Benign Partial Epilepsy Of Childhood Research Articles

Pharmacoresistant Atypical Benign Partial Epilepsy of Childhood: Early Introduction of Specific Therapies to Prevent Cognitive Decline

AbstractAtypical benign partial epilepsy (ABPE) of childhood is a special type of epileptic syndrome characterized by the combination of epileptic negative myoclonus, atypical absence seizures, focal motor seizures, and a continuous spike–wave during slow sleep (CSWS). Although the seizures are resistant to antiseizure drugs (ASDs) effective for focal epilepsy, they are markedly responsive to ethosuximide (ESM). An incorrect ASD choice may aggravate the seizures and CSWS, resulting in a pseudo-catastrophic state or nonconvulsive status epilepticus. In this study, we retrospectively investigated the effectiveness of steroid and ketogenic diet (KD) therapies for children with ABPE resistant to ASDs, including ESM. Adrenocorticotropic hormone (ACTH) therapy (n = 4), KD therapy (n = 4), and an oral steroid (n = 2) were tried for eight patients (two patients tried two therapies). An excellent response (seizure-free period > 1 year) was achieved by four out of four patients undergoing ACTH therapy, two out of four undergoing KD therapy, and one out of two receiving oral steroid therapy. In conclusion, steroid and KD therapies are reasonable treatment options early in the course of treatment of children with pharmacoresistant ABPE to prevent a decline in cognitive function due to the persistence of epileptic encephalopathy. The majority of patients in our case series had an excellent response, though further studies are warranted to confirm their efficacy and safety.

Atypical Benign Partial Epilepsy of Childhood Treated with Prednisolone and Ethosuximide

Atypical Benign Partial Epilepsy of Childhood Treated with Prednisolone and Ethosuximide

PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS

Pseudo-Lennox syndrome (PLS), or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized seizures, cognitive impairments, as well as regional and diffuse epileptiform activity on electroencephalogram (EEG) by the morphology identical to that of benign epileptiform patterns of childhood. The disease was first described by J. Aicardi and J. J. Chevrie in 1982, based on a study of 7 cases. Its diagnostic complexity is the polymorphism of both epileptic seizures and EEG data, as well as low awareness of the syndrome among physicians and its absence in the international classification of epilepsies. The typical triad of seizures, which occurs in nearly 100 % of patients, encompasses focal motor paroxysms (identical to those as observed in Rolandic epilepsy), atypical absences, and atonic seizures. Seizures in PLS in its active period (generally up to 7–8 years) are highly resistant to antiepileptic drugs. Only a few agents have been proven to be effective in PLS; these include valproates, succinimides, benzodiazepines, topiramate, and sulthiame. The frequency of seizures are noted to increase in patients with PLS treated with drugs, such as vigabatrin, gabapentin, lamotrigine, phenobarbital, or phenytoin. The author considers in detail the history of studies of the disease, clinical manifestations, diagnostic criteria, therapeutic approaches, and prognosis.

Atypical benign partial epilepsy of childhood with acquired neurocognitive, lexical semantic, and autistic spectrum disorder.

Atypical benign partial epilepsy (ABPE) of childhood or pseudo-Lennox syndrome is a form of idiopathic focal epilepsy characterized by multiple seizure types, focal and/or generalized epileptiform discharges, continuous spike–wave during sleep (CSWS), and sometimes reversible neurocognitive deficits. There are few reported cases of ABPE describing detailed correlative longitudinal follow-up of the various associated neurocognitive, language, social communicative, or motor deficits, in parallel with the epilepsy. Furthermore, the molecular inheritance pattern for ABPE and the wider spectrum of epilepsy aphasia disorders have yet to be fully elucidated. We describe the phenotype–genotype study of a boy with ABPE with follow-up from ages 5 to 13 years showing acquired oromotor and, later, a specific lexical semantic and pervasive developmental disorder. Exome sequencing identified variants in SCN9A, CPA6, and SCNM1. A direct role of the epilepsy in the pathogenesis of the oromotor and neurocognitive deficits is apparent.

