Autonomic Status Epilepticus Research Articles

Status epilepticus in patients with genetic generalized epilepsy: a case series study

BackgroundGenetic generalized epilepsy (GGE) accounts for nearly one-third of all epilepsies. The feature of status epilepticus (SE) in patients with GGE has been rarely studied. We aimed to determine the electroclinical characteristics of SE in patients with GGE.MethodsIn this retrospective study, nine patients with GGE were enrolled at Xijing Hospital, Xi’an, China from May 2014 to May 2020. SE was confirmed by 24-h video-EEG recording. The demography, clinical manifestation, brain MRI and SE pattern were analyzed.ResultsOf the nine patients in the study, seven were female. The mean age of the patients at the time of inclusion was 16.8 years (range 7–31 years), and the mean age at the onset of epilepsy was 10.9 years (range 6–17 years). The follow-up time ranged from 3 months to 6 years. Myoclonic absence status was identified in four patients showing eyelid myoclonia with absence and one patient showing perioral myoclonia with absences. Myoclonic SE was identified in three patients showing juvenile myoclonic epilepsy. Autonomic SE was found in one patient with eyelid myoclonia with absence. SE was terminated by oral midazolam in four patients. In the other five patients, SE terminated spontaneously.ConclusionsThe seizure type of SE in patients with GGE is often consistent with their major symptoms. Oral midazolam may be an option to terminate SE in patients with GGE.

Faecal Incontinence: An Uncommon Manifestation of Panayiotopoulos Syndrome

Panayiotopoulos Syndrome (PS) is a benign, common, age-dependent syndrome, with susceptibility to idiopathic vegetative seizures and autonomic status epilepticus. It is formally recognized by the International League against Epilepsy (ILAE). It is one of the three focal epileptic child benign syndromes next to rolandic epilepsy and idiopathic occipital epilepsy in children. It is characterized by its relative mildness and confusing manifestations. It is frequently misdiagnosed as non-epileptic disorders.

Panayiotopoulos syndrome: a clinico-electroencephalographic presentation of three cases from Nigeria

Panayiotopoulos syndrome (PS) is a common benign, childhood, focal, seizure susceptibility syndrome presenting with mainly focal aware or focal impaired awareness autonomic seizures and autonomic status epilepticus that is under diagnosed in our locality. Increased awareness through reporting will aid diagnosis, reduce misdiagnosis and prevent aggressive and deleterious interventions. The classic clinical and electroencephalographic (EEG) features of three cases are described and the literature reviewed. The aim is to underscore the presence in our locality of this remarkably benign epileptic syndrome that must be differentiated from febrile seizures, encephalopathy, migraine or cardiogenic syncope.

Pupillary hippus as clinical manifestation of refractory autonomic nonconvulsive status epilepticus: Pathophysiological implications

Pupillary hippus as clinical manifestation of refractory autonomic nonconvulsive status epilepticus: Pathophysiological implications

Panayiotopoulos Syndrome with a special emphasis of ictal semiologic features

PurposeThe aim was to identify the demographic, electroencephalographic findings, clinical features, especially ictal semiological symptoms of patients diagnosed with Panayiotopoulos Syndrome. MethodThe semiologic and EEG findings of 42 patients were reviewed. Statistical analysis was used to determine the frequencies of semiologic features and to define the symptom clusters. ResultsThe most common clinical features were nausea (54.8%), vomiting (50.0%), pallor (50.0%), deviation of head and eyes (45.2%), generalized seizures (35.7%), ictal syncope (33.3%) and stomach ache (26.2%). Visual manifestations which were observed in 38.9% of the patients were amaurosis, flashes of colourful lights, flashes of brightful lights, visual hallucinations and blurring of vision. Autonomic status epilepticus was observed in 21.4% of the patients. Cluster analysis of 13 most common symptoms showed a tendency toward 3 clusters. Pallor constituted a subgroup on its own whereas nausea, vomiting, deviation of head and eyes tended to occur together in the second subgroup and the third subgroup included semiologic features some of which were atypical for PS. ConclusionsA broad spectrum of typical and atypical clinical features might be observed in patients with PS. Some of the semiologic features of PS might have tendency to occur together. Overlapping of typical autonomic features with some of the atypical features might suggest that PS, RE and ICOE-G were the constituents of the same neurobiological spectrum. Our detailed data might contribute to the awareness of a wide range of variabilites in the electroclinical features of PS.

Treatment of Classic Syndromes in Idiopathic Focal Epilepsies in Childhood

Of the classic syndromes of idiopathic (benign) focal epilepsies in childhood, more than half of children with Rolandic epilepsy and Panayiotopoulos syndrome do not need antiepileptic drug (AED) treatment. Monotherapy with carbamazepine, levetiracetam, sodium valproate, or clobazam is usually recommended for those with seizures that are unusually frequent (more than three), lengthy, distressing, or otherwise significantly interfering with the child's life. Other factors in favor of treatment include a short interval between the first three seizures, a younger age of onset (less than 4 years), or a presence of recurrent tonic–clonic seizures and diurnal seizures. In contrast to Rolandic epilepsy and Panayiotopoulos syndrome that may not need treatment, idiopathic childhood occipital epilepsy of Gastaut should be treated because seizures are frequent and secondarily generalized convulsions are probably unavoidable without medication. It is likely that all AEDs licensed as monotherapy for focal seizures and mainly carbamazepine and levetiracetam are suitable.The electroencephalogram does not predict prognosis or AED treatment needs, though there are views supporting that interictal spikes have an adverse effect on cognition.Strategies of withdrawing medication in idiopathic focal epilepsies in childhood differ among experts, although all agree that there is no need to continue medication 1 to 3 years after the last seizure, and certainly not after the age of 14 years, when most benign childhood focal seizures remit, or age 16, when they are practically nonexistent.In the acute stage of status epilepticus, control of the seizures is of paramount importance. Convulsive status epilepticus is a life threatening condition and should be terminated as soon as possible. Autonomic status epilepticus needs thorough evaluation for proper diagnosis and management. Benzodiazepines, intravenously, in rectal or buccal preparations are commonly used to terminate long lasting seizures and status epilepticus. Early parental treatment is more effective than late emergency treatment. Buccal midazolam is probably the first choice medication for out-of-hospital termination of status epilepticus.Parental education and psychological support are the cornerstone of the optimal management because despite excellent prognosis, idiopathic focal epilepsies in childhood usually have a dramatic impact to parents.

Early Onset Benign Occipital Epilepsy (Panayiotopoulos syndrome): a case Report & Literature Review

Panayiotopoulos syndrome is a type of benign childhood epilepsy syndrome characterized by episodic autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent and most do not require anti epileptic medications. Remission usually occurs within 1-2 years from onset. One third have a single seizure but 5-10% may have more than 10 seizures or a more prolonged course. We present a 5 year old boy with this condition, who presented with episodes of severe vomiting, deviation of eyes, focal seizure, altered sensorium, characteristic EEG in the form of multiple occipital spikes and normal neuroimaging studies.J. Natl Inst. Neurosci Bangladesh 2015;1(1):27-30

Panayiotopoulos Syndrome Presenting With Status Epilepticus and Cardiorespiratory Arrest

Panayiotopoulos syndrome is early-onset benign childhood epilepsy, now classified as an electroclinical syndrome. The original description in 1989 focused on the triad of nocturnal seizures, tonic eye deviation, and vomiting. With available data from the long-term studies, a wide variety of manifestations have been described, with recognition of autonomic features as being the most prominent aspect of this epilepsy. The presenting symptoms are usually focal seizures comprising autonomic symptoms and/or behavioral changes. Majority of these seizures are in sleep, with half of the seizures progressing to become secondarily generalized. Occasionally, these seizures can present with prominent autonomic features such as ictal vomiting, pallor, flushing/cyanosis, and tachycardia with prolonged thermoregulatory changes lasting for hours, constituting autonomic status epilepticus. Recovery from this autonomic status epilepticus is within hours and is always complete. Autonomic status epilepticus has been previously reported in this epilepsy syndrome, but ictal cardiorespiratory arrest is extremely rare, with only 4 cases being reported in literature. All 4 cases reported in literature recovered spontaneously and did not require resuscitation. Here we present a 3½-year-old male child with Panayiotopoulos syndrome who presented with status epilepticus and ictal cardiorespiratory arrest requiring cardiopulmonary resuscitation for revival.

Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome

Panayiotopoulos syndrome (PS) is a common childhood susceptibility to autonomic seizures and status epilepticus. Despite its high prevalence, PS has been a source of significant debate. We present ictal EEG documentation of autonomic seizures and autonomic status epilepticus in six cases of PS and a review of 14 reported cases. Interictal EEGs showed spikes of variable locations that often changed with time. Ictal EEG onsets were also variable, starting from wide anterior or posterior regions usually with theta waves intermixed with small spikes and fast rhythms. Ictal vomiting and other autonomic manifestations, as well as deviation of the eyes, did not appear to relate to any specific region of EEG activation. These data document that PS is a multifocal autonomic epilepsy and support the view that the clinical manifestations are likely to be generated by variable and widely spread epileptogenic foci acting on a temporarily hyperexcitable central autonomic network.

Efficacy and safety of levetiracetam in the treatment of Panayiotopoulos syndrome

Panayiotopoulos syndrome (PS) represents the second commonest benign partial epilepsy of childhood. This study evaluated the effects of levetiracetam (LEV) in three children with this syndrome. All three children (aged 8, 12 and 10 years) had episodic autonomic symptoms for 4, 6 and 2 years, respectively. Symptoms duration varied between a few minutes and 5-7 days, reflecting an autonomic status epilepticus. Children previously controlled on valproate (VPA) but with recurring seizures, were given LEV (1000-2000 mg/day) initially as add-on therapy, and after as monotherapy. All three children received LEV monotherapy and remained seizure-free after 3, 3 and 2 years of treatment, respectively. One child, after 2 years seizure free, stopped LEV treatment. Now, he is asymptomatic for 2 years. LEV has shown efficacy on autonomic seizure control in three patients with PS where VPA was ineffective.

Introduction

Introduction

Misdiagnosis due to gastrointestinal symptoms in an adolescent with probable autonomic status epilepticus and Panayiotopoulos syndrome

Panayiotopoulos syndrome is a common benign epilepsy affecting otherwise healthy children that present with autonomic seizures, in which nausea, retching, and vomiting are particularly common and prominent. Because of the unusual ictal symptoms and lengthy manifestations, misdiagnosis is a common major problem. We describe a young girl with intractable and lengthy vomiting attacks, several admissions to hospitals, and extensive gastroenterological workup for several years from early childhood. On all previous occasions the diagnosis varied from psychosomatic disease, to functional dyspepsia, to cyclic vomiting syndrome. The possibility of autonomic epileptic seizures and Panayiotopoulos syndrome, though likely, was not considered.

Aphasic or amnesic status epilepticus detected on PET but not EEG

To describe five patients with ictal aphasia and one patient with ictal amnesia, who had focal positron emission tomography (PET) hypermetabolism but no clear ictal activity on electroencephalography (EEG). (18)F-Fluorodeoxyglucose (FDG)-PET scans with concomitant EEG were obtained in five patients with suspected ictal aphasia or ictal amnesia without ictal activity on EEG. We reviewed medical history, EEG, imaging data, and treatment outcome. Brain magnetic resonance imaging (MRI) showed no structural abnormalities in any of the patients. EEG showed left temporal irregular delta activity in three patients, with aphasia and nonspecific abnormalities in two other patients, all without clear ictal pattern. All patients demonstrated focal hypermetabolism on PET scan. The hypermetabolism was in the left frontotemporal region in patients with ictal aphasia and in the bilateral hippocampal region in the patient with amnesia. Three patients who received intravenous benzodiazepines during their episodes had transient clinical improvement. With antiepileptic drug (AED) treatment, symptoms gradually resolved in all patients. Concomitant resolution of PET hypermetabolism was documented in three patients who had follow up scans. One patient with ictal aphasia later developed recurrent episodes, each with recurrent PET hypermetabolism. This patient and one other patient required immune-modulating therapy in addition to AEDs. FDG-PET imaging should be considered as a diagnostic tool in patients with suspected ictal aphasia or amnesia, who fail to show clear evidence of ictal activity on EEG.

