Panayiotopoulos Syndrome

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Colin D Ferrie - One of the best experts on this subject based on the ideXlab platform.

  • antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos Syndrome clinical practice survey and clinical trial feasibility
    Archives of Disease in Childhood, 2015
    Co-Authors: Louise C Mellish, Colin Dunkley, Colin D Ferrie
    Abstract:

    Background The evidence base for management of childhood epilepsy is poor, especially for the most common specific Syndromes such as rolandic epilepsy (RE) and Panayiotopoulos Syndrome (PS). Considerable international variation in management and controversy about non-treatment indicate the need for high quality randomised controlled trials (RCT). The aim of this study is, therefore, to describe current UK practice and explore the feasibility of different RCT designs for RE and PS. Methods We conducted an online survey of 590 UK paediatricians who treat epilepsy. Thirty-two questions covered annual caseload, investigation and management practice, factors influencing treatment, antiepileptic drug preferences and hypothetical trial design preferences. Results 132 responded (22%): 81% were paediatricians and 95% at consultant seniority. We estimated, annually, 751 new RE cases and 233 PS cases. Electroencephalography (EEG) is requested at least half the time in approximately 70% of cases; MRI brain at least half the time in 40%–65% cases and neuropsychological evaluation in 7%–8%. Clinicians reported non-treatment in 40%: main reasons were low frequency of seizures and parent/child preferences. Carbamazepine is the preferred older, and levetiracetam the preferred newer, RCT arm. Approximately one-half considered active and placebo designs acceptable, choosing seizures as primary and cognitive/behavioural measures as secondary outcomes. Conclusions Management among respondents is broadly in line with national guidance, although with possible overuse of brain imaging and underuse of EEG and neuropsychological assessments. A large proportion of patients in the UK remains untreated, and clinicians seem amenable to a range of RCT designs, with carbamazepine and levetiracetam the preferred active drugs.

  • syncope like epileptic seizures in Panayiotopoulos Syndrome
    Neurology, 2012
    Co-Authors: Michael Koutroumanidis, Colin D Ferrie, Sue Sanders, Michael Michael, Thalia Valeta, CP Panayiotopoulos
    Abstract:

    Objective: To describe the clinical features of syncope-like epileptic seizures (SLES) and their frequency in Panayiotopoulos Syndrome (PS). Methods: This was a 6-year prospective study of all children aged 1−15 years referred for an EEG. PS was defined by the occurrence of at least one autonomic seizure (AS) in a neurodevelopmentally normal child and at least one EEG with focal spikes. SLES were defined as self-terminating events of sudden loss of postural tone and unresponsiveness, occurring either concurrently with other ictal autonomic symptoms and signs that characterize PS (AS + SLES) or on their own (pure SLES). Results: PS was diagnosed in 33 of 394 consecutive children with at least one afebrile seizure (8.4%). SLES occurred at least once in 17 of 33 children (51.5%); 12 presented SLES in all their AS, and 5 had also AS without SLES. Overall, 53 of 74 AS manifested with SLES (71.6%); 25 were AS + SLES and 28 were pure SLES. The latter occurred in 7 children suddenly and without premonition or obvious triggers while standing, sitting, lying down, or asleep, did not resolve in the horizontal position, and were not associated with stiffening or any involuntary movements, even when longer than a few minutes. Concurrent autonomic symptoms during AS + SLES included emesis, incontinence, mydriasis, miosis, and cardiorespiratory abnormalities. Conclusions: SLES is a common ictal manifestation of PS and should be considered in the differential diagnosis of suspected syncope, particularly when clinical signs are atypical for neurocardiogenic syncope and the EEG shows focal spikes.

  • Panayiotopoulos Syndrome an important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non epileptic disorders
    Brain & Development, 2010
    Co-Authors: Michael Michael, Katerina Tsatsou, Colin D Ferrie
    Abstract:

    Abstract Panayiotopoulos Syndrome is a common multifocal autonomic childhood epileptic disorder with significant clinical, pathophysiological and management implications. It affects otherwise normal children with onset at around 3–6 years. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting. EEG shows shifting and/or multiple foci, often with occipital dominance. Despite characteristic clinical and EEG manifestations Panayiotopoulos Syndrome is often confused with occipital epilepsy and acute non-epileptic disorders such as encephalitis, syncope, cyclic vomiting or atypical migraine. This review aims to describe Panayiotopoulos Syndrome on the basis of independent major studies and provide clinical clues for diagnosis and management.

