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Katsuhiro Kobayashi - One of the best experts on this subject based on the ideXlab platform.
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Predictive factors for relapse of Epileptic Spasms after adrenocorticotropic hormone therapy in West syndrome.
Brain & development, 2015Co-Authors: Yumiko Hayashi, Fumika Endoh, Yoko Ohtsuka, Harumi Yoshinaga, Tomoyuki Akiyama, Katsuhiro KobayashiAbstract:Abstract Purpose To investigate whether serial electroencephalographic (EEG) findings can predict relapse of Epileptic Spasms after synthetic adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS). Subjects and methods Thirty-nine WS patients (8 cryptogenic and 31 symptomatic) were included in this study. These patients received ACTH therapy for the first time and were regularly followed up for more than three years at our hospital. Sixteen patients (41.0%) showed seizure relapse (relapse group) and 23 patients (59.0%) did not show relapse (non-relapse group). We used survival analysis to investigate the influence of etiology and presence of Epileptic discharges after the ACTH therapy on seizure outcome. Results Immediately after the ACTH therapy, etiology was associated with seizure outcome ( p = 0.003). In the early stage (1 month after the ACTH therapy), only the presence of Epileptic discharges ( p = 0.001) had a significant association with seizure outcome, regardless of etiology. Because all relapsed patients were in the symptomatic group, we performed the same statistical analysis on symptomatic WS patient data only. We found that the group with no Epileptic discharges on EEG showed a significantly higher seizure-free rate than those with Epileptic discharges in the early stage ( p = 0.0091). Conclusion This study demonstrated that serial EEG findings after ACTH therapy are significantly related to relapse of Epileptic Spasms.
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Occurrence of bilaterally independent Epileptic Spasms after a corpus callosotomy in West syndrome.
Brain & Development, 2015Co-Authors: Katsuhiro Kobayashi, Hiroshi Baba, Fumika Endoh, Yoshihiro Toda, Makio Oka, Yoko Ohtsuka, Harumi YoshinagaAbstract:We report a patient with intractable West syndrome whose Epileptic Spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery. This case suggests that ESs with a hemispheric origin may appear generalized because of synchronizing effects in the corpus callosum in some patients.
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Late-onset Epileptic Spasms in a female patient with a CASK mutation.
Brain & development, 2015Co-Authors: Tomoshi Nakajiri, Katsuhiro Kobayashi, Makio Oka, Nobuhiko Okamoto, Fuyuki Miya, Kenjiro Kosaki, Harumi YoshinagaAbstract:We report a female patient with late-onset Epileptic Spasms (ESs) of a rare form, distinct from those seen in typical West syndrome, in association with a heterozygous frameshift CASK mutation (c.1896dupC (p.C633fs(∗)2)). She has a phenotype of microcephaly with pontine and cerebellar hypoplasia (MICPCH), and has had intractable ESs in clusters since 3 years 8 months of age with multifocal, particularly bifrontal, Epileptic discharges in electroencephalogram. The available literature on patients with both ESs and CASK mutations has been reviewed, revealing that four of the five female children, including the present girl, had late-onset ESs, in contrast to the four males, who tended toward early-onset ESs.
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Bilaterally independent Epileptic Spasms in a case of Aicardi syndrome
Epileptic disorders : international epilepsy journal with videotape, 2011Co-Authors: Katsuhiro Kobayashi, Harumi Yoshinaga, Kiyoko Watanabe, Yoko OhtsukaAbstract:A girl with Aicardi syndrome was observed to have two distinct types of asymmetric Epileptic Spasms, as detected by ictal video-EEG recording at three months of age. When the two types of spasm concurred, they showed no mutual interactions based on either clinical or EEG aspects. This observation does not support the hypothesis that the brainstem always plays an initiating role in generating Spasms.
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Characteristics of slow waves on EEG associated with Epileptic Spasms.