The genetic research progress in benign childhood epilepsy with centro-temporal spikes

Benign epilepsy with centro-temporal spikes(BECT)is the most common epilepsy syndrome in childhood.The seizures are manifested with facial jerking and twitching to guttural noises, salivating with inability to speak and twitched limbs.The episode is proved to be closely associated with sleep, especially the non-rapid eye movement period.Centrotemporal spikes are the hallmark of the syndrome of BECT.Although the pathogenesis of BECT is still unknown, it is generally acknowledged that hereditary features play a critical role in the occurrence of BECT.In this article we are going to summarize the progresses of BECT in the fields of clinical genetics, cytogenetics and molecular genetics. Key words: Benign partial epilepsy of childhood with centro-temporal spikes; Genetics; Epilepsy

Treatment of pediatric epilepsy in Poland

BackgroundThe many types of childhood epilepsies make the diagnosis and treatment difficult and the outcomes frequently poor. Furthermore, there are few clinical trials in pediatric epilepsy that provide useful results to guide daily practice. Therefore for pediatric neurologists expert opinion may be useful. AimsTo provide an overview of current practice in Poland and compare results with European and US clinical guidelines. MethodsPolish specialists in pediatric neurology were asked to participate in a survey about pediatric epilepsy. The focus of the questions was on the overall strategy and treatment options for different syndromic diagnoses. The survey was developed and performed according to a previous European survey (Wheless et al., 2007). ResultsFifty-one Polish specialists, working in academic or clinical settings, completed the questionnaire. They limited combination therapy to two or three antiepileptic drugs. Valproate was the treatment of choice for myoclonic, generalized tonic-clonic seizures and Lennox-Gastaut syndrome. For infantile spasms caused by tuberous sclerosis and of symptomatic etiology, vigabatrin was treatment of choice; valproate and ACTH were other first line options. Valproate and ethosuximide were chosen for childhood absence epilepsy and valproate for juvenile absence epilepsy. Carbamazepine was the first-line treatment option for benign partial epilepsy of childhood with centrotemporal spikes and complex partial seizures. In the treatment of juvenile myoclonic epilepsy for males valproate, for females lamotrigine were chosen. ConclusionPolish pediatric neurologists agreed on the majority of questions. Their views reflect the clinical utility and availability of treatment options in Poland. Results may provide direction for clinicians.

Clinical characteristics and outcome of children with electrical status epilepticus during slow wave sleep.

Background:Electrical status epilepticus in slow wave sleep (ESES) is a rare, age-related, self-limited disorder characterized as epilepsy with different seizure types, neuropsychological impairment in the form of global or selective regression of cognitive functions, motor impairment, and typical electroencephalographic (EEG) findings of continuous epileptic activity occupying 85% of nonrapid eye movement sleep.Aims:The aim is to examine the clinical and electrophysiological findings and treatment modalities of children with ESES and to evaluate the outcome of the disorder.Materials and Methods:Fourteen patients with a diagnosis of electrical status epilepticus during slow wave sleep and followed-up at least 2 years were included.Statistical Analysis:Pearson correlation test was used in the study.Results:Among the 14 patients, eight of them had normal mental development before ESES. Twelve of the patients mentioned cognitive impairment and decline in school performance during ESES. After ESES, seven patients had mental retardation in different severity. One of these patients was diagnosed with benign partial epilepsy of childhood with centrotemporal spikes and had normal intelligence quotient level prior to ESES. The diagnosis of ESES was made after newly occurred different seizure types in four of the patients while two of the previously known epileptic patients presented with only severe psychiatric impairment. Valproic acid and carbamazepine were the mostly frequently used drugs before the onset of ESES. After at least 2 years of follow-up, seven patients were seizure free, but still taking antiepileptic treatment. Five patients were seizure free, while two of them had ongoing seizures despite antiepileptic therapy.Conclusion:ESES should be kept in mind in children with unexplained regression or stagnation of development associated with seizures or not. Sleep EEGs should be performed for timely diagnosis, proper treatment and prevention of permanent cognitive impairment.

