necessary to produce the autonomic seizure manifestations seen in PS. Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Keywords: Panayiotopoulos syndrome; Benign childhood epilepsy with centro-temporal spikes; Rolandic spikes; Occipital spikes; Emetic seizures; Sylvian seizures
1. Introduction Benign childhood epilepsy with centro-temporal spikes (BECTS) [1] and Panayiotopoulos syndrome (PS) [2] are both types of benign childhood partial epilepsy. Both are observed in normal children without any abnormal brain structures and both have a good prognosis. The differences between two syndromes are ⇑ Corresponding author. Address: Department of Child Neurology, Okayama University Medical School, 2-5-1 Shikatacho, Okayama 700-8558, Japan. Tel.: +81 86 235 7372; fax: +81 86 235 7377. E-mail address: [email protected] (H. Yoshinaga).
onset age, seizure manifestations and typical electroencephalogram (EEG) findings. The characteristic seizure manifestation of BECTS is the so-called sylvian seizure [3], while the typical seizure manifestation of PS is characterized mainly by autonomic symptoms, particularly vomiting [2,4–6]. Centro-temporal spikes, namely rolandic spikes, are the hallmark of BECTS, whereas interictal EEGs of PS show greater variability, with a predominance of occipital spikes and occasional rolandic spikes [4]. The overlap of these two syndromes has been widely investigated by several authors including groups of
http://dx.doi.org/10.1016/j.braindev.2014.01.013 0387-7604/Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
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Panayiotopoulos as a main stream. Sibling cases of PS and BECTS have been reported [4] and it has also been reported that 3.5–12% of patients with PS developed sylvian seizures later in their course [2,4,5]. Therefore, in this study, we performed an investigation on patients who manifested both sylvian seizures and emetic seizures. 2. Subjects and methods For this study, we recruited consecutive patients from the 9840-EEG database of patients who visited our hospital between 2008 and 2010 and who had been diagnosed with BECTS or PS. The diagnostic criteria for BECTS were unilateral sensory and/or motor seizures involving the face and rolandic spikes, and those for PS were autonomic seizures, especially emetic ones. As a result, 45 patients with BECTS and 50 patients with PS were selected from the original database of 1386 epileptic patients under 16 years of age. Viewing the clinical records of these 95 patients, five patients were found who had exhibited both clinical seizure types, namely, sylvian seizures and emetic seizures. Next, the clinical features and EEG findings of these five patients were retrospectively observed at the date of investigation: October 1, 2011. The epileptic focus of each EEG was determined using referential and bipolar recordings to find the maximum voltage or the phase reversal of each spike. Rolandic (R) spikes were defined as spikes discretely localized on electrodes C3, C4, T3, T4 or spikes with phase reversal on these electrodes. Occipital (O) spikes were defined as spikes discretely localized on electrodes P3, P4, O1, O2, Pz or spikes with phase reversal on electrodes P3, P4, Pz. Multi-focal spikes were defined as rolandic spikes and occipital spikes concurrently observed with or without frontal spikes. This study was approved by the Okayama University Ethics Committee. 3. Results We found one male and four females who had both types of seizures. The clinical courses with the features of seizures and spike foci are shown in Fig. 1. Age at onset of afebrile seizures ranged from 3 years and 1 month to 6 years and 1 month. The timing of occurrence of the two types of seizures was as follows: All the patients experienced one or several seizures in which both types of seizure semiology were mixed and observed concurrently. Vomiting seizures appeared before sylvian seizures in one patient. In two patients, sylvian seizures occurred before the mixed type of seizures. As shown in Fig. 1, sylvian seizures did not necessarily occur after emetic seizures or the mixed type of seizures. All of the patients studied had prolonged
seizures, and autonomic epileptic status was observed in four patients. In patient 5, the onset seizure type was status epilepticus with the mixed semiology, and no other seizures occurred after this first single seizure in spite of the fact that this patient received no anti-epileptic treatment. The foci of the interictal spikes varied by case. The most severe case, patient 3, once showed continuous spike-waves during slow wave sleep (CSWS). In contrast, patient 5 showed EEG improvement without treatment within just 1 year of the first seizure manifestation. Occipital spikes or multifocal spikes including the occipital area were commonly observed when the patients experienced the mixed type of seizures. In what follows, we will discuss patient 2 in detail because this case is of particular relevance to the present study. This patient was a girl in whom two patterns of ictal EEGs were recorded. She was referred to us because two febrile seizures with prolonged generalized atonia and facial pallor occurred at 2 years and 2 months of age. She had had a normal development after an uneventful gestation and delivery. Her EEG first showed occipital spikes at the first visit. At the age of 3 