Epilepsia, 2005Co-Authors: Katsuhiro Kobayashi, Makio Oka, Harumi Yoshinaga, Tatsuya Ogino, Takushi Inoue, Yoko OhtsukaAbstract:Summary: Purpose: The high-voltage slow waves (HVSs) on EEG associated with Epileptic Spasms were investigated to clarify their characteristics and their relation to the pathophysiology of Spasms in West syndrome and related disorders. Methods: In 14 patients, digitally recorded EEG segments showing the ictal HVSs were extracted and their traces were overlaid by using an average reference. The ictal HVSs were also averaged to build maps for investigation of the pattern of potential distribution over the scalp. Results: In a total of 685 recorded Spasms, 346 (50.5%) with minimal artifacts were selected to demonstrate that the ictal HVSs had a largely consistent waveform and distribution in each patient. The ictal HVSs were symmetrical in 10 patients and asymmetrical in the other four, and were relatively negative over the posterior region and positive over the frontal or temporal regions in 11 patients. Two symptomatic patients showed a marked deviation of the distribution of HVSs to the pathologically more involved hemisphere. An infant with Aicardi syndrome had two different types of Spasms, each type showing a consistent pattern of HVSs with a lateralized distribution. Conclusions: The patterns of distribution of the ictal HVS may be related to the abnormal activation of the brain in the generation of Spasms.
Hiroshi Otsubo - One of the best experts on this subject based on the ideXlab platform.
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the fault in their stars accumulating astrocytic inclusions associated with clusters of Epileptic Spasms in children with global developmental delay
Pediatric Neurology, 2017Co-Authors: Robyn Whitney, Ayako Ochi, Hiroshi Otsubo, James T. Rutka, Shelly K. Weiss, Bláthnaid Mccoy, Ivanna Yau, Lili-naz Hazrati, Sameer Almehmadi, Carter O SneadAbstract:Abstract Background The presence of cerebral astrocytic inclusions recently has been described in a subset of children with early-onset refractory epilepsy, with or without structural brain malformations, and varying degrees of developmental delay. Methods We describe two new individuals with epilepsy with astrocytic inclusions and suggest that in some children this disorder may represent a unique hemispheric epilepsy. We review previously reported individuals with epilepsy with astrocytic inclusions. Results Two children with early onset epilepsy with astrocytic inclusions had refractory clusters of Epileptic Spasms, developmental delay, abnormal neuroimaging, and hemispheric or diffuse interictal epileptiform discharges. In both children, the initial focal resection of the putative epileptogenic zone was unsuccessful and pathology failed to show astrocytic inclusions. Subsequently, both children underwent functional hemispherectomy due to ongoing clusters of Epileptic Spasms, and the presence of multilobar astrocytic inclusions was demonstrated. Postoperatively, both children have remained seizure free in the short-term with improved development. Conclusions We highlight that functional hemispherectomy may be required for seizure control in a select subset of children with clusters of Epileptic Spasms, astrocytic inclusions, and global developmental delay. Given the small number of documented patients, however, ongoing collaboration is needed to better understand the pathophysiology of this condition and determine the optimal way to diagnose and manage these children.
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The Fault in Their Stars—Accumulating Astrocytic Inclusions Associated With Clusters of Epileptic Spasms in Children With Global Developmental Delay
Pediatric neurology, 2017Co-Authors: Robyn Whitney, Ayako Ochi, Hiroshi Otsubo, James T. Rutka, Shelly K. Weiss, Sameer Al-mehmadi, Bláthnaid Mccoy, Ivanna Yau, Lili-naz Hazrati, O. Carter SneadAbstract:Abstract Background The presence of cerebral astrocytic inclusions recently has been described in a subset of children with early-onset refractory epilepsy, with or without structural brain malformations, and varying degrees of developmental delay. Methods We describe two new individuals with epilepsy with astrocytic inclusions and suggest that in some children this disorder may represent a unique hemispheric epilepsy. We review previously reported individuals with epilepsy with astrocytic inclusions. Results Two children with early onset epilepsy with astrocytic inclusions had refractory clusters of Epileptic Spasms, developmental delay, abnormal neuroimaging, and hemispheric or diffuse interictal epileptiform discharges. In both children, the initial focal resection of the putative epileptogenic zone was unsuccessful and pathology failed to show astrocytic inclusions. Subsequently, both children underwent functional hemispherectomy due to ongoing clusters of Epileptic Spasms, and the presence of multilobar astrocytic inclusions was demonstrated. Postoperatively, both children have remained seizure free in the short-term with improved development. Conclusions We highlight that functional hemispherectomy may be required for seizure control in a select subset of children with clusters of Epileptic Spasms, astrocytic inclusions, and global developmental delay. Given the small number of documented patients, however, ongoing collaboration is needed to better understand the pathophysiology of this condition and determine the optimal way to diagnose and manage these children.