A Novel Inherited Mutation in the Voltage Sensor Region of SCN1A Is Associated With Panayiotopoulos Syndrome in Siblings and Generalized Epilepsy With Febrile Seizures Plus

We report 2 families harboring a novel SCN1A mutation, one of whom had Panayiotopoulos syndrome and the other a phenotype consistent with generalized epilepsy with febrile seizures plus. Two siblings had recurrent episodes of autonomic status epilepticus with focal features consistent with the diagnosis of Panayiotopoulos syndrome. Both have the SCN1A mutation p.Phe218Leu. The mutation was present in their father who has never had a seizure. The same mutation was identified in a child diagnosed with intractable childhood epilepsy with generalized tonic clonic seizures. From the age of 5, he developed complex focal seizures associated with left hippocampal sclerosis. The mutation was present in his mother, aged 25, who had febrile seizures and developed generalized tonic clonic seizures and his sister who had 1 febrile seizure. Our findings suggest that SCN1A mutations may cause susceptibility to an idiopathic focal epilepsy phenotype, the final phenotype depending on other (genetic or nongenetic) factors.

Benign childhood focal epilepsies: assessment of established and newly recognized syndromes

A big advance in epileptology has been the recognition of syndromes with distinct aetiology, clinical and EEG features, treatment and prognosis. A prime and common example of this is rolandic epilepsy that is well known by the general paediatricians for over 50 years, thus allowing a precise diagnosis that predicts an excellent prognosis. However, rolandic is not the only benign childhood epileptic syndrome. Converging evidence from multiple and independent clinical, EEG and magnetoencephalographic studies has documented Panayiotopoulos syndrome (PS) as a model of childhood autonomic epilepsy, which is also common and benign. Despite high prevalence, lengthy and dramatic features, PS as well as autonomic status epilepticus had eluded recognition because emetic and other ictal autonomic manifestations were dismissed as non-epileptic events of other diseases. Furthermore, PS because of frequent EEG occipital spikes has been erroneously considered as occipital epilepsy and thus confused with the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G), which is another age-related but rarer and of unpredictable prognosis syndrome. Encephalitis is a common misdiagnosis for PS and migraine with visual aura for ICOE-G. Pathophysiologically, the symptomatogenic zone appears to correspond to the epileptogenic zone in rolandic epilepsy (sensory-motor symptomatology of the rolandic cortex) and the ICOE-G (occipital lobe symptomatology), while the autonomic clinical manifestations of PS are likely to be generated by variable and widely spread epileptogenic foci acting upon a temporarily hyperexcitable central autonomic network. Rolandic epilepsy, PS, ICOE-G and other possible clinical phenotypes of benign childhood focal seizures are likely to be linked together by a genetically determined, functional derangement of the systemic brain maturation that is age related (benign childhood seizure susceptibility syndrome). This is usually mild but exceptionally it may diverge to serious epileptic disorders such as epileptic encephalopathy with continuous spike and wave during sleep. Links with other benign and age-related seizures in early life such as febrile seizures, benign focal neonatal and infantile seizures is possible. Overlap with idiopathic generalized epilepsies is limited and of uncertain genetic significance. Taking all these into account, benign childhood focal seizures and related epileptic syndromes would need proper multi-disciplinary re-assessment in an evidence-based manner.

