The Experts below are selected from a list of 9405 Experts worldwide ranked by ideXlab platform
Makiko Osawa - One of the best experts on this subject based on the ideXlab platform.
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Effect of ACTH therapy for epileptic spasms without hypsarrhythmia
Epilepsia, 2005Co-Authors: Hirokazu Oguni, Makoto Funatsuka, Kaori Sasaki, Tae Nakajima, Keisuke Yoshii, Tsutomu Nishimura, Makiko OsawaAbstract:Summary: Purpose: We analyzed the short- and long-term effects of adrenocorticotropic hormone (ACTH) therapy for patients with epileptic spasms (ESs) who did not meet the criteria of West syndrome (WS). Methods: The subjects were 30 patients, including 13 boys and 17 girls, who had received ACTH therapy between 1970 and 2003. We excluded patients with WS, but included those with a history of WS who no longer showed hypsarrhythmia at the period of ACTH therapy. The age at onset of ESs and at ACTH therapy ranged from 2 to 82 months with a median of 18 months, and from 11 to 86 months with a median of 29 months, respectively. Results: Excellent and poor responses were obtained in 19 (63%) and 11 (37%) patients, respectively, as a short-term effect. Although the patients could be subclassified into five subgroups according to the previous reports, no difference was seen in shortterm response to ACTH. Among 17 of the 19 patients with excellent short-term outcomes and a follow-up of >1 year after the ACTH therapy, eight patients have continued to be seizure free (29%; excellent long-term effect), whereas the remaining nine patients had a recurrence of seizures (complex partial seizures, four; generalized tonic seizures, three; ESs, two) at 9 months to 198 months (median, 49 months) after ACTH therapy. In addition, nine of the 17 patients demonstrated a localized frontal EEG focus after the ACTH therapy, although most of these had previously shown diffuse epileptic EEG Abnormality. Conclusions: ACTH therapy is worth trying for patients with resistant ESs, even without features of WS. However, the longterm effect is uncertain because recurrences of various types of seizures, including focal, were frequently observed. Key Words: Epileptic spasms—ACTH therapy—West syndrome— Hypsarrhythmia—Frontal EEG focus. Epileptic spasms (ESs) are the primary seizure manifestation not only in patients with West syndrome (WS) but also in patients with other forms of generalized or focal epilepsies (1‐4). The latter groups are subdivided largely into patients characterized by early onset of ESs without hypsarrhythmia, those with late onset of ESs and clinicoelectrical features of Lennox‐Gastaut syndrome, and those with focal epilepsy manifesting both partial seizures and ESs (2‐4). Detailed studies focusing on ESs in the latter groups have been conducted by several investigators, who demonstrated that the clinical and EEG characteristics of the attacks were identical to those of WS, whereas the interictal EEG pattern manifested no typical hypsarrhythmia (5‐8). The prognosis of the seizures has been reported to be poor, because ESs without hypsarrhythmia (ESwoH) have been resistant to medical treatment and also to the epileptic surgery attempted in some patients
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acquired epileptiform opercular syndrome a case report and results of single photon emission computed tomography and computer assisted electroencephalographic analysis
Brain & Development, 2001Co-Authors: Emiko Tachikawa, Hirokazu Oguni, Makoto Funatsuka, Seigo Shirakawa, Kitami Hayashi, Makiko OsawaAbstract:Abstract We report here a girl aged 5 years 3 months with cryptogenic localization-related epilepsy who showed a prolonged episode characterized by dysarthria, dysphagia, drooling and paresis of the right arm associated with almost continuous diffuse sharp–slow wave complexes during sleep. These symptoms were not directly related to seizures or to each sharp–slow wave complex revealed by examination during the video electroencephalographic (EEG) recording. The interictal single photon emission compute tomography showed a localized high perfusion area in the left posterior frontal region. The introduction of clonazepam completely controlled the clinical symptoms as well as the EEG Abnormality within 2 weeks. After 4 months of remission, a similar episode recurred which was associated with aggravation of EEG. The clinical and EEG characteristics of this patient were identical to those of acquired epileptiform opercular syndrome (AEOS), a newly proposed epileptic syndrome, in which a transient operculum syndrome develops in association with continuous spike-and-wave activity during slow sleep (CSWS). Computer-assisted EEG analysis demonstrated that the epileptic EEG focus was located in the left sylvian fissure, and produced secondary bilateral synchronous sharp–slow complexes. The present study further supports the hypothesis that the electrical interference by CSWS creates bilateral opercular dysfunction through the mechanism of secondary bilateral synchrony, thus producing AEOS.