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histopathology of cortex and white matter in pediatric Epileptic Spasms comparison with those of partial seizures
Brain & Development, 2012Co-Authors: Yukiko Inage, William Halliday, Ayako Ochi, Tomoyuki Akiyama, Mari Akiyama, Elysa Widjaja, Hiroshi OtsuboAbstract:Epileptic Spasms in older children have increasingly been recognized as a distinct seizure type and subset of these patients are considered for surgical resection. This study compares histopathology and magnetic resonance imaging (MRI), especially focusing the difference between the cortical grey matter and the subcortical white matter to understand the extensive Epileptic brain in patients with Epileptic Spasms. We examined 22 patients consisting of 11 patients with Epileptic Spasms and 11 with partial seizures. Scalp video electroencephalography (EEG) showed interictal generalized epileptiform discharges (9 patients with Epileptic Spasms vs. 1 with partial seizures) and ictal generalized epileptiform discharges (10 vs. 3). We found MRI abnormalities in a single lobe (6 vs. 7) and multiple lobes (2 vs. 1). Surgical resections were performed across multiple lobes (9 vs. 2), comparing within a single lobe (2 vs. 9), (p < 0.001). Histopathology showed abnormal cortical organizations as FCD (2 vs. 5) and microdysgenesis (4 vs. 4), normal (4 vs. 1). Two patients with Epileptic Spasms showed hyaline proteoplasmic astrocytopathy. There were heterotopic neurons (10 vs. 10), cluster of oligodendroglia (8 vs. 7), balloon cells (2 vs. 5) and blurred myelination (1 vs. 4), in the white matter. Seizure-free outcomes were seen in seven patients with Epileptic Spasms (64%) and four with partial seizures (36%). The multilobar epileptogenic zones existed in patients with Epileptic Spasms, compared with the focal epileptogenic zone in patients with partial seizures. There was no difference of MRI and histopathology findings in cortex and subcortical white matter between two groups.
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Histopathology of cortex and white matter in pediatric Epileptic Spasms: comparison with those of partial seizures.
Brain & development, 2011Co-Authors: Yukiko Inage, William Halliday, Ayako Ochi, Tomoyuki Akiyama, Mari Akiyama, Elysa Widjaja, Hiroshi OtsuboAbstract:Epileptic Spasms in older children have increasingly been recognized as a distinct seizure type and subset of these patients are considered for surgical resection. This study compares histopathology and magnetic resonance imaging (MRI), especially focusing the difference between the cortical grey matter and the subcortical white matter to understand the extensive Epileptic brain in patients with Epileptic Spasms. We examined 22 patients consisting of 11 patients with Epileptic Spasms and 11 with partial seizures. Scalp video electroencephalography (EEG) showed interictal generalized epileptiform discharges (9 patients with Epileptic Spasms vs. 1 with partial seizures) and ictal generalized epileptiform discharges (10 vs. 3). We found MRI abnormalities in a single lobe (6 vs. 7) and multiple lobes (2 vs. 1). Surgical resections were performed across multiple lobes (9 vs. 2), comparing within a single lobe (2 vs. 9), (p
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Epileptic Spasms in older pediatric patients: MEG and ictal high-frequency oscillations suggest focal-onset seizures in a subset of Epileptic Spasms.
Epilepsy Research, 2008Co-Authors: Rajesh Ramachandrannair, Katsumi Imai, Ayako Ochi, Tomoyuki Akiyama, Mony Benifla, Stephanie Holowka, James T. Rutka, O. Carter Snead, Hiroshi OtsuboAbstract:Summary Objectives To elucidate the pathophysiology of intractable Epileptic Spasms in older children by describing the interictal magnetoencephalography spike sources (MEGSSs), intracranial EEG ictal-onset zones (IOZs) and their ictal high-frequency oscillations (HFOs) and surgical outcomes. Methods We studied five patients (4.5–14 years) who underwent surgery following intracranial video-EEG (VEEG) monitoring. We analyzed clinical profiles, MRIs, scalp and intracranial VEEGs, and MEGSSs. We localized ictal HFOs using a sampling rate of 1000 Hz and multiple band frequency analysis (MBFA). Results Seizure onset ranged from 0.4 to 8 years. Three patients presented with asymmetrical Spasms. Interictal scalp VEEG recorded predominantly unilateral epileptiform discharges in four; generalized and multifocal in one. Ictal scalp VEEG showed generalized high-amplitude slow waves with superimposed fast waves in four patients; hemispheric electrodecremental episodes in one. MRI findings were normal in three, hemispheric polymicrogyria and periventricular heterotopia in one each. All patients had unilateral MEGSS clusters. Ictal HFOs, ranging from 150 to 250 Hz, localized over Rolandic and frontal regions in four, with one also having extensive temporo-occipital HFOs. After cortical resection, three patients were seizure free; one had >90% reduction in seizure frequency. One patient experienced residual seizures after resection of the hemispheric ictal HFO region. Conclusion Unilateral clusters of MEGSSs overlapped regional IOZs in older patients with Epileptic Spasms. High spatio-temporal MBFA before and during Spasms revealed the regional ictal HFOs. Seizure-free outcomes following resection of zone with MEGSS clusters and ictal HFOs suggested that a subset of Epileptic Spasms was focal-onset seizures.