The new ILAE Report on Classification and Evidence‐Based Commentary on Panayiotopoulos Syndrome and Autonomic Status Epilepticus

The new ILAE Report on Classification and Evidence‐Based Commentary on Panayiotopoulos Syndrome and Autonomic Status Epilepticus

Response to Koutroumanidis

To the Editors: We appreciate the comments from Dr. Koutroumanidis about our article on autonomic symptoms (AS's) during partial seizures in children. Dr. Koutroumanidis raises some important questions, and we will try to address them briefly below. Regarding the predominant (most important) ictal manifestations seen in our population of children – Typical psychomotor or motor seizures were predominant. Autonomic symptoms occurred frequently, but were not the predominant feature of these seizures. Regarding the timing (early or late in the seizure sequence) of AS's – Most of the AS's appeared later in the seizures. Some of them were typically postictal (or occurred at the end of the ictal episode) – e.g., nose-wiping, vomiting, coughing, hiccupping. Other signs occurred within the ictal period, but late in the session – e.g., genital automatisms, flushing, pallor, hypersalivation. Only a few AS's appeared at an early stage – e.g., epigastric aura and apnea. In short, the occurrence of AS's in these children followed the typical adult pattern of late-appearing AS's, as cited by Dr. Koutroumanidis. Regarding the duration of seizures with and without AS's – The duration of the AS's was usually short and constituted a minor part of the seizure. We did not see any seizures with autonomic status epilepticus, and none of the seizures were characterized exclusively by AS's. Rather, the AS period appeared as part of the whole psychomotor or motor seizure attack. We did not measure the exact length of seizures (an enormous undertaking for 514 attacks), and therefore cannot compare statistically the length of seizures with and without AS's. Regarding signs typical of Panayiotopoulos syndrome (PS) – Loss of muscle tone or awareness, a typical sign of PS, was noted in our semiological seizure classification of “atonic seizure.” Only 4 of 514 seizures (<1%) contained atonic components, so loss of muscle tone was clearly not a characteristic symptom of this patient group. We believe that the differences of our seizure with AS's, as compared to the longer autonomic seizures of PS, result from our special patient population. In our study, all the children had focal, well-localized, therapy-resistant epilepsy – a group very much different from the idiopathic childhood epilepsy population.

Autonomic Status Epilepticus in Panayiotopoulos Syndrome and Other Childhood and Adult Epilepsies: A Consensus View

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. 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. 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. 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.

Panayiotopoulos Syndrome: A Benign Childhood Autonomic Epilepsy Frequently Imitating Encephalitis, Syncope, Migraine, Sleep Disorder, or Gastroenteritis

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." The purpose of this review is to provide guidance for appropriate diagnosis and management of Panayiotopoulos syndrome. 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. 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. An electroencephalogram is the only investigation with abnormal results, usually showing multiple spikes in various brain locations. 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. 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. 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.

Autonomic seizures and autonomic status epilepticus in early onset benign childhood occipital epilepsy (Panayiotopoulos syndrome)

To study clinical and EEG features of children with ictal vomiting and no underlying brain lesions (Panayiotopoulos syndrome). The subjects were 36 children aged 2-13 years. The onset of seizures occurred between 1 and 5 years of age. Fourteen children (38.8%) had a single seizure. Fourteen children (38.8%) had autonomic status epilepticus. Impairment of consciousness was reported in 30 (83.3%) children, eye deviation in 10 (27.7%) other autonomic symptoms and head deviation in 9, generalization in 8, visual symptoms in one child, and, speech arrest or hemifacial motor symptoms in 8 cases. The EEG showed occipital spikes or spike-wave complexes in 27 (75.0%) children, blocked by opening of the eyes in 8 (22.2%) cases. Nine patients (25%) also had rolandic spikes and 3 had extraoccipital spikes. Six (16.6%) patients had normal EEG. No clinical differences were observed between patients having occipital or extraoccipital spikes. In children only with autonomic seizures, the spikes are predominantly occipital but blockage by opening of the eyes is a less frequent feature. In some children there is an overlapping of different focal childhood idiopathic syndromes.