  • autonomic status epilepticus in Panayiotopoulos Syndrome and other childhood and adult epilepsies a consensus view
    Epilepsia, 2007
    Co-Authors: Colin D Ferrie, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Osamu Kanazawa, Roberto Horacio Caraballo, Natalio Fejerman, Lucia Fusco, R A Grunewald, Michael Koutroumanidis
    Abstract:

    Summary: Purpose: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management. Methods: An international consortium of established researchers in the field was identified from their published work, agreed the purpose of the project, searched the literature, and, by use of e-mail communication, agreed the consensus document. Results: Autonomic SE is a condition lasting at least 30 min and characterized by epileptic activity causing altered autonomic function of any type at seizure onset or in which manifestations consistent with altered autonomic function are prominent (quantitatively dominant or clinically important) even if not present at seizure onset. It is best described, and probably most commonly encountered in children, with Panayiotopoulos Syndrome. However, it also occurs in children with symptomatic epilepsies and, exceptionally, in adults. Its pathogenesis and most appropriate management are poorly understood Conclusions: It is hoped that this document will help clinical recognition of Autonomic SE, reduce misdiagnosis, and promote further interest and studies into what has been a relatively neglected area. Key Words: Epileptic seizures— Epilepsy—Status epilepticus—Autonomic nervous system— Consensus statement.

  • Panayiotopoulos Syndrome a consensus view
    Developmental Medicine & Child Neurology, 2006
    Co-Authors: Colin D Ferrie, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Michael Koutroumanidis, Hirokazu Oguni, Roberto Horacio Caraballo, Sara Kivity, Zarko Martinovic, Alberto Verrotti
    Abstract:

    The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of Panayiotopoulos Syndrome (PS). An international consortium of established researchers in the field collaborated to produce a consensus document. The resulting document defines PS, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. We conclude that PS is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.

Michael Koutroumanidis - One of the best experts on this subject based on the ideXlab platform.

  • Case Report Recurrent autonomic status epilepticus in Panayiotopoulos Syndrome: Video/EEG studies
    2020
    Co-Authors: Michael Koutroumanidis, Shaun Rowlinson, Sue Sanders
    Abstract:

    We describe a neurologically and developmentally normal child with infrequent seizures characterized by emetic symptoms and other autonomic phenomena and interictal spikes that satisfy the diagnostic criteria of Panayiotopoulos Syndrome (PS). Two video/ EEG recordings, taken a year apart, revealed prolonged autonomic seizures and other subtle behavioral changes, suggesting that episodes of nonconvulsive status epilepticus in PS may be more frequent than appreciated. 2005 Elsevier Inc. All rights reserved.

  • syncope like epileptic seizures in Panayiotopoulos Syndrome
    Neurology, 2012
    Co-Authors: Michael Koutroumanidis, Colin D Ferrie, Sue Sanders, Michael Michael, Thalia Valeta, CP Panayiotopoulos
    Abstract:

    Objective: To describe the clinical features of syncope-like epileptic seizures (SLES) and their frequency in Panayiotopoulos Syndrome (PS). Methods: This was a 6-year prospective study of all children aged 1−15 years referred for an EEG. PS was defined by the occurrence of at least one autonomic seizure (AS) in a neurodevelopmentally normal child and at least one EEG with focal spikes. SLES were defined as self-terminating events of sudden loss of postural tone and unresponsiveness, occurring either concurrently with other ictal autonomic symptoms and signs that characterize PS (AS + SLES) or on their own (pure SLES). Results: PS was diagnosed in 33 of 394 consecutive children with at least one afebrile seizure (8.4%). SLES occurred at least once in 17 of 33 children (51.5%); 12 presented SLES in all their AS, and 5 had also AS without SLES. Overall, 53 of 74 AS manifested with SLES (71.6%); 25 were AS + SLES and 28 were pure SLES. The latter occurred in 7 children suddenly and without premonition or obvious triggers while standing, sitting, lying down, or asleep, did not resolve in the horizontal position, and were not associated with stiffening or any involuntary movements, even when longer than a few minutes. Concurrent autonomic symptoms during AS + SLES included emesis, incontinence, mydriasis, miosis, and cardiorespiratory abnormalities. Conclusions: SLES is a common ictal manifestation of PS and should be considered in the differential diagnosis of suspected syncope, particularly when clinical signs are atypical for neurocardiogenic syncope and the EEG shows focal spikes.