Harry T. Chugani - One of the best experts on this subject based on the ideXlab platform.
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Epileptic Spasms in paediatric post-traumatic epilepsy at a tertiary referral centre.
Epileptic disorders : international epilepsy journal with videotape, 2017Co-Authors: Jun T. Park, Harry T. ChuganiAbstract:AimTo recognize Epileptic Spasms (ES) as a seizure type after traumatic brain injury (TBI), accidental or non-accidental, in infants and children. In the process, we aim to gain some insight into the mechanisms of epileptogenesis in ES.
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Evolution of Surgical Management for Intractable Epileptic Spasms.
Seminars in pediatric neurology, 2016Co-Authors: Salman Rashid, Harry T. ChuganiAbstract:The understanding and management of Epileptic Spasms has considerably evolved since the mid 19th century. The realization that Epileptic Spasms can be generated from a focal brain lesion played a pivotal role in the development of neurosurgical management for intractable forms of this epilepsy. During pre-surgical planning, the addition of functional FDG PET imaging has further refined the electroencephalographic localization of epileptogenic lesions. In some cases, neurosurgical resection of a focus that is co-localized by the FDG PET scan and electroencephalography can lead to partial or complete reversal of developmental delay along with reduced seizure frequency or seizure freedom. In cases where near-complete hemispheric cortex is implicated in spasm generation, subtotal hemispherectomy has shown encouraging results. Moreover, palliative resection of the major perpetrating focus in carefully chosen patients with bilateral multifocal Spasms has also led to favorable outcomes. However, in patients with tuberous sclerosis with high tuber burden, the localizing value of FDG PET imaging may be limited. In such cases, employment of AMT PET technology has become a valuable tool for localization of actively epileptogenic tubers. This article highlights the historic steps in the successful advancements of neurosurgical interventions for the treatment of intractable Epileptic Spasms.
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Ictal high-frequency oscillations at 80-200 Hz coupled with delta phase in Epileptic Spasms.
Epilepsia, 2011Co-Authors: Hiroki Nariai, Csaba Juhász, Sandeep Sood, Harry T. Chugani, Tetsuro Nagasawa, Naoyuki Matsuzaki, Eishi AsanoAbstract:SUMMARY Previous studies of Epileptic Spasms reported that ictal events were associated with high-frequency oscillations (HFOs) or delta waves involving widespread regions. We determined whether ictal HFOs at 80–200 Hz were coupled with a phase of slow-wave, whether ictal slowwaves were diffusely or locally synchronous signals, and whether the mode of coupling between HFOs and slowwave phases differed between ictal and interictal states. We studied 11 children who underwent extraoperative electrocorticography (ECoG) recording. The phases and amplitudes of slow-waves were measured at the peak of ictal and interictal HFOs in the seizure-onset sites. Ictal HFOs were locked tightly to the phase of slow-wave at £1 Hz. Ictal slow-waves propagated from the seizureonset site to other regions. In contrast, interictal HFOs in the seizure-onset site were loosely locked to the phase of slow-wave at £1 Hz but tightly to that of ‡3-Hz. Ictal slow-waves coupled with HFOs can be explained as near-field and locally synchronized potentials generated by the neocortex rather than far-field potentials generated by subcortical structures. Ictal slow-waves in Epileptic Spasms may be generated by a mechanism different from what generates interictal HFOs–slow
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Statistical mapping of ictal high-frequency oscillations in Epileptic Spasms.