years and 9 months, she developed an afebrile seizure during sleep with hemifacial twitching. Since then her EEG began to show rolandic spikes interictally. At the age of 5 years and 6 months of age, she experienced a prolonged left hemiconvulsion with vomiting that lasted for 45 min. Just after this seizure, we performed an EEG and fortuitously caught an ictal EEG of a left hemiconvulsion with vomiting. Although her interictal EEG showed multifocal spikes including rolandic spikes, her ictal EEG showed predominance in the occipital area and occipital focal slow waves remained after seizure cessation (Fig. 2). After this seizure, she started to have intractable sylvian seizures with hemifacial twitching without any autonomic signs including vomiting. Her EEG began again to show rolandic spikes without occipital spikes. In this type of seizure, as shown in Fig. 3, her ictal EEG started with repetitive small spikes in the rolandic area followed by alpha activity in the same area, while the occipital area was never involved. This ictal pattern is different from that shown in Fig. 2. After being started on lamotrigine, her seizures ceased. Both her neurological and mental states were normal, and her MRI was also normal. 4. Discussion Caraballo et al. reported in their prospective study of 192 PS patients that 24 children (12.5%) also had seizures with speech arrest and hemifacial motor symptoms that are characteristic of BECTS, concomitantly with otherwise typical PS seizures [5]. Sylvian seizures in
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
H. Yoshinaga et al. / Brain & Development xxx (2014) xxx–xxx
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Fig. 1. Clinical course of each patient. Dotted bars show febrile seizures. Black bars show vomiting seizures. White bars show sylvian seizures with or without generalization. Shaded bars show the mixed type of seizures. Wide bars show prolonged seizures, namely, status epilepticus. The letters R, O and M indicate rolandic, occipital spikes and multifocal spikes, respectively, and EEGs were performed at these points. In patient 2, a star indicates the EEG shown in Fig. 2 and a double star indicates the EEG shown in Fig. 3.
Fig. 2. An ictal EEG of a left hemiconvulsion with vomiting in patient 2. The ictal pattern with spiky alpha activity started in the bilateral occipital area preceding the clinical manifestation (left). Then, a left hemiconvulsion started (middle). After seizure cessation, focal slow waves were observed in the right occipital area (right).
BECTS indicate its sensory cortex origin, while the mainly autonomic symptoms in PS indicate no specific cortical area as the site of seizure onset. It has been debated how these two syndromes, which have different pathophysiologies, overlap each other. Various methods have been applied to investigate the relation of these two syndromes [6–8].
In an attempt to resolve this issue, we performed a detailed investigation on patients with both types of seizures that correspond to PS and BECTS. Despite the variation of the clinical courses of the patients we studied, all the patients showed rolandic spikes when they had sylvian seizures, and occipital spikes or multifocal spikes when they had emetic seizures. Ictal EEGs in
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
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H. Yoshinaga et al. / Brain & Development xxx (2014) xxx–xxx
Fig. 3. An ictal EEG of sylvian seizure in patient 2. The ictal pattern with spiky theta activity started in the right central area preceding the clinical manifestation (left). Then, left oral twitching started and the spike evolved in the right hemisphere (middle). Note that the right occipital area was not involved, although the left frontal area was involved (right).
patient 2 revealed that the migration of the ictal onset from the occipital area to the rolandic area corresponded to the change of the ictal clinical features, namely, the change from emetic seizures to sylvian seizures. Covanis et al. thoroughly investigated 24 patients who had emetic manifestations in at least one seizure and rolandic spikes on at least one EEG [9]. They concluded that clinical manifestations do not depend on whether rolandic spikes occur alone or with occipital spikes. However, we found one interesting pure PS patient. He had only typical emetic seizures in spite of having interictal rolandic spikes alone, so he was not included in the present study. The day after a vomiting seizure, his EEG showed focal slowing in the occipital area and interictal spikes located somewhat posteriorly, namely, in the parietal electrode. Thus, the timing of the EEG recording and the emetic seizure may have affected the EEG findings. Unfortunately, ictal EEG findings of emetic seizure were not investigated and the timing of the EEG recording and the emetic seizure were not described in the Covanis report [9]. Although insufficient ictal EEG data are available, occipital onset EEGs are predominantly reported in PS. Based on these findings, we still believe that the occipital area plays an important role in producing the characteristic vomiting seizures in PS, even when the patients show rolandic spikes on their EEGs. On the other hand, ictal EEGs in PS with onset in areas other than the occipital area, such as the frontal area, have also been reported [5,10].