  • multifocal spatiotemporal distribution of interictal spikes in Panayiotopoulos Syndrome
    Clinical Neurophysiology, 2010
    Co-Authors: Vasileios Kokkinos, Michael Koutroumanidis, Katerina Tsatsou, Andreas M Koupparis, Dimitrios Tsiptsios, Chrysostomos P. Panayiotopoulos
    Abstract:

    Abstract Objective To investigate the spatiotemporal course of interictal spikes in Panayiotopoulos Syndrome (PS), and in particular whether seemingly independent extra-occipital spikes are truly autonomous or secondary, triggered by occipital spikes. Methods Seven children with the most representative interictal spike patterns on visual analysis were studied. Five had a single focus (occipital in two, suggestive of posterior to anterior spike propagation in two, and frontal) and two had ⩾two foci over the posterior and the anterior areas independently. Spikes were marked, clustered and waveform – averaged, and mapped on electrode space. Results The patterns of spatial and temporal dynamics of the interictal spikes were not stereotypical for any brain area, including the occipital lobe. Some of the anterior and the posterior spikes remained focal or showed little spread, but others appeared to propagate to the opposite direction (occipital to frontal and vice versa). Conclusions In PS all cerebral locations are able to spontaneously and independently generate and propagate interictal spikes, indicating that PS is a multifocal epileptic Syndrome. Significance Confirmation of the multifocal character of PS improves clinical diagnosis and challenges our current taxonomic concepts by expanding the anatomical boundaries of a distinct focal epilepsy phenotype from lobar to system.

  • eeg dipole characteristics in Panayiotopoulos Syndrome
    Epilepsia, 2006
    Co-Authors: Harumi Yoshinaga, Michael Koutroumanidis, Kenichi Kikumoto, Katsuhiro Kobayashi, Atsushi Shirasawa, Takushi Inoue, Yoko Ohtsuka
    Abstract:

    Summary: Purpose: Panayiotopoulos Syndrome (PS) is a type of benign childhood partial epilepsy that is frequently associated with abundant multifocal spikes other than main occipital spikes on the EEG. In this study, we investigated the characteristic features of dipoles in PS. Methods: We performed dipole analysis of the interictal occipital spike discharges seen in 10 children with PS (group A) and in 10 children with other types of symptomatic localization-related epilepsy (group B). We analyzed the dipoles of the averaged spike in each patient. Results: In group A, the averaged occipital spikes in each patient showed dense dipole locations in the mesial occipital area; in group B, widely scattered dipole locations were observed. In Group A, the geometric centers of the dipoles at each time point (such as at the main negative peak and before or after the main peak) were estimated in the neighboring locations. In contrast, they tended to be scattered in group B. Conclusions: Our study reveals that PS has high dipole stability, similar to that of rolandic epilepsy. From the electroencephalographic view, this seems to indicate a close link between these two Syndromes.

  • Panayiotopoulos Syndrome a consensus view
    Developmental Medicine & Child Neurology, 2006
    Co-Authors: Colin D Ferrie, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Michael Koutroumanidis, Hirokazu Oguni, Roberto Horacio Caraballo, Sara Kivity, Zarko Martinovic, Alberto Verrotti
    Abstract:

    The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of Panayiotopoulos Syndrome (PS). An international consortium of established researchers in the field collaborated to produce a consensus document. The resulting document defines PS, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. We conclude that PS is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.

Athanasios Covanis - One of the best experts on this subject based on the ideXlab platform.

  • autonomic status epilepticus in Panayiotopoulos Syndrome and other childhood and adult epilepsies a consensus view
    Epilepsia, 2007
    Co-Authors: Colin D Ferrie, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Osamu Kanazawa, Roberto Horacio Caraballo, Natalio Fejerman, Lucia Fusco, R A Grunewald, Michael Koutroumanidis
    Abstract:

    Summary: Purpose: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management. Methods: An international consortium of established researchers in the field was identified from their published work, agreed the purpose of the project, searched the literature, and, by use of e-mail communication, agreed the consensus document. Results: Autonomic SE is a condition lasting at least 30 min and characterized by epileptic activity causing altered autonomic function of any type at seizure onset or in which manifestations consistent with altered autonomic function are prominent (quantitatively dominant or clinically important) even if not present at seizure onset. It is best described, and probably most commonly encountered in children, with Panayiotopoulos Syndrome. However, it also occurs in children with symptomatic epilepsies and, exceptionally, in adults. Its pathogenesis and most appropriate management are poorly understood Conclusions: It is hoped that this document will help clinical recognition of Autonomic SE, reduce misdiagnosis, and promote further interest and studies into what has been a relatively neglected area. Key Words: Epileptic seizures— Epilepsy—Status epilepticus—Autonomic nervous system— Consensus statement.