Epilepsia, 2010Co-Authors: Hiroki Nariai, Csaba Juhász, Sandeep Sood, Harry T. Chugani, Tetsuro Nagasawa, Eishi AsanoAbstract:Summary Purpose: We assessed 636 Epileptic Spasms seen in 11 children (median 44 Spasms per child) and determined the spatial and temporal characteristics of ictal high-frequency oscillations (HFOs) in relation to the onset of Spasms. Methods: Electrocorticography (ECoG) signals were sampled from 104–148 cortical sites per child, and the dynamic changes of ictal HFOs were animated on each individual’s three-dimensional (3D) magnetic resonance (MR) image surface. Key Findings: Visual assessment of ictal ECoG recordings revealed that each spasm event was characterized by augmentation of HFOs. Time-frequency analysis demonstrated that ictal augmentation of HFOs at 80–200 Hz was most prominent and generally preceded those at 210–300 Hz and at 70 Hz and slower. Recruitment of HFOs in the rolandic cortex preceded the clinical onset objectively visualized as electromyographic deflection. The presence or absence of ictal motor symptoms was related more to the amplitude of HFOs in the Rolandic cortex than in the seizure-onset zone. In a substantial proportion of Epileptic Spasms, seizure termination began at the seizure-onset zone and propagated to the surrounding areas; we referred to this observation as the “ictal doughnut phenomenon.” Univariate analysis suggested that complete resection of the sites showing the earliest augmentation of ictal HFOs was associated with a good surgical outcome. Significance: Recruitment of HFOs at 80–200 Hz in the rolandic area may play a role in determining seizure semiology in Epileptic Spasms. Our study using macroelectrodes demonstrated that ictal HFOs at 80–200 Hz preceded those at 210–300 Hz.
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Origin and Propagation of Epileptic Spasms Delineated on Electrocorticography
Epilepsia, 2005Co-Authors: Eishi Asano, Csaba Juhász, Aashit Shah, Otto Muzik, Diane C. Chugani, Jagdish Shah, Sandeep Sood, Harry T. ChuganiAbstract:Epileptic Spasms are characterized by clusters of short contractions typically involving the axial muscles and proximal limb segments (1). Because the Spasms begin mostly in infancy between months 3 and 12, they were previously called “infantile Spasms.” This seizure semiology is referred to as “Epileptic Spasms” in the present study, because Spasms occasionally persist or may even have their onset in older children (2–4). West syndrome is characterized by the triad of Epileptic Spasms, hypsarrhythmic EEG pattern, and arrest in psychomotor development, but not all children with Epileptic Spasms show hypsarrhythmia on interictal EEG or developmental delays (1,5,6). In addition, subsets of patients with Epileptic Spasms have other types of Epileptic seizures, such as partial seizures (1,2,7–10). In patients with epilepsy, intracranial digital electro-corticography (ECoG) monitoring is currently considered the gold standard to assess neuronal activity, including ictal discharges, with a temporal resolution of ≥5 ms and a spatial resolution of 1 cm (11,12). Ictal ECoG patterns associated with Epileptic Spasms have been briefly described as “diffuse fast wave activity” in a single patient (4), but more detailed descriptions or systematic studies of ictal ECoG changes during Spasms are not available. It remains unclear how focal features on ECoG recordings before or during Epileptic Spasms can be used to tailor cortical resection for alleviating Spasms. In the present study, ictal ECoG changes associated with Epileptic Spasms were assessed on three-dimensional reconstructed magnetic resonance imaging (MRI) in children who underwent long-term ECoG monitoring for subsequent cortical resection, and the quantitatively processed ictal ECoG findings were compared with interictal ECoG findings, neuroimaging abnormalities, and surgical outcomes.
Harumi Yoshinaga - One of the best experts on this subject based on the ideXlab platform.
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Predictive factors for relapse of Epileptic Spasms after adrenocorticotropic hormone therapy in West syndrome.
Brain & development, 2015Co-Authors: Yumiko Hayashi, Fumika Endoh, Yoko Ohtsuka, Harumi Yoshinaga, Tomoyuki Akiyama, Katsuhiro KobayashiAbstract:Abstract Purpose To investigate whether serial electroencephalographic (EEG) findings can predict relapse of Epileptic Spasms after synthetic adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS). Subjects and methods Thirty-nine WS patients (8 cryptogenic and 31 symptomatic) were included in this study. These patients received ACTH therapy for the first time and were regularly followed up for more than three years at our hospital. Sixteen patients (41.0%) showed seizure relapse (relapse group) and 23 patients (59.0%) did not show relapse (non-relapse group). We used survival analysis to investigate the influence of etiology and presence of Epileptic discharges after the ACTH therapy on seizure outcome. Results Immediately after the ACTH therapy, etiology was associated with seizure outcome ( p = 0.003). In the early stage (1 month after the ACTH therapy), only the presence of Epileptic discharges ( p = 0.001) had a significant association with seizure outcome, regardless of etiology. Because all relapsed patients were in the symptomatic group, we performed the same statistical analysis on symptomatic WS patient data only. We found that the group with no Epileptic discharges on EEG showed a significantly higher seizure-free rate than those with Epileptic discharges in the early stage ( p = 0.0091). Conclusion This study demonstrated that serial EEG findings after ACTH therapy are significantly related to relapse of Epileptic Spasms.