Panayiotopoulos referred that diffuse cortical hyperexcitability limited not only to the occipital area but also involving emetic centers such as the hypothalamus may cause emetic seizures, while concentrated epileptogenesis in the rolandic area produces sylvian seizures [2]. Kanazawa supported and proved this hypothesis using MEG dipole analysis [8], and we also supported this hypothesis by investigation with the EEG dipole method [7]. Our present study indicates that sylvian seizures develop before emetic seizures in some cases. Therefore, it appears that the diffusion and concentration of epileptogenic foci depends not only on the age, but also on other individual factors. And during the period of cortical hyperexcitability, each patient experiences emetic seizures with occipital involvement, and during the period of concentration of this excitability, the patient experiences the sylvian seizures with rolandic spikes. This notion is supported by the fact that all the patients in this series showed status epilepticus during the period with emetic seizures. In fact, PS is the most common specific cause of afebrile nonconvulsive status epilepticus in childhood. However, prolonged sylvian seizures are rarely observed in BECTS. Parameggiani and Guerrini reported an interesting case of BECTS that showed two types of ictal EEG recordings. One short classic rolandic seizure involved only the rolandic area and the other longer one started from the rolandic area and showed late occipital involvement [11]. They suggested the cortical neurons of both areas are prone to develop prolonged seizures and seizure spread.
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
H. Yoshinaga et al. / Brain & Development xxx (2014) xxx–xxx
In conclusion, based on the clinical course and the associated EEG findings of the patients who have both emetic seizures and rolandic seizures, it appears that the distribution of the hyperexcitability of the cortex affects the clinical features of the benign partial epilepsy syndromes. However, our explanation of the manifestation of the two syndromes is still hypothetical. Further research on the genetic etiology of these benign agedependent epileptic syndromes is necessary to draw firm conclusions. Acknowledgement This study was supported in part by the Japan Epilepsy Research Foundation. References [1] Beausarrt M. Benign epilepsy of children with Rolandic (centrotemporal) paroxysmal foci. A clinical study. Study of 221 cases. Epilepsia 1972;13:795–811. [2] Panayiotopoulos CP, Micahel M, Sanders S, Valeta T, Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008;131: 2264–86.
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[3] Loiseau P, Beaussart M. The seizures of benign childhood epilepsy with Rolandic paroxysmal discharges. Epilepsia 1973; 14:381–9. [4] Oguni H, Hayashi K, Imai K, Hirano Y, Mutoh A, Osawa M. Study on the early-onset variant of benign childhood epilepsy with occipital paroxysms otherwise described as early-onset benign occipital seizure susceptibility syndrome. Epilepsia 1999;40:1020–30. [5] Caraballo R, Cersosimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007;48:1054–61. [6] Koutroumanidis M. Panayiotopoulos syndrome: an important electroclinical example of benign childhood system epilepsy. Epilepsia 2007;48:1044–53. [7] Yoshinaga H, Koutroumanidis M, Kobayashi K, Shirasawa A, Kikumoto K, Inoue T, et al. EEG dipole characteristics in Panayiotopoulos syndrome. Epilepsia 2006;47:781–7. [8] Kanazawa O, Tohyama J, Akasaka N, Kamimura T. A magnetoencephalographic study of patients with Panayiotopoulos syndrome. Epilepsia 2005;46:1106–13. [9] Covanis A, Lada C, Skiadas K. Children with spikes and ictal vomiting: Rolandic epilepsy or Panayiotopoulos syndrome? Rolandic Epileptic Disord 2003;5:139–43. [10] Specchio N, Trivisano M, Ciommo VD, Cappelletti S, Masciarelli G, Volkov J, et al. Panayiotopoulos syndrome: a clinical, EEG, and neuropsychological study of 93 consecutive patients. Epilepsia 2010;51:2098–107. [11] Parmeggiani L, Guerrini R. Idiopathic partial epilepsy: electroclinical demonstration of a prolonged seizure with sequential rolandic and occipital involvement. Seizure spread due to regional susceptibility? Epileptic Disord 1999;1:35–40.
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
Original article
Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy Harumi Yoshinaga ⇑, Katsuhiro Kobayashi, Takashi Shibata, Takushi Inoue, Makio Oka, Tomoyuki Akiyama Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan Received 21 August 2013; received in revised form 26 January 2014; accepted 26 January 2014
Abstract Purpose: Benign childhood epilepsy with centro-temporal spikes (BECTS) and Panayiotopoulos syndrome (PS) have different pathophysiologies and show different types of seizures, yet they overlap in some important respects. In an attempt to understand the ways in which they differ from each other and overlap each other, we performed a detailed investigation on patients who had both characteristic types of seizure manifestations, namely, sylvian seizures and emetic seizures. Subjects and methods: We recruited consecutive subjects from the EEG database of outpatients who had visited our hospital between 2008 and 2010 and who had been diagnosed with BECTS or PS. As a result, 45 patients with BECTS and 50 patients with PS were selected from the database. Viewing the clinical records of these 95 patients, five patients were selected who had experienced both sylvian seizures and emetic seizures. Next, the clinical features and EEG findings of these five patients were retrospectively observed at the date of investigation: October 1, 2011. Results: We found that all the patients showed rolandic spikes when they had sylvian seizures, and occipital spikes or multifocal spikes when they had emetic seizures. We also report in detail on one patient who showed two different types of ictal EEG patterns: one of which started in the occipital area and the other of which was located in the rolandic area. Conclusion: Based on these findings, we conclude that widespread cortical hyperexcitability that includes the occipital area is necessary to produce the autonomic seizure manifestations seen in PS. Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Keywords: Panayiotopoulos syndrome; Benign childhood epilepsy with centro-temporal spikes; Rolandic spikes; Occipital spikes; Emetic seizures; Sylvian seizures
1. Introduction Benign childhood epilepsy with centro-temporal spikes (BECTS) [1] and Panayiotopoulos syndrome (PS) [2] are both types of benign childhood partial epilepsy. Both are observed in normal children without any abnormal brain structures and both have a good prognosis. The differences between two syndromes are ⇑ Corresponding author. Address: Department of Child Neurology, Okayama University Medical School, 2-5-1 Shikatacho, Okayama 700-8558, Japan. Tel.: +81 86 235 7372; fax: +81 86 235 7377. E-mail address: [email protected] (H. Yoshinaga).
onset age, seizure manifestations and typical electroencephalogram (EEG) findings. The characteristic seizure manifestation of BECTS is the so-called sylvian seizure [3], while the typical seizure manifestation of PS is characterized mainly by autonomic symptoms, particularly vomiting [2,4–6]. Centro-temporal spikes, namely rolandic spikes, are the hallmark of BECTS, whereas interictal EEGs of PS show greater variability, with a predominance of occipital spikes and occasional rolandic spikes [4]. The overlap of these two syndromes has been widely investigated by several authors including groups of
http://dx.doi.org/10.1016/j.braindev.2014.01.013 0387-7604/Ó 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
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Panayiotopoulos as a main stream. Sibling cases of PS and BECTS have been reported [4] and it has also been reported that 3.5–12% of patients with PS developed sylvian seizures later in their course [2,4,5]. Therefore, in this study, we performed an investigation on patients who manifested both sylvian seizures and emetic seizures. 2. Subjects and methods For this study, we recruited consecutive patients from the 9840-EEG database of patients who visited our hospital between 2008 and 2010 and who had been diagnosed with BECTS or PS. The diagnostic criteria for BECTS were unilateral sensory and/or motor seizures involving the face and rolandic spikes, and those for PS were autonomic seizures, especially emetic ones. As a result, 45 patients with BECTS and 50 patients with PS were selected from the original database of 1386 epileptic patients under 16 years of age. Viewing the clinical records of these 95 patients, five patients were found who had exhibited both clinical seizure types, namely, sylvian seizures and emetic seizures. Next, the clinical features and EEG findings of these five patients were retrospectively observed at the date of investigation: October 1, 2011. The epileptic focus of each EEG was determined using referential and bipolar recordings to find the maximum voltage or the phase reversal of each spike. Rolandic (R) spikes were defined as spikes discretely localized on electrodes C3, C4, T3, T4 or spikes with phase reversal on these electrodes. Occipital (O) spikes were defined as spikes discretely localized on electrodes P3, P4, O1, O2, Pz or spikes with phase reversal on electrodes P3, P4, Pz. Multi-focal spikes were defined as rolandic spikes and occipital spikes concurrently observed with or without frontal spikes. This study was approved by the Okayama University Ethics Committee. 3. Results We found one male and four females who had both types of seizures. The clinical courses with the features of seizures and spike foci are shown in Fig. 1. Age at onset of afebrile seizures ranged from 3 years and 1 month to 6 years and 1 month. The timing of occurrence of the two types of seizures was as follows: All the patients experienced one or several seizures in which both types of seizure semiology were mixed and observed concurrently. Vomiting seizures appeared before sylvian seizures in one patient. In two patients, sylvian seizures occurred before the mixed type of seizures. As shown in Fig. 1, sylvian seizures did not necessarily occur after emetic seizures or the mixed type of seizures. All of the patients studied had prolonged
seizures, and autonomic epileptic status was observed in four patients. In patient 5, the onset seizure type was status epilepticus with the mixed semiology, and no other seizures occurred after this first single seizure in spite of the fact that this patient received no anti-epileptic treatment. The foci of the interictal spikes varied by case. The most severe case, patient 3, once showed continuous spike-waves during slow wave sleep (CSWS). In contrast, patient 5 showed EEG improvement without treatment within just 1 year of the first seizure manifestation. Occipital spikes or multifocal spikes including the occipital area were commonly observed when the patients experienced the mixed type of seizures. In what follows, we will discuss patient 2 in detail because this case is of particular relevance to the present study. This patient was a girl in whom two patterns of ictal EEGs were recorded. She was referred to us because two febrile seizures with prolonged generalized atonia and facial pallor occurred at 2 years and 2 months of age. She had had a normal development after an uneventful gestation and delivery. Her EEG first showed occipital spikes at the first visit. At the age of 3 years and 9 months, she developed an afebrile seizure during sleep with hemifacial twitching. Since then her EEG began to show rolandic spikes interictally. At the age of 5 years and 6 months of age, she experienced a prolonged left hemiconvulsion with vomiting that lasted for 45 min. Just after this seizure, we performed an EEG and fortuitously caught an ictal EEG of a left hemiconvulsion with vomiting. Although her interictal EEG showed multifocal spikes including rolandic spikes, her ictal EEG showed predominance in the occipital area and occipital focal slow waves remained after seizure cessation (Fig. 2). After this seizure, she started to have intractable sylvian seizures with hemifacial twitching without any autonomic signs including vomiting. Her EEG began again to show rolandic spikes without occipital spikes. In this type of seizure, as shown in Fig. 3, her ictal EEG started with repetitive small spikes in the rolandic area followed by alpha activity in the same area, while the occipital area was never involved. This ictal pattern is different from that shown in Fig. 2. After being started on lamotrigine, her seizures ceased. Both her neurological and mental states were normal, and her MRI was also normal. 4. Discussion Caraballo et al. reported in their prospective study of 192 PS patients that 24 children (12.5%) also had seizures with speech arrest and hemifacial motor symptoms that are characteristic of BECTS, concomitantly with otherwise typical PS seizures [5]. Sylvian seizures in
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
H. Yoshinaga et al. / Brain & Development xxx (2014) xxx–xxx
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Fig. 1. Clinical course of each patient. Dotted bars show febrile seizures. Black bars show vomiting seizures. White bars show sylvian seizures with or without generalization. Shaded bars show the mixed type of seizures. Wide bars show prolonged seizures, namely, status epilepticus. The letters R, O and M indicate rolandic, occipital spikes and multifocal spikes, respectively, and EEGs were performed at these points. In patient 2, a star indicates the EEG shown in Fig. 2 and a double star indicates the EEG shown in Fig. 3.
Fig. 2. An ictal EEG of a left hemiconvulsion with vomiting in patient 2. The ictal pattern with spiky alpha activity started in the bilateral occipital area preceding the clinical manifestation (left). Then, a left hemiconvulsion started (middle). After seizure cessation, focal slow waves were observed in the right occipital area (right).
BECTS indicate its sensory cortex origin, while the mainly autonomic symptoms in PS indicate no specific cortical area as the site of seizure onset. It has been debated how these two syndromes, which have different pathophysiologies, overlap each other. Various methods have been applied to investigate the relation of these two syndromes [6–8].