Benjamin D. Philpot - One of the best experts on this subject based on the ideXlab platform.
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Delta rhythmicity is a reliable EEG biomarker in Angelman syndrome: a parallel mouse and human analysis
Journal of Neurodevelopmental Disorders, 2017Co-Authors: Michael S. Sidorov, Ronald L Thibert, Gina M. Deck, Marjan Dolatshahi, Lynne M. Bird, Catherine J. Chu, Benjamin D. PhilpotAbstract:Clinicians have qualitatively described rhythmic delta activity as a prominent EEG Abnormality in individuals with Angelman syndrome, but this phenotype has yet to be rigorously quantified in the clinical population or validated in a preclinical model. Here, we sought to quantitatively measure delta rhythmicity and evaluate its fidelity as a biomarker. We quantified delta oscillations in mouse and human using parallel spectral analysis methods and measured regional, state-specific, and developmental changes in delta rhythms in a patient population. Delta power was broadly increased and more dynamic in both the Angelman syndrome mouse model, relative to wild-type littermates, and in children with Angelman syndrome, relative to age-matched neurotypical controls. Enhanced delta oscillations in children with Angelman syndrome were present during wakefulness and sleep, were generalized across the neocortex, and were more pronounced at earlier ages. Delta rhythmicity phenotypes can serve as reliable biomarkers for Angelman syndrome in both preclinical and clinical settings.
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Delta rhythmicity is a reliable EEG biomarker in Angelman syndrome: a parallel mouse and human analysis
Journal of Neurodevelopmental Disorders, 2017Co-Authors: Michael S. Sidorov, Ronald L Thibert, Gina M. Deck, Marjan Dolatshahi, Lynne M. Bird, Benjamin D. PhilpotAbstract:Background Clinicians have qualitatively described rhythmic delta activity as a prominent EEG Abnormality in individuals with Angelman syndrome, but this phenotype has yet to be rigorously quantified in the clinical population or validated in a preclinical model. Here, we sought to quantitatively measure delta rhythmicity and evaluate its fidelity as a biomarker. Methods We quantified delta oscillations in mouse and human using parallel spectral analysis methods and measured regional, state-specific, and developmental changes in delta rhythms in a patient population. Results Delta power was broadly increased and more dynamic in both the Angelman syndrome mouse model, relative to wild-type littermates, and in children with Angelman syndrome, relative to age-matched neurotypical controls. Enhanced delta oscillations in children with Angelman syndrome were present during wakefulness and sleep, were generalized across the neocortex, and were more pronounced at earlier ages. Conclusions Delta rhythmicity phenotypes can serve as reliable biomarkers for Angelman syndrome in both preclinical and clinical settings.
Ali Bozorg - One of the best experts on this subject based on the ideXlab platform.
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tilt table test misdiagnosis of epilepsy p02 169
Neurology, 2012Co-Authors: Deepali Jain, Ali BozorgAbstract:Objective: To discuss four cases, where diagnosis of epilepsy was missed secondary to an “abnormal tilt table test.” Background Although patho-physiologically distinct, syncope and seizures share clinical characteristics which may make diagnosis difficult. Syncope may be associated with seizure-like motor manifestations, and seizures may be complicated by cardiac arrhythmia and loss of consciousness. Over the last decade, upright tilt table testing has emerged as an important diagnostic method for the identification of individuals whose syncope is likely to be neurocardiogenic in origin. Design/Methods: We studied all the patients referred to our epilepsy center for evaluation of loss of consciousness between ages 18 to 65 years. This included all outpatient referrals and direct admits to the video-EEG monitoring (VEEM) unit over one year. The diagnosis of epilepsy was made based on the presence of strong interictal EEG abnormalities, or ictal EEG Abnormality correlating with the events to suggest the diagnosis of epilepsy. Results: We diagnosed four patients with epilepsy, who were previously diagnosed with syncope based on “abnormal tilt-table test.” Two patients were diagnosed based on outpatient EEG, during which one patient had clinical and electrographic seizure and the other one had strong evidence of interictal generalized spikes. The remaining two patients had prolonged VEEM, and multiple events were captured, which confirmed the diagnosis of epilepsy. At present time, three out of four patients are seizures-free with appropriate antiepileptic drugs (AEDs), and one has improved dramatically with addition of AEDs. Conclusions: Although the vast majority of patients that neurologist encounters in day to day life with possible syncope truly suffer from syncope, a small fraction of them may have epilepsy. The correct diagnosis can often be made if emphasis is put on adequate history taking instead of overemphasis on unnecessary ancillary testing. Disclosure: Dr. Jain has nothing to disclose. Dr. Bozorg has nothing to disclose.
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Tilt Table Test & Misdiagnosis of Epilepsy (P02.169)
Neurology, 2012Co-Authors: Deepali Jain, Ali BozorgAbstract:Objective: To discuss four cases, where diagnosis of epilepsy was missed secondary to an “abnormal tilt table test.” Background Although patho-physiologically distinct, syncope and seizures share clinical characteristics which may make diagnosis difficult. Syncope may be associated with seizure-like motor manifestations, and seizures may be complicated by cardiac arrhythmia and loss of consciousness. Over the last decade, upright tilt table testing has emerged as an important diagnostic method for the identification of individuals whose syncope is likely to be neurocardiogenic in origin. Design/Methods: We studied all the patients referred to our epilepsy center for evaluation of loss of consciousness between ages 18 to 65 years. This included all outpatient referrals and direct admits to the video-EEG monitoring (VEEM) unit over one year. The diagnosis of epilepsy was made based on the presence of strong interictal EEG abnormalities, or ictal EEG Abnormality correlating with the events to suggest the diagnosis of epilepsy. Results: We diagnosed four patients with epilepsy, who were previously diagnosed with syncope based on “abnormal tilt-table test.” Two patients were diagnosed based on outpatient EEG, during which one patient had clinical and electrographic seizure and the other one had strong evidence of interictal generalized spikes. The remaining two patients had prolonged VEEM, and multiple events were captured, which confirmed the diagnosis of epilepsy. At present time, three out of four patients are seizures-free with appropriate antiepileptic drugs (AEDs), and one has improved dramatically with addition of AEDs. Conclusions: Although the vast majority of patients that neurologist encounters in day to day life with possible syncope truly suffer from syncope, a small fraction of them may have epilepsy. The correct diagnosis can often be made if emphasis is put on adequate history taking instead of overemphasis on unnecessary ancillary testing. Disclosure: Dr. Jain has nothing to disclose. Dr. Bozorg has nothing to disclose.
Hirokazu Oguni - One of the best experts on this subject based on the ideXlab platform.
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Effect of ACTH therapy for epileptic spasms without hypsarrhythmia
Epilepsia, 2005Co-Authors: Hirokazu Oguni, Makoto Funatsuka, Kaori Sasaki, Tae Nakajima, Keisuke Yoshii, Tsutomu Nishimura, Makiko OsawaAbstract:Summary: Purpose: We analyzed the short- and long-term effects of adrenocorticotropic hormone (ACTH) therapy for patients with epileptic spasms (ESs) who did not meet the criteria of West syndrome (WS). Methods: The subjects were 30 patients, including 13 boys and 17 girls, who had received ACTH therapy between 1970 and 2003. We excluded patients with WS, but included those with a history of WS who no longer showed hypsarrhythmia at the period of ACTH therapy. The age at onset of ESs and at ACTH therapy ranged from 2 to 82 months with a median of 18 months, and from 11 to 86 months with a median of 29 months, respectively. Results: Excellent and poor responses were obtained in 19 (63%) and 11 (37%) patients, respectively, as a short-term effect. Although the patients could be subclassified into five subgroups according to the previous reports, no difference was seen in shortterm response to ACTH. Among 17 of the 19 patients with excellent short-term outcomes and a follow-up of >1 year after the ACTH therapy, eight patients have continued to be seizure free (29%; excellent long-term effect), whereas the remaining nine patients had a recurrence of seizures (complex partial seizures, four; generalized tonic seizures, three; ESs, two) at 9 months to 198 months (median, 49 months) after ACTH therapy. In addition, nine of the 17 patients demonstrated a localized frontal EEG focus after the ACTH therapy, although most of these had previously shown diffuse epileptic EEG Abnormality. Conclusions: ACTH therapy is worth trying for patients with resistant ESs, even without features of WS. However, the longterm effect is uncertain because recurrences of various types of seizures, including focal, were frequently observed. Key Words: Epileptic spasms—ACTH therapy—West syndrome— Hypsarrhythmia—Frontal EEG focus. Epileptic spasms (ESs) are the primary seizure manifestation not only in patients with West syndrome (WS) but also in patients with other forms of generalized or focal epilepsies (1‐4). The latter groups are subdivided largely into patients characterized by early onset of ESs without hypsarrhythmia, those with late onset of ESs and clinicoelectrical features of Lennox‐Gastaut syndrome, and those with focal epilepsy manifesting both partial seizures and ESs (2‐4). Detailed studies focusing on ESs in the latter groups have been conducted by several investigators, who demonstrated that the clinical and EEG characteristics of the attacks were identical to those of WS, whereas the interictal EEG pattern manifested no typical hypsarrhythmia (5‐8). The prognosis of the seizures has been reported to be poor, because ESs without hypsarrhythmia (ESwoH) have been resistant to medical treatment and also to the epileptic surgery attempted in some patients