  • Panayiotopoulos Syndrome a benign childhood autonomic epilepsy frequently imitating encephalitis syncope migraine sleep disorder or gastroenteritis
    Pediatrics, 2006
    Co-Authors: Athanasios Covanis
    Abstract:

    BACKGROUND. Panayiotopoulos Syndrome is a common idiopathic childhood-specific seizure disorder formally recognized by the International League Against Epilepsy. An expert consensus has defined Panayiotopoulos Syndrome as “a benign age-related focal seizure disorder occurring in early and mid-childhood. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms, and by an EEG [electroencephalogram] that shows shifting and/or multiple foci, often with occipital predominance.” OBJECTIVE. The purpose of this review is to provide guidance for appropriate diagnosis and management of Panayiotopoulos Syndrome. CLINICAL FEATURES. Autonomic epileptic seizures and autonomic status epilepticus are the cardinal manifestations of Panayiotopoulos Syndrome. Autonomic seizures in Panayiotopoulos Syndrome consist of episodes of disturbed autonomic function with emesis as the predominant symptom. Other autonomic manifestations include pallor (or, less often, flushing or cyanosis), mydriasis (or, less often, miosis), cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal motility. In approximately one fifth of the seizures the child becomes unresponsive and flaccid (ictal syncope) before or often without convulsions. Cardiorespiratory arrest is exceptional. More-conventional seizure symptoms often appear after the onset of autonomic manifestations. The child, who was initially fully conscious, becomes confused and unresponsive. Eyes turn to one side or gaze widely open. Only half of the seizures end with brief hemiconvulsions or generalized convulsions. Convulsive status epilepticus is extremely rare. Autonomic symptoms may be the only features of the seizures. Half of the seizures in Panayiotopoulos Syndrome last for >30 minutes, thus constituting autonomic status epilepticus, which is the more common nonconvulsive status epilepticus in normal children. Two thirds of seizures occur during sleep. EPIDEMIOLOGY. Panayiotopoulos Syndrome probably affects 13% of children aged 3 to 6 years who have had 1 or more afebrile seizures and 6% of such children in the 1- to 15-year age group. DIAGNOSTIC TESTS. An electroencephalogram is the only investigation with abnormal results, usually showing multiple spikes in various brain locations. PATHOPHYSIOLOGY. Panayiotopoulos Syndrome is probably the early-onset and Rolandic epilepsy the late-onset phenotype of a maturation-related benign childhood seizure-susceptibility Syndrome. Ictal epileptic discharges in Panayiotopoulos Syndrome, irrespective of their location at onset, activate autonomic disturbances and emesis, to which children are particularly vulnerable. The symptoms/sequence of autonomic seizures and autonomic status epilepticus in Panayiotopoulos Syndrome are specific to childhood, and they do not occur in adults. PROGNOSIS. Panayiotopoulos Syndrome is remarkably benign in terms of seizure frequency and evolution. Autonomic status epilepticus imparts no residual neurologic deficit. The risk of epilepsy in adult life seems to be no higher than in the general population. However, autonomic seizures are potentially life-threatening in the rare context of cardiorespiratory arrest, an area in which additional study is required. MISDIAGNOSIS. The clinical features of Panayiotopoulos Syndrome are frequently mistaken as nonepileptic conditions such as acute encephalitis, syncope, migraine, cyclic vomiting Syndrome, motion sickness, sleep disorder, or gastroenteritis. The consequence is avoidable misdiagnosis, high morbidity, and costly mismanagement. MANAGEMENT. Education about Panayiotopoulos Syndrome is the cornerstone of management. Prophylactic treatment with antiepileptic medication may not be needed for most patients. Autonomic status epilepticus in the acute stage needs thorough evaluation; aggressive treatment may cause iatrogenic complications including cardiorespiratory arrest.

  • Panayiotopoulos Syndrome a consensus view
    Developmental Medicine & Child Neurology, 2006
    Co-Authors: Colin D Ferrie, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Michael Koutroumanidis, Hirokazu Oguni, Roberto Horacio Caraballo, Sara Kivity, Zarko Martinovic, Alberto Verrotti
    Abstract:

    The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of Panayiotopoulos Syndrome (PS). An international consortium of established researchers in the field collaborated to produce a consensus document. The resulting document defines PS, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. We conclude that PS is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.