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Occurrence of bilaterally independent Epileptic Spasms after a corpus callosotomy in West syndrome.
Brain & Development, 2015Co-Authors: Katsuhiro Kobayashi, Hiroshi Baba, Fumika Endoh, Yoshihiro Toda, Makio Oka, Yoko Ohtsuka, Harumi YoshinagaAbstract:We report a patient with intractable West syndrome whose Epileptic Spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery. This case suggests that ESs with a hemispheric origin may appear generalized because of synchronizing effects in the corpus callosum in some patients.
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Late-onset Epileptic Spasms in a female patient with a CASK mutation.
Brain & development, 2015Co-Authors: Tomoshi Nakajiri, Katsuhiro Kobayashi, Makio Oka, Nobuhiko Okamoto, Fuyuki Miya, Kenjiro Kosaki, Harumi YoshinagaAbstract:We report a female patient with late-onset Epileptic Spasms (ESs) of a rare form, distinct from those seen in typical West syndrome, in association with a heterozygous frameshift CASK mutation (c.1896dupC (p.C633fs(∗)2)). She has a phenotype of microcephaly with pontine and cerebellar hypoplasia (MICPCH), and has had intractable ESs in clusters since 3 years 8 months of age with multifocal, particularly bifrontal, Epileptic discharges in electroencephalogram. The available literature on patients with both ESs and CASK mutations has been reviewed, revealing that four of the five female children, including the present girl, had late-onset ESs, in contrast to the four males, who tended toward early-onset ESs.
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Bilaterally independent Epileptic Spasms in a case of Aicardi syndrome
Epileptic disorders : international epilepsy journal with videotape, 2011Co-Authors: Katsuhiro Kobayashi, Harumi Yoshinaga, Kiyoko Watanabe, Yoko OhtsukaAbstract:A girl with Aicardi syndrome was observed to have two distinct types of asymmetric Epileptic Spasms, as detected by ictal video-EEG recording at three months of age. When the two types of spasm concurred, they showed no mutual interactions based on either clinical or EEG aspects. This observation does not support the hypothesis that the brainstem always plays an initiating role in generating Spasms.
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Characteristics of slow waves on EEG associated with Epileptic Spasms.
Epilepsia, 2005Co-Authors: Katsuhiro Kobayashi, Makio Oka, Harumi Yoshinaga, Tatsuya Ogino, Takushi Inoue, Yoko OhtsukaAbstract:Summary: Purpose: The high-voltage slow waves (HVSs) on EEG associated with Epileptic Spasms were investigated to clarify their characteristics and their relation to the pathophysiology of Spasms in West syndrome and related disorders. Methods: In 14 patients, digitally recorded EEG segments showing the ictal HVSs were extracted and their traces were overlaid by using an average reference. The ictal HVSs were also averaged to build maps for investigation of the pattern of potential distribution over the scalp. Results: In a total of 685 recorded Spasms, 346 (50.5%) with minimal artifacts were selected to demonstrate that the ictal HVSs had a largely consistent waveform and distribution in each patient. The ictal HVSs were symmetrical in 10 patients and asymmetrical in the other four, and were relatively negative over the posterior region and positive over the frontal or temporal regions in 11 patients. Two symptomatic patients showed a marked deviation of the distribution of HVSs to the pathologically more involved hemisphere. An infant with Aicardi syndrome had two different types of Spasms, each type showing a consistent pattern of HVSs with a lateralized distribution. Conclusions: The patterns of distribution of the ictal HVS may be related to the abnormal activation of the brain in the generation of Spasms.
Tomoyuki Akiyama - One of the best experts on this subject based on the ideXlab platform.
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Predictive factors for relapse of Epileptic Spasms after adrenocorticotropic hormone therapy in West syndrome.