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acquired epileptiform opercular syndrome a case report and results of single photon emission computed tomography and computer assisted electroencephalographic analysis
Brain & Development, 2001Co-Authors: Emiko Tachikawa, Hirokazu Oguni, Makoto Funatsuka, Seigo Shirakawa, Kitami Hayashi, Makiko OsawaAbstract:Abstract We report here a girl aged 5 years 3 months with cryptogenic localization-related epilepsy who showed a prolonged episode characterized by dysarthria, dysphagia, drooling and paresis of the right arm associated with almost continuous diffuse sharp–slow wave complexes during sleep. These symptoms were not directly related to seizures or to each sharp–slow wave complex revealed by examination during the video electroencephalographic (EEG) recording. The interictal single photon emission compute tomography showed a localized high perfusion area in the left posterior frontal region. The introduction of clonazepam completely controlled the clinical symptoms as well as the EEG Abnormality within 2 weeks. After 4 months of remission, a similar episode recurred which was associated with aggravation of EEG. The clinical and EEG characteristics of this patient were identical to those of acquired epileptiform opercular syndrome (AEOS), a newly proposed epileptic syndrome, in which a transient operculum syndrome develops in association with continuous spike-and-wave activity during slow sleep (CSWS). Computer-assisted EEG analysis demonstrated that the epileptic EEG focus was located in the left sylvian fissure, and produced secondary bilateral synchronous sharp–slow complexes. The present study further supports the hypothesis that the electrical interference by CSWS creates bilateral opercular dysfunction through the mechanism of secondary bilateral synchrony, thus producing AEOS.
Michael S. Sidorov - One of the best experts on this subject based on the ideXlab platform.
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Delta rhythmicity is a reliable EEG biomarker in Angelman syndrome: a parallel mouse and human analysis
Journal of Neurodevelopmental Disorders, 2017Co-Authors: Michael S. Sidorov, Ronald L Thibert, Gina M. Deck, Marjan Dolatshahi, Lynne M. Bird, Catherine J. Chu, Benjamin D. PhilpotAbstract:Clinicians have qualitatively described rhythmic delta activity as a prominent EEG Abnormality in individuals with Angelman syndrome, but this phenotype has yet to be rigorously quantified in the clinical population or validated in a preclinical model. Here, we sought to quantitatively measure delta rhythmicity and evaluate its fidelity as a biomarker. We quantified delta oscillations in mouse and human using parallel spectral analysis methods and measured regional, state-specific, and developmental changes in delta rhythms in a patient population. Delta power was broadly increased and more dynamic in both the Angelman syndrome mouse model, relative to wild-type littermates, and in children with Angelman syndrome, relative to age-matched neurotypical controls. Enhanced delta oscillations in children with Angelman syndrome were present during wakefulness and sleep, were generalized across the neocortex, and were more pronounced at earlier ages. Delta rhythmicity phenotypes can serve as reliable biomarkers for Angelman syndrome in both preclinical and clinical settings.
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Delta rhythmicity is a reliable EEG biomarker in Angelman syndrome: a parallel mouse and human analysis
Journal of Neurodevelopmental Disorders, 2017Co-Authors: Michael S. Sidorov, Ronald L Thibert, Gina M. Deck, Marjan Dolatshahi, Lynne M. Bird, Benjamin D. PhilpotAbstract:Background Clinicians have qualitatively described rhythmic delta activity as a prominent EEG Abnormality in individuals with Angelman syndrome, but this phenotype has yet to be rigorously quantified in the clinical population or validated in a preclinical model. Here, we sought to quantitatively measure delta rhythmicity and evaluate its fidelity as a biomarker. Methods We quantified delta oscillations in mouse and human using parallel spectral analysis methods and measured regional, state-specific, and developmental changes in delta rhythms in a patient population. Results Delta power was broadly increased and more dynamic in both the Angelman syndrome mouse model, relative to wild-type littermates, and in children with Angelman syndrome, relative to age-matched neurotypical controls. Enhanced delta oscillations in children with Angelman syndrome were present during wakefulness and sleep, were generalized across the neocortex, and were more pronounced at earlier ages. Conclusions Delta rhythmicity phenotypes can serve as reliable biomarkers for Angelman syndrome in both preclinical and clinical settings.