  • children with rolandic spikes and ictal vomiting rolandic epilepsy or Panayiotopoulos Syndrome
    Epileptic Disorders, 2003
    Co-Authors: Athanasios Covanis, Christina Lada, Konstantinos Skiadas
    Abstract:

    Centrotemporal spikes are the EEG marker of Rolandic epilepsy, while ictus emeticus is one of the main seizure manifestations of Panayiotopoulos Syndrome. Ictus emeticus has not been reported in Rolandic epilepsy. Out of a population of 1340 children with focal afebrile seizures we studied 24 children who had emetic manifestations in at least one seizure and centrotemporal spikes in at least one EEG. They were of normal neurological status and had a follow‐up of at least two years after the last seizure. All children had sleep EEG following sleep deprivation. Two groups of patients were identified. Group A (12 patients) with EEG centrotemporal spikes only and group B (12 patients) with centrotemporal spikes and spikes in other locations. In 21 patients, ictal emetic manifestations culminated in vomiting and in three only nausea or retching occurred. The commonest presentation was ictus emeticus at onset followed by deviation of the eyes or staring, loss of contact and floppiness. In 79%, seizures occurred during sleep. Autonomic status epilepticus occurred in 37.5%. The mean age at onset was 5.3 years. Overall analysis of the clinical and EEG data points out that the vast majority of these patients primarily suffer from Panayiotopoulos Syndrome. Twenty patients (83%) had ictal semiology typical of Panayiotopoulos Syndrome, but five also had concurrent Rolandic symptoms and four later developed pure Rolandic seizures. The other four patients (17%) had typical Rolandic seizures with concurrent ictus emeticus. These findings suggest a link between Rolandic epilepsy and Panayiotopoulos Syndrome, the two most important phenotypes of the benign childhood seizure susceptibility Syndrome.

  • a study of 43 patients with Panayiotopoulos Syndrome a common and benign childhood seizure susceptibility
    Epilepsia, 2003
    Co-Authors: Christina Lada, Konstantinos Skiadas, Virginia Theodorou, Nomiki Loli, Athanasios Covanis
    Abstract:

    Summary: Purpose: To determine prevalence, clinical, EEG features, and prognosis of Panayiotopoulos Syndrome and to examine the proposition that clinical manifestations are more important than EEG findings. Methods: We analyzed retrospectively the clinical and EEG records of 1,340 children with one or more focal seizures seen in the last 18 years, supplemented with a prospective study from 1998. Panayiotopoulos Syndrome was defined by clinical criteria, mainly ictal emesis, irrespective of EEG findings. Results: We analyzed 43 of 90 patients with Panayiotopoulos Syndrome who were seizure free >2 years. Girls predominated. Mean age at first seizure was 5 years. Seizures consisted mainly of autonomic manifestations; ictal emesis was often the first symptom, culminating in vomiting in 86%. Of nonautonomic manifestations, lateral eye deviation was the most common; visual symptoms were exceptional. Impairment of consciousness ensued in all seizures, half of which ended with hemi or generalized convulsions. Nearly 46.5% of cases had at least one seizure >30 min, constituting autonomic status epilepticus. Seizures during sleep (84%) were more common than those in wakefulness. EEG showed occipital spikes in 29 patients. Of the other 14 cases, five had extraoccipital abnormalities or brief generalized discharges, and nine had normal awake and sleep EEG. Prognosis was excellent. All 43 children have been free of seizures for 2 years, 53% having a single seizure, and 47%, an average two to three seizures. Conclusions: Panayiotopoulos Syndrome is common and needs wider recognition. EEG shows occipital or extraoccipital abnormalities, is normal in one third of patients, and does not determine clinical manifestations or prognosis, which is excellent despite the high prevalence of lengthy seizures. Key Words: Children—Autonomic s eizures—Epileps y— Prognosis.

Michael Koutroumanidis - One of the best experts on this subject based on the ideXlab platform.

  • Panayiotopoulos Syndrome an important electroclinical example of benign childhood system epilepsy
    Epilepsia, 2007
    Co-Authors: Michael Koutroumanidis
    Abstract:

    Summary:  As a result of the converging evidence from multiple large independent studies, Panayiotopoulos Syndrome (PS) is now formally recognized as a distinct clinical entity within the spectrum of benign focal epilepsies of childhood. Clinically, PS is manifested by predominantly autonomic seizures and electrographically with multifocal interictal spikes, while the few published ictal recordings have documented onsets of variable lobar topography. These typical electroclinical features do not allow straightforward assignment to a distinctive cortical area, rendering the term “focal”—as we currently understand it—problematic. This is a critical review of the clinical and EEG features of PS, focusing on those characteristics that may shed some light on its so far elusive pathophysiology. We also explore its electroclinical similarities to other idiopathic “focal” epilepsies and its differences to symptomatic focal epilepsies that may also manifest with autonomic ictal symptoms and signs. This methodology allows the formation of a rational hypothesis on the pathophysiology of PS that seems to be emerging as a good model for the so-called “system” (nonsymptomatic) epilepsies, with potentially important taxonomic implications.

  • autonomic status epilepticus in Panayiotopoulos Syndrome and other childhood and adult epilepsies a consensus view
    Epilepsia, 2007
    Co-Authors: Colin D Ferrie, Athanasios Covanis, Veysi Demirbilek, Aysin Dervent, Osamu Kanazawa, Roberto Horacio Caraballo, Natalio Fejerman, Lucia Fusco, R A Grunewald, Michael Koutroumanidis
    Abstract:

    Summary: Purpose: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management. Methods: An international consortium of established researchers in the field was identified from their published work, agreed the purpose of the project, searched the literature, and, by use of e-mail communication, agreed the consensus document. Results: Autonomic SE is a condition lasting at least 30 min and characterized by epileptic activity causing altered autonomic function of any type at seizure onset or in which manifestations consistent with altered autonomic function are prominent (quantitatively dominant or clinically important) even if not present at seizure onset. It is best described, and probably most commonly encountered in children, with Panayiotopoulos Syndrome. However, it also occurs in children with symptomatic epilepsies and, exceptionally, in adults. Its pathogenesis and most appropriate management are poorly understood Conclusions: It is hoped that this document will help clinical recognition of Autonomic SE, reduce misdiagnosis, and promote further interest and studies into what has been a relatively neglected area. Key Words: Epileptic seizures— Epilepsy—Status epilepticus—Autonomic nervous system— Consensus statement.

  • the contribution of the eeg technologists in the diagnosis of Panayiotopoulos Syndrome susceptibility to early onset benign childhood autonomic seizures
    Seizure-european Journal of Epilepsy, 2004
    Co-Authors: Sue Sanders, Colin D Ferrie, Shaun Rowlinson, Ioannis Manidakis, Michael Koutroumanidis
    Abstract:

    Abstract Purpose : To assess the contribution of the EEG technologists in the diagnosis of children with epileptic seizures. Methods : We analysed the clinical information obtained by the EEG technologists from children with epileptic seizures and their parents, and assessed its value for the generation of a clinically useful EEG report and a plausible electroclinical diagnosis. Interviews were based on a qualitative questionnaire, and were videotaped. We focused on Panayiotopoulos Syndrome (PS) because it has a high rate of misdiagnosis, usually for encephalitis or other severe cerebral insults. Results : Between 1998 and 2001, 424 EEG were performed in 308 children aged 1–14 years, of whom 228 (74%) had one or more epileptic seizures. We diagnosed PS in 14 children (6.1%), mainly based on clinical information. Three other had symptomatic ictal vomiting. In 9 of the 14 children with PS, diagnosis was achieved by the information collected by the EEG technologist. Five of these children were being treated for encephalitis, and management was altered accordingly. In a further three children the diagnosis of PS was confirmed. Conclusion : These findings demonstrate that the contribution of the EEG technologists to the diagnosis of people with epilepsies can expand well beyond their established role of recording and describing an EEG. We propose that technologists should be actively involved in prospective electroclinical studies if carefully designed protocols are used.

  • Panayiotopoulos Syndrome a common benign but underdiagnosed and unexplored early childhood seizure Syndrome
    BMJ, 2002
    Co-Authors: Michael Koutroumanidis
    Abstract:

    Epilepsy affects 1% of the general population and 4% of children, encompassing heterogeneous seizure Syndromes.1 These are defined by distinct aetiology, age at onset, seizure type, and electroencephalographic features, which taken together provide the key to diagnosis, prognosis, and optimal management. Over the past two decades various distinct paediatric epilepsy Syndromes, such as rolandic epilepsy, have been formally recognised.2 Panayiotopoulos Syndrome is a new idiopathic childhood epilepsy, recently recognised by the International League Against Epilepsy.2 3 It is common, benign, and may mimic other common illnesses. Awareness of this Syndrome is important for all professionals who care for children with epileptic seizures, including general practitioners and community nurses, paediatricians and paediatric neurologists and clinical neurophysiologists, for the following reasons. Firstly, it is common. It probably affects about 13% of children of 3-6 years old with one or more non-febrile seizures (peak age 4-5 years), and 6% of the age group 1-15.4 5 Secondly, seizures can be prolonged, may mimic non-epileptic disorders, and may vary in severity from trivial to apparently life threatening—implying that the diagnosis may need to be considered in a variety of clinical settings and by medical professionals of different specialties. Thirdly, it is benign—its recognition therefore can provide firm reassurance to families in situations that …

Makiko Osawa - One of the best experts on this subject based on the ideXlab platform.

  • neurobehavioral abnormalities may correlate with increased seizure burden in children with Panayiotopoulos Syndrome
    Pediatric Neurology, 2009
    Co-Authors: Yoshiko Hirano, Hirokazu Oguni, Makoto Funatsuka, Kaoru Imai, Makiko Osawa
    Abstract:

    Prognostic factors for frequent seizure recurrences were studied in patients with Panayiotopoulos Syndrome. The subjects were 79 children fulfilling the criteria of Panayiotopoulos Syndrome who were monitored for longer than 2 years. Medical records and electroencephalograms were analyzed retrospectively. The total number of seizures in each patient at the final follow-up ranged from 1 to 22. The 79 patients were classified into three groups: typical Panayiotopoulos Syndrome (seizure recurrence = 1-5 times, n = 45), borderline (6-9 times, n = 16), and atypical (>10 times, n = 18). Data analyzed included family history of seizure disorders, peri- and postnatal complications, previous seizure histories, age at epilepsy onset, clinical seizure manifestations, the frequency of status epilepticus, interictal electroencephalographic patterns, and the possible association of neurobehavioral disorders among the three groups. An association with pre-existing neurobehavioral disorders was significantly more frequent in the atypical than in the typical group (P 0.05). In patients with Panayiotopoulos Syndrome and pre-existing mild neurobehavioral disorders, seizures tend to be pharmacoresistant and to repeat more than 10 times. However, all patients experience seizure remission by 12 years of age, and should not be evaluated for surgery.

  • Early-Onset Form of Benign Childhood Epilepsy with Centro-Temporal EEG Foci - A Different Nosological Perspective from Panayiotopoulos Syndrome
    Neuropediatrics, 2008
    Co-Authors: Mayu Ohtsu, Hirokazu Oguni, Makoto Funatsuka, Katsumi Imai, Makiko Osawa
    Abstract:

    Purpose: We have studied the clinical differences between early-onset benign epilepsy with centro-temporal spikes (early-onset BECT) and Panayiotopoulos Syndrome (PS) to investigate the hypothesis that BECT and PS nosologically constitute age-dependent benign childhood seizure susceptibility Syndromes based on a common etiopathogenesis. Subjects and Methods: The subjects were 24 patients with BECT and 62 patients with PS, who satisfied the following definitions: 1) onset of epilepsy before 5 years of age; 2) the BECT and PS seizures started mainly with orofacial focal motor attacks and emetic symptoms followed by focal seizures, respectively; 3) follow-up examinations for longer than 2 years. We compared the various clinical features between these two groups. Results: In children with early-onset BECT, the seizures at times manifested with hypersalivation, vomiting, and focal motor seizures, but the vomiting that developed in the middle of seizures was different from the initial vomiting observed in patients with PS. Although the seizures recurred more frequently in patients with early-onset BECT, the incidence of status epilepticus as well as prolonged seizures was higher in those with PS. The patients demonstrating below borderline IQ scores and mild developmental behavioral disorders were more frequently seen in early-onset BECT than PS, accounting for 37.5 and 14.6% (P

  • early onset form of benign childhood epilepsy with centro temporal eeg foci a different nosological perspective from Panayiotopoulos Syndrome
    Neuropediatrics, 2008
    Co-Authors: Mayu Ohtsu, Hirokazu Oguni, Makoto Funatsuka, Katsumi Imai, Makiko Osawa
    Abstract:

    Purpose: We have studied the clinical differences between early-onset benign epilepsy with centro-temporal spikes (early-onset BECT) and Panayiotopoulos Syndrome (PS) to investigate the hypothesis that BECT and PS nosologically constitute age-dependent benign childhood seizure susceptibility Syndromes based on a common etiopathogenesis. Subjects and Methods: The subjects were 24 patients with BECT and 62 patients with PS, who satisfied the following definitions: 1) onset of epilepsy before 5 years of age; 2) the BECT and PS seizures started mainly with orofacial focal motor attacks and emetic symptoms followed by focal seizures, respectively; 3) follow-up examinations for longer than 2 years. We compared the various clinical features between these two groups. Results: In children with early-onset BECT, the seizures at times manifested with hypersalivation, vomiting, and focal motor seizures, but the vomiting that developed in the middle of seizures was different from the initial vomiting observed in patients with PS. Although the seizures recurred more frequently in patients with early-onset BECT, the incidence of status epilepticus as well as prolonged seizures was higher in those with PS. The patients demonstrating below borderline IQ scores and mild developmental behavioral disorders were more frequently seen in early-onset BECT than PS, accounting for 37.5 and 14.6% (P<0.05), and for 8% and 21%, respectively (P<0.05). Discussion: Early-onset BECT and PS have heterogeneous clinical characteristics, except for the same onset age, and appear to be nosologically different epileptic Syndromes. The former seems to develop in combination with other acquired disturbances based on a BECT predisposition, while the latter develops based on a PS predisposition and involves a better prognosis.