The penelope syndrome not always means bad prognosis

Introduction Penelope spun during the day and undid it during the night so, which the brain makes during the day, is erased during the night by the epileptic activity. The epileptic syndromes with continuous spike-wave during slow sleep (CSWS) are a group of authentic epileptic encephalopathies and show a wide clinical spectrum with common characteristics that includes the increase and generalization of epileptifrom discharges in the EEG during sleep. In addition, patients have neuropsychological disturbances, including aphasia. The onset of these syndromes is around 4 or 5 years of age and stabilize or even improve around puberty. It is a rare syndrome that occurs exclusively in childhood, with a variable etiology. Prognosis is influenced by the age of onset and the persistence over the time of the CSWSS pattern, being worse in cases in which this abnormality appears at early ages and longer permanence. Materials and methods We report two cases of children with CSWS pattern but with different clinical presentations. Results The first one is a 6 years aged girl with a diagnosis of benign epilepsy of childhood, who changes her seizures pattern and starts to have delay in the school, showing a CSWS in the sleep EEG. She was diagnosed of atypical benign partial epilepsy of childhood, treated, with a good outcome (disappearance both seizures and CSWS pattern). This case would be the benign side of the CSWS encephalopathies spectrum. The second one is a case of a sudden acquired aphasia when the patient was 6 years old, who never suffered epileptic seizures, but shows a CSWS pattern in the EEG. He was diagnosed of Landau–Kleffner syndrome, trated with antiepileptics drugs and corticosteroids, with a benign clinical course (language recovering and EEG normalization). However this case would be at the more severe end of the spectrum. Conclusion The status epilepticus during slow sleep can cause serious neurocognitive development alterations of children affected. Early age of onset and persistence of CSWS pattern significantly influence the prognosis of these patients. Our cases would confirm this assumption, because the delayed onset and benign course, despite one of them was a case of Landau–Kleffner syndrome. In a child with sudden decrease in school performance or language difficulties would be entirely necessary to carry out a study of sleep EEG. Acknowledgements Dres. Paloma Quintana, Julia Saez and Felix Paradinas.

All children who experience epileptic falls do not necessarily have Lennox‐Gastaut syndrome… but many do

ABSTRACTLennox‐Gastaut syndrome (LGS) is a severe, chronic, epileptic encephalopathy, primarily with childhood onset, which is characterised by a triad of features: multiple seizure types, including tonic seizures that may appear late in the course of the disorder; abnormal EEG features with slow spike‐wave discharges; and cognitive impairment. Recognition of LGS is problematic, since the seizure types and EEG features that characterise it are not pathognomonic and often change over time. Furthermore, although seizures associated with LGS may occur de novo, the appearance of core LGS seizures may be preceded by prolonged periods of other seizure types, including myoclonic seizures, partial seizures, or infantile spasms. This has led some authors to postulate that a continuum between LGS and other types of childhood epilepsy may exist. Accurate diagnosis requires careful assessment of both clinical and EEG features, in order to distinguish LGS from other childhood epilepsy syndromes, such as atypical benign partial epilepsy of childhood, Dravet syndrome or epilepsies with predominantly myoclonic‐astatic seizures. Since there is no biological marker that can be used to confirm diagnosis of LGS and because of the multiple aetiologies that could lead to its development, early referral to a specialist team may prove to be crucial for facilitating both diagnosis and management. Such an approach ensures that patients receive the appropriate treatment at the correct time, providing the best opportunity for the clinical course and overall prognosis to be improved. Effective management of LGS requires regular reappraisal of the evolving symptoms and features, and adjustment of the treatment accordingly.

All children who experience epileptic falls do not necessarily have Lennox-Gastaut syndrome... but many do

Lennox-Gastaut syndrome (LGS) is a severe, chronic, epileptic encephalopathy, primarily with childhood onset, which is characterised by a triad of features: multiple seizure types, including tonic seizures that may appear late in the course of the disorder; abnormal EEG features with slow spike-wave discharges; and cognitive impairment. Recognition of LGS is problematic, since the seizure types and EEG features that characterise it are not pathognomonic and often change over time. Furthermore, although seizures associated with LGS may occur de novo, the appearance of core LGS seizures may be preceded by prolonged periods of other seizure types, including myoclonic seizures, partial seizures, or infantile spasms. This has led some authors to postulate that a continuum between LGS and other types of childhood epilepsy may exist. Accurate diagnosis requires careful assessment of both clinical and EEG features, in order to distinguish LGS from other childhood epilepsy syndromes, such as atypical benign partial epilepsy of childhood, Dravet syndrome or epilepsies with predominantly myoclonic-astatic seizures. Since there is no biological marker that can be used to confirm diagnosis of LGS and because of the multiple aetiologies that could lead to its development, early referral to a specialist team may prove to be crucial for facilitating both diagnosis and management. Such an approach ensures that patients receive the appropriate treatment at the correct time, providing the best opportunity for the clinical course and overall prognosis to be improved. Effective management of LGS requires regular reappraisal of the evolving symptoms and features, and adjustment of the treatment accordingly.