Brain & development, 2015Co-Authors: Yumiko Hayashi, Fumika Endoh, Yoko Ohtsuka, Harumi Yoshinaga, Tomoyuki Akiyama, Katsuhiro KobayashiAbstract:Abstract Purpose To investigate whether serial electroencephalographic (EEG) findings can predict relapse of Epileptic Spasms after synthetic adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS). Subjects and methods Thirty-nine WS patients (8 cryptogenic and 31 symptomatic) were included in this study. These patients received ACTH therapy for the first time and were regularly followed up for more than three years at our hospital. Sixteen patients (41.0%) showed seizure relapse (relapse group) and 23 patients (59.0%) did not show relapse (non-relapse group). We used survival analysis to investigate the influence of etiology and presence of Epileptic discharges after the ACTH therapy on seizure outcome. Results Immediately after the ACTH therapy, etiology was associated with seizure outcome ( p = 0.003). In the early stage (1 month after the ACTH therapy), only the presence of Epileptic discharges ( p = 0.001) had a significant association with seizure outcome, regardless of etiology. Because all relapsed patients were in the symptomatic group, we performed the same statistical analysis on symptomatic WS patient data only. We found that the group with no Epileptic discharges on EEG showed a significantly higher seizure-free rate than those with Epileptic discharges in the early stage ( p = 0.0091). Conclusion This study demonstrated that serial EEG findings after ACTH therapy are significantly related to relapse of Epileptic Spasms.
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histopathology of cortex and white matter in pediatric Epileptic Spasms comparison with those of partial seizures
Brain & Development, 2012Co-Authors: Yukiko Inage, William Halliday, Ayako Ochi, Tomoyuki Akiyama, Mari Akiyama, Elysa Widjaja, Hiroshi OtsuboAbstract:Epileptic Spasms in older children have increasingly been recognized as a distinct seizure type and subset of these patients are considered for surgical resection. This study compares histopathology and magnetic resonance imaging (MRI), especially focusing the difference between the cortical grey matter and the subcortical white matter to understand the extensive Epileptic brain in patients with Epileptic Spasms. We examined 22 patients consisting of 11 patients with Epileptic Spasms and 11 with partial seizures. Scalp video electroencephalography (EEG) showed interictal generalized epileptiform discharges (9 patients with Epileptic Spasms vs. 1 with partial seizures) and ictal generalized epileptiform discharges (10 vs. 3). We found MRI abnormalities in a single lobe (6 vs. 7) and multiple lobes (2 vs. 1). Surgical resections were performed across multiple lobes (9 vs. 2), comparing within a single lobe (2 vs. 9), (p < 0.001). Histopathology showed abnormal cortical organizations as FCD (2 vs. 5) and microdysgenesis (4 vs. 4), normal (4 vs. 1). Two patients with Epileptic Spasms showed hyaline proteoplasmic astrocytopathy. There were heterotopic neurons (10 vs. 10), cluster of oligodendroglia (8 vs. 7), balloon cells (2 vs. 5) and blurred myelination (1 vs. 4), in the white matter. Seizure-free outcomes were seen in seven patients with Epileptic Spasms (64%) and four with partial seizures (36%). The multilobar epileptogenic zones existed in patients with Epileptic Spasms, compared with the focal epileptogenic zone in patients with partial seizures. There was no difference of MRI and histopathology findings in cortex and subcortical white matter between two groups.
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Histopathology of cortex and white matter in pediatric Epileptic Spasms: comparison with those of partial seizures.
Brain & development, 2011Co-Authors: Yukiko Inage, William Halliday, Ayako Ochi, Tomoyuki Akiyama, Mari Akiyama, Elysa Widjaja, Hiroshi OtsuboAbstract:Epileptic Spasms in older children have increasingly been recognized as a distinct seizure type and subset of these patients are considered for surgical resection. This study compares histopathology and magnetic resonance imaging (MRI), especially focusing the difference between the cortical grey matter and the subcortical white matter to understand the extensive Epileptic brain in patients with Epileptic Spasms. We examined 22 patients consisting of 11 patients with Epileptic Spasms and 11 with partial seizures. Scalp video electroencephalography (EEG) showed interictal generalized epileptiform discharges (9 patients with Epileptic Spasms vs. 1 with partial seizures) and ictal generalized epileptiform discharges (10 vs. 3). We found MRI abnormalities in a single lobe (6 vs. 7) and multiple lobes (2 vs. 1). Surgical resections were performed across multiple lobes (9 vs. 2), comparing within a single lobe (2 vs. 9), (p
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Epileptic Spasms in older pediatric patients: MEG and ictal high-frequency oscillations suggest focal-onset seizures in a subset of Epileptic Spasms.