  • EEG in Children with Early‐onset Benign Occipital Seizure Susceptibility Syndrome: Panayiotopoulos Syndrome
    Epilepsia, 2003
    Co-Authors: Mayu Ohtsu, Hirokazu Oguni, Makoto Funatsuka, Kaoru Imai, Kitami Hayashi, Makiko Osawa
    Abstract:

    Summary:  Purpose: We analyzed sequential changes in the localization of EEG foci along with age to identify a specific EEG pattern, and the relation between the clinical manifestations and the EEG pattern in patients with Panayiotopoulos Syndrome (PS). Methods: The subjects were 76 children, who had been followed up >2 years with repeated EEG examinations at 6-month intervals. Analysis of EEG findings included the determination of localization of spike foci, as a function of age, by using cross-sectional data, and the identification of subgroups with homogeneous EEG patterns. Then we compared certain clinical features among these subgroups. Results: In the cross-sectional EEG study, the occipital EEG spike focus was most frequently seen between ages 2 and 5 years. Independent and synchronous frontopolar and occipital spikes (Fp-O spikes) and centroparietotemporal (CPT) EEG spike foci had increased incidences between ages 4 and 7 years, and between ages 6 and 10 years, respectively. We subclassified the 76 patients into the following five subgroups based on the evolutional changes in epileptic EEG foci, which frequently showed shifting, multiplications, and generalization: (a) persistent occipital focus group (O group), (b) Fp-O spikes group (Fp-O pattern group), (c) generalized EEG pattern group, (d) CPT foci group (CPT group), and (e) no epileptic EEG focus group. The Fp-O group showed the latest age at onset of epilepsy. The generalized EEG pattern group had the highest frequency of seizures as well as recurrences of status epilepticus (SE), as well as the longest active seizure period among the five groups. Conclusions: These results indicated that the EEG foci in most of patients with PS are frequently shifting location, multiplying, and propagating diffusely with age, rather than persistently localizing in the occipital region. In addition, the EEG patterns showed a certain trend and roughly corresponded to certain clinical characteristics. However, the prognosis of the seizures appeared to be favorable regardless of the EEG pattern.

  • eeg in children with early onset benign occipital seizure susceptibility Syndrome Panayiotopoulos Syndrome
    Epilepsia, 2003
    Co-Authors: Mayu Ohtsu, Hirokazu Oguni, Makoto Funatsuka, Kaoru Imai, Kitami Hayashi, Makiko Osawa
    Abstract:

    Summary:  Purpose: We analyzed sequential changes in the localization of EEG foci along with age to identify a specific EEG pattern, and the relation between the clinical manifestations and the EEG pattern in patients with Panayiotopoulos Syndrome (PS). Methods: The subjects were 76 children, who had been followed up >2 years with repeated EEG examinations at 6-month intervals. Analysis of EEG findings included the determination of localization of spike foci, as a function of age, by using cross-sectional data, and the identification of subgroups with homogeneous EEG patterns. Then we compared certain clinical features among these subgroups. Results: In the cross-sectional EEG study, the occipital EEG spike focus was most frequently seen between ages 2 and 5 years. Independent and synchronous frontopolar and occipital spikes (Fp-O spikes) and centroparietotemporal (CPT) EEG spike foci had increased incidences between ages 4 and 7 years, and between ages 6 and 10 years, respectively. We subclassified the 76 patients into the following five subgroups based on the evolutional changes in epileptic EEG foci, which frequently showed shifting, multiplications, and generalization: (a) persistent occipital focus group (O group), (b) Fp-O spikes group (Fp-O pattern group), (c) generalized EEG pattern group, (d) CPT foci group (CPT group), and (e) no epileptic EEG focus group. The Fp-O group showed the latest age at onset of epilepsy. The generalized EEG pattern group had the highest frequency of seizures as well as recurrences of status epilepticus (SE), as well as the longest active seizure period among the five groups. Conclusions: These results indicated that the EEG foci in most of patients with PS are frequently shifting location, multiplying, and propagating diffusely with age, rather than persistently localizing in the occipital region. In addition, the EEG patterns showed a certain trend and roughly corresponded to certain clinical characteristics. However, the prognosis of the seizures appeared to be favorable regardless of the EEG pattern.