10·5 million children worldwide are estimated to have active epilepsy. Over the past 15 years, syndrome-oriented clinical and EEG diagnosis, and better aetiological diagnosis, especially supported by neuroimaging, has helped to clarify the diversity of epilepsy in children, and has improved management. Perinatal and postinfective encephalopathy, cortical dysplasia, and hippocampal sclerosis account for the most severe symptomatic epilepsies. Ion channel defects can underlie both benign age-related disorders and severe epileptic encephalopathies with a progressive disturbance in cerebral function. However, the reasons for age-related expression in children are not understood. Neither are the mechanisms whereby an epileptic encephalopathy originates. Several new drugs have been recently introduced but have provided limited therapeutic benefits. However, treatment and quality of life have improved because the syndrome-specific efficacy profile of drugs is better known, and there is heightened awareness that compounds with severe cognitive side-effects and heavy polytherapies should be avoided. Epilepsy surgery is an important option for a few well-selected individuals, but should be considered with great caution when there is no apparent underlying brain lesion.

https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(06)68182-8.pdf

Epilepsy in Children

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.

/pdf/benign-childhood-focal-epilepsies-assessment-of-established-3xu3lw0op0.pdf

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

Summary: Purpose: To emphasize that, in some patients, different atypical evolutions occur in the course of so-called benign focal epilepsies of childhood (BFEC) and to promote interest in finding clinical and/or electroencephalographic (EEG) clues to which patients might be prone to these risky evolutions.

Methods: Twenty-six patients who started with the typical clinical and EEG features of benign childhood epilepsy with centrotemporal spikes (BCECTS) but who had reversible or persistent, serious epileptic events including status epilepticus and language, cognitive, or behavioral impairments were followed for <14 years. Repeated neurologic examinations, EEG records, and neuropsychological evaluations were done, and brain-imaging studies [computerized axial tomography (CAT) or magnetic resonance imaging (MRI)] were obtained in all patients.

Results: The 26 patients were in four separate groups according to the nature of their atypical evolution. Eleven children had atypical benign focal epilepsy of childhood (ABFEC), three with Landau–Kleffner syndrome (LKS), seven with status epilepticus of BCECTS, and five with mixed features of the other three groups. All the children whose BCECTS evolved into ABFEC have finally recovered and are attending normal schools, although five have learning difficulties. Two of the three patients diagnosed with LKS recovered from aphasia, although some language difficulties persist in one. The seven who showed status epilepticus of BCECTS are now normal after 3–14 years of follow-up, and three of the five children showing mixed features fulfilled the criteria for a diagnosis of epilepsy with continuous spikes and waves during slow sleep (CSWS).

Conclusions: A small proportion of cases starting with BCECTS evolve into ABFEC, LKS, status of BCECTS, or epilepsy with CSWS. In such cases, BCECTS is not always benign. Clinical and EEG markers should be sought to predict these atypical evolutions.

Atypical evolutions of benign localization-related epilepsies in children: are they predictable?

In April 2004, a group of physicians with an interest in nonconvulsive status epilepticus representing a spectrum of opinion met in Oxford, sponsored by the Epilepsy Research Foundation (a charitable organization), to discuss and debate the definition, diagnosis and treatment of nonconvulsive status epilepticus. We felt that such a meeting would be useful, as nonconvulsive status epilepticus is a subject that provokes strong reactions, perhaps largely due to the relative lack of evidence and the surfeit of opinion. The meeting was arranged such that there were formal talks followed by a discussion led by one of the attendees. We present here the extended abstracts of the main talks with the points raised by the discussants. 

Despite disagreements on certain issues there was much in the way of consensus. First, it was agreed that nonconvulsive status epilepticus is a term that covers a range of disparate conditions with varying prognoses and treatments. The agreed definition was thus suitably vague, >. Secondly, it was agreed that even within a specific condition (e.g. complex partial status epilepticus), the prognosis and treatment depends upon the context in which the condition occurs (e.g. in the critically ill, in coma, in the > and in people with prior epilepsy). Perhaps, most importantly it was agreed that we lacked good clinical data, and the challenge was to design good studies for a condition that is underrecognised and often difficult to diagnose.

http://www.jle.com/fr/revues/epd/e-docs/nonconvulsive_status_epilepticus_epilepsy_research_foundation_workshop_reports__266516/article.phtml?tab=download&pj_key=doc_attach_2170

Nonconvulsive status epilepticus: Epilepsy Research Foundation Workshop Reports

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 benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis.

Purpose: Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. Methods: This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting. Results: One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3): 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was I year. Conclusions: Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.

Early-onset benign occipital seizure susceptibility syndrome.

Resume Les auteurs ont etudie l'electroencephalogramme (EEG) de deux patients dans la phase de debut du syndrome de Rasmussen. Ils se sont particulierement interesses a la phase prodromique lorsque les donnees cliniques ne suggerent pas l'existence d'une encephalite chronique a l'origine de l'epilepsie. A ce stade, l'EEG met en evidence des anomalies lentes polymorphes focales inhabituelles en periode post-critique, associees a des complexes lents biphasiques (CLB). Le CLB, constitue de deux ondes lentes de polarite opposee, d'amplitude pic a pic comprise entre 150 et 250 μV et d'une duree d'environ 500 ms n'a ete decrit jusqu'a present que dans des syndromes inflammatoires du systeme nerveux central. Sa presence les premiers mois du syndrome de Rasmussen est discutee.

A. Beaumanoir

Papers

Early-onset benign occipital seizure susceptibility syndrome.

Anomalies eeg dans la phase prémonitoire du syndrome de rasmussen. À propos de deux observations