Epilepsy Research, 2008Co-Authors: Rajesh Ramachandrannair, Katsumi Imai, Ayako Ochi, Tomoyuki Akiyama, Mony Benifla, Stephanie Holowka, James T. Rutka, O. Carter Snead, Hiroshi OtsuboAbstract:Summary Objectives To elucidate the pathophysiology of intractable Epileptic Spasms in older children by describing the interictal magnetoencephalography spike sources (MEGSSs), intracranial EEG ictal-onset zones (IOZs) and their ictal high-frequency oscillations (HFOs) and surgical outcomes. Methods We studied five patients (4.5–14 years) who underwent surgery following intracranial video-EEG (VEEG) monitoring. We analyzed clinical profiles, MRIs, scalp and intracranial VEEGs, and MEGSSs. We localized ictal HFOs using a sampling rate of 1000 Hz and multiple band frequency analysis (MBFA). Results Seizure onset ranged from 0.4 to 8 years. Three patients presented with asymmetrical Spasms. Interictal scalp VEEG recorded predominantly unilateral epileptiform discharges in four; generalized and multifocal in one. Ictal scalp VEEG showed generalized high-amplitude slow waves with superimposed fast waves in four patients; hemispheric electrodecremental episodes in one. MRI findings were normal in three, hemispheric polymicrogyria and periventricular heterotopia in one each. All patients had unilateral MEGSS clusters. Ictal HFOs, ranging from 150 to 250 Hz, localized over Rolandic and frontal regions in four, with one also having extensive temporo-occipital HFOs. After cortical resection, three patients were seizure free; one had >90% reduction in seizure frequency. One patient experienced residual seizures after resection of the hemispheric ictal HFO region. Conclusion Unilateral clusters of MEGSSs overlapped regional IOZs in older patients with Epileptic Spasms. High spatio-temporal MBFA before and during Spasms revealed the regional ictal HFOs. Seizure-free outcomes following resection of zone with MEGSS clusters and ictal HFOs suggested that a subset of Epileptic Spasms was focal-onset seizures.
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Epileptic Spasms in older pediatric patients: MEG and ictal high-frequency oscillations suggest focal-onset seizures in a subset of Epileptic Spasms.
Epilepsy research, 2008Co-Authors: Rajesh Ramachandrannair, Katsumi Imai, Ayako Ochi, Tomoyuki Akiyama, Mony Benifla, Stephanie Holowka, James T. Rutka, O. Carter Snead, Hiroshi OtsuboAbstract:To elucidate the pathophysiology of intractable Epileptic Spasms in older children by describing the interictal magnetoencephalography spike sources (MEGSSs), intracranial EEG ictal-onset zones (IOZs) and their ictal high-frequency oscillations (HFOs) and surgical outcomes. We studied five patients (4.5-14 years) who underwent surgery following intracranial video-EEG (VEEG) monitoring. We analyzed clinical profiles, MRIs, scalp and intracranial VEEGs, and MEGSSs. We localized ictal HFOs using a sampling rate of 1000 Hz and multiple band frequency analysis (MBFA). Seizure onset ranged from 0.4 to 8 years. Three patients presented with asymmetrical Spasms. Interictal scalp VEEG recorded predominantly unilateral epileptiform discharges in four; generalized and multifocal in one. Ictal scalp VEEG showed generalized high-amplitude slow waves with superimposed fast waves in four patients; hemispheric electrodecremental episodes in one. MRI findings were normal in three, hemispheric polymicrogyria and periventricular heterotopia in one each. All patients had unilateral MEGSS clusters. Ictal HFOs, ranging from 150 to 250 Hz, localized over Rolandic and frontal regions in four, with one also having extensive temporo-occipital HFOs. After cortical resection, three patients were seizure free; one had >90% reduction in seizure frequency. One patient experienced residual seizures after resection of the hemispheric ictal HFO region. Unilateral clusters of MEGSSs overlapped regional IOZs in older patients with Epileptic Spasms. High spatio-temporal MBFA before and during Spasms revealed the regional ictal HFOs. Seizure-free outcomes following resection of zone with MEGSS clusters and ictal HFOs suggested that a subset of Epileptic Spasms was focal-onset seizures.