In an attempt to resolve this issue, we performed a detailed investigation on patients with both types of seizures that correspond to PS and BECTS. Despite the variation of the clinical courses of the patients we studied, all the patients showed rolandic spikes when they had sylvian seizures, and occipital spikes or multifocal spikes when they had emetic seizures. Ictal EEGs in
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
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H. Yoshinaga et al. / Brain & Development xxx (2014) xxx–xxx
Fig. 3. An ictal EEG of sylvian seizure in patient 2. The ictal pattern with spiky theta activity started in the right central area preceding the clinical manifestation (left). Then, left oral twitching started and the spike evolved in the right hemisphere (middle). Note that the right occipital area was not involved, although the left frontal area was involved (right).
patient 2 revealed that the migration of the ictal onset from the occipital area to the rolandic area corresponded to the change of the ictal clinical features, namely, the change from emetic seizures to sylvian seizures. Covanis et al. thoroughly investigated 24 patients who had emetic manifestations in at least one seizure and rolandic spikes on at least one EEG [9]. They concluded that clinical manifestations do not depend on whether rolandic spikes occur alone or with occipital spikes. However, we found one interesting pure PS patient. He had only typical emetic seizures in spite of having interictal rolandic spikes alone, so he was not included in the present study. The day after a vomiting seizure, his EEG showed focal slowing in the occipital area and interictal spikes located somewhat posteriorly, namely, in the parietal electrode. Thus, the timing of the EEG recording and the emetic seizure may have affected the EEG findings. Unfortunately, ictal EEG findings of emetic seizure were not investigated and the timing of the EEG recording and the emetic seizure were not described in the Covanis report [9]. Although insufficient ictal EEG data are available, occipital onset EEGs are predominantly reported in PS. Based on these findings, we still believe that the occipital area plays an important role in producing the characteristic vomiting seizures in PS, even when the patients show rolandic spikes on their EEGs. On the other hand, ictal EEGs in PS with onset in areas other than the occipital area, such as the frontal area, have also been reported [5,10].
Panayiotopoulos referred that diffuse cortical hyperexcitability limited not only to the occipital area but also involving emetic centers such as the hypothalamus may cause emetic seizures, while concentrated epileptogenesis in the rolandic area produces sylvian seizures [2]. Kanazawa supported and proved this hypothesis using MEG dipole analysis [8], and we also supported this hypothesis by investigation with the EEG dipole method [7]. Our present study indicates that sylvian seizures develop before emetic seizures in some cases. Therefore, it appears that the diffusion and concentration of epileptogenic foci depends not only on the age, but also on other individual factors. And during the period of cortical hyperexcitability, each patient experiences emetic seizures with occipital involvement, and during the period of concentration of this excitability, the patient experiences the sylvian seizures with rolandic spikes. This notion is supported by the fact that all the patients in this series showed status epilepticus during the period with emetic seizures. In fact, PS is the most common specific cause of afebrile nonconvulsive status epilepticus in childhood. However, prolonged sylvian seizures are rarely observed in BECTS. Parameggiani and Guerrini reported an interesting case of BECTS that showed two types of ictal EEG recordings. One short classic rolandic seizure involved only the rolandic area and the other longer one started from the rolandic area and showed late occipital involvement [11]. They suggested the cortical neurons of both areas are prone to develop prolonged seizures and seizure spread.
Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
H. Yoshinaga et al. / Brain & Development xxx (2014) xxx–xxx
In conclusion, based on the clinical course and the associated EEG findings of the patients who have both emetic seizures and rolandic seizures, it appears that the distribution of the hyperexcitability of the cortex affects the clinical features of the benign partial epilepsy syndromes. However, our explanation of the manifestation of the two syndromes is still hypothetical. Further research on the genetic etiology of these benign agedependent epileptic syndromes is necessary to draw firm conclusions. Acknowledgement This study was supported in part by the Japan Epilepsy Research Foundation. References [1] Beausarrt M. Benign epilepsy of children with Rolandic (centrotemporal) paroxysmal foci. A clinical study. Study of 221 cases. Epilepsia 1972;13:795–811. [2] Panayiotopoulos CP, Micahel M, Sanders S, Valeta T, Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008;131: 2264–86.
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Please cite this article in press as: Yoshinaga H et al. Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy. Brain Dev (2014), http://dx.doi.org/10.1016/j.braindev.2014.01.013