Atypical Benign Partial Epilepsy: Recognition Can Prevent Pseudocatastrophe

To characterize and distinguish atypical benign partial epilepsy of childhood among various epileptic syndromes, we conducted a clinical and electroencephalogram study. Seventeen children with atypical benign partial epilepsy of childhood were followed at our hospital. They all underwent a video/polygraphic study of characteristic daily seizures, facilitating a diagnosis of atypical benign partial epilepsy of childhood. Their clinical and electroencephalogram features were retrospectively analyzed. A video/polygraphic study indicated negative motor seizures including epileptic negative myoclonus, atonic absence seizures, or atonic seizures corresponding to spike-and-wave complexes arising from centro-parieto-temporal regions. Early in the clinical course, these seizures appeared every 4 ± 2 months, and lasted 1-3 months. Interictal sleep electroencephalograms, initially localizing in the centro-parieto-temporal regions, became widespread and displayed continuous, diffuse, spike-and-wave complexes, although the spike-wave index did not exceed 85%. Negative motor seizures responded to ethosuximide, corticotropin, and high-dose steroid, whereas other antiepileptic drugs were much less effective. All patients ultimately entered remission before age 12 years. Patients with atypical benign partial epilepsy of childhood exhibited a characteristic clinical course, and responded favorably to anti-absence treatment. Atypical benign partial epilepsy of childhood should be recognized as a discrete epileptic syndrome. Its early diagnosis leads to the prevention of pseudocatastrophe.

Partial seizures with affective semiology versus pavor nocturnus

Sudden nocturnal events with an affective semiology may have various etiologies, such as nocturnal panic attacks, nightmares, pavor nocturnus and/or different types of focal epileptic seizures, particularly in children. We describe the case of a normally developed boy who, at the age of two years, experienced nocturnal paroxysmal events that occurred about one hour after falling asleep and lasted up to 15 minutes. The clinical picture was characterized by sudden arousal, fear, inconsolable crying and an apparently voluntary search for his mother. The state of consciousness was basically maintained, and the inter-ictal EEG pattern showed independent bilateral centro-temporal spikes and spike-and-wave complexes. The child had a family history of benign partial epilepsy of childhood with rolandic spikes, and the paroxysmal events remitted after valproic acid treatment. This case report highlights the difficulties in the differential diagnosis of panic attacks, nightmares, pavor nocturnus and focal nocturnal epileptic seizures, especially those arising from the temporal and frontal lobes.

Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES)

To describe the clinical spectrum and to evaluate the efficacy of different therapeutic agents in children with electrical status epilepticus in sleep (ESES). Clinical data of all patients with ESES (not including patients with Landau-Kleffner syndrome) in four pediatric neurology outpatient clinics were analyzed. Thirty patients with ESES had been treated between 1994 and 2007. Eleven (37%) children had benign partial epilepsies of childhood, five (17%) had cerebral palsy, five (17%) had hydrocephalus, one (3%) had schizencephaly, one (3%) had prenatal parenchymal bleeding, and the etiology was unclear in seven (23%). The duration of ESES ranged between 2 and 60 months. The antiepileptic drugs that were found to be efficacious were: levetiracetam (41%), clobazam (31%), and sulthiame (17%). Valproic acid, lamotrigine, topiramate, and ethosuximide showed no efficacy. Steroids were efficacious in 65%; immunoglobulins were efficacious in 33%. High-dose diazepam was efficacious in 37%, but all the children had temporary response. Seventeen patients (57%) had cognitive deterioration, whereas the rest presented with regression in attention, speech, communication, and behavior. Fourteen children had permanent cognitive deficit. There was a significant correlation (p = 0.029) between the duration of ESES and residual intellectual deficit at follow-up. ESES reflects an evolution of benign partial epilepsy of childhood in more than one-third of the patients, whereas there is an underlying structural brain anomaly in another one-third. The most efficacious antiepileptic drugs (AEDs) are levetiracetam and clobazam. The duration of ESES correlated significantly with residual intellectual deficit at follow-up.

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.

A study on epileptic negative myoclonus in atypical benign partial epilepsy of childhood

Objective. To investigate the clinical and neurophysiological characteristics, particularly therapeutic considerations, of epileptic negative myoclonus (ENM) in atypical benign partial epilepsy (ABPE) of childhood. Methods. From 1998 to 2006, 14/242 patients with benign children epilepsy with centrotemporal spikes (BECTS) were diagnosed as having ABPE with ENM. In all 14 patients, we performed video-EEG monitoring along with tests with the patient’s arms outstretched; 6/14 patients were also simultaneously underwent surface electromyogram (EMG). ENM manifestations, electrophysiological features, and responses to antiepileptic drugs were analyzed. Results. In all cases, ENM developed after the onset of epilepsy and during antiepileptic drug therapy, and the appearance of ENM were corresponding to EEG findings of high-amplitude spikes followed by a slow wave in the contralateral motor areas with secondary generalization. This was further confirmed by time-locked silent EMG. During ENM occurrence or recurrence, habitual seizures and interictal discharges were exaggerated. In some patients, the changes in antiepileptic drug regimens in relation to ENM appearance included add-on therapy with carbamazepine, oxcarbazepine, and phenobarbital or withdrawal of valproate. ENM was controlled in most cases by administration of various combinations of valproate, clonazepam, and corticosteroids. Conclusion. The incidence of ENM or ABPE in our center was approximately 5.79%. A combination of video-EEG monitoring with the patient’s arms outstretched and EMG is essential to identify ENM. The aggravation of habitual seizures and interictal discharges indicate ENM. Some antiepileptic drugs, such as carbamazepine, oxcarbazepine, and phenobarbital, may be related to ENM occurrence during spontaneous aggravation of ABPE. Various combinations of valproate, benzodiazepines, and corticosteroids are relatively effective for treating ENM that occurs in ABPE.

Inverted Fixation-Off Sensitivity in Atypical Benign Partial Epilepsy

Fixation-off sensitivity is an electroencephalographic phenomenon characterized by spike-and-wave discharges that only occur when central vision and fixation are eliminated. It is especially seen in children with Panayiotopoulos-type, early-onset, benign childhood occipital epilepsy or Gastaut type, late-onset, childhood occipital epilepsy. It can also be seen in eyelid myoclonia with absences, in other idiopathic generalized epilepsies, and in asymptomatic children without epilepsy. We describe a boy with atypical, benign partial epilepsy of childhood who exhibited the reverse: epileptiform activity that was suppressed by the absence of central vision or fixation, and activated by central vision or fixation.

Successful treatment of atypical idiopathic focal epilepsy with CSWS with levetiracetam

Benign childhood epilepsy with centrotemporal spikes (BECTS) also known as benign rolandic epilepsy is the most frequent of all childhood epileptic syndrome. The debate about the differentiation of other (sub-)groups of idiopathic focal epilepsies is still going on. In 1982 Aicardi and Chevrie published their data on 32 patients with a less favourable course of the epilepsy and introduced the term atypical benign partial epilepsy of childhood. However, the term “benign“ in most instances seems not appropriate considering the outcome in many children. Some authors prefer the term atypical idiopathic partial epilepsy. In spite of the general benign course of BECTS children with atypical idiopathic focal epilepsy are characterized by different types of seizures occurring sometimes with high frequency. Unrelated to seizure frequency some children show an intense activation of EEG changes, sometimes with continuous spikes and waves during slow wave sleep (CSWS) with language dysfunction and/or neuropsychologic involvement. In theses cases, frequently many different antiepileptic drugs, often in combination, are used for seizure control with sometimes little or no success.

Sulthiame in childhood epilepsy

Sulthiame is a central carbonic anhydrase inhibitor found to be effective for both partial and generalized seizures. It has been in use in some European countries and in Israel for over 30 years. The aim of the present study was to evaluate the efficacy and tolerability of sulthiame in childhood epilepsy by conducting a multicenter, retrospective study of patients who received this drug. The charts of 125 consecutive epilepsy patients treated with sulthiame as monotherapy or add-on therapy were reviewed. Twenty-nine out of 39 patients with benign focal epilepsy of childhood became seizure-free. Total seizure control was also achieved in 17 of 42 patients with symptomatic, non-refractory localization-related epilepsy, and in all 10 cases with juvenile myoclonic epilepsy. Complete normalization of the EEG occurred in 13 of 20 patients with benign partial epilepy of childhood. Side-effects were minimal and caused discontinuation of treatment in only seven children. The high tolerability, efficacy, convenience of use and low cost suggest that sulthiame should become a first line drug in the benign partial epilepsies of childhood and juvenile myoclonic epilepsy. It also has a role as add-on treatment in other partial and myoclonic epilepsies.

‘Attention deficits and subclinical epileptiform discharges: are EEG diagnostics in ADHD optional or essential?’

Developmental Medicine & Child NeurologyVolume 46, Issue 7 p. 501-502 Free Access ‘Attention deficits and subclinical epileptiform discharges: are EEG diagnostics in ADHD optional or essential?’ Katja Becker MD, Corresponding Author Katja Becker MD Department of Child and Adolescent Psychiatry and Psychotherapy, Central Institute of Mental Health, Mannheim Department of Child and Adolescent Psychiatry and Psychotherapy Central Institute of Mental Health Mannheim, Germany E-mail: kbecker@zi-mannheim.deSearch for more papers by this authorJudith K Sinzig MD, Judith K Sinzig MD Department of Child and Adolescent Psychiatry and Psychotherapy, University of CologneSearch for more papers by this authorMartin Holtmann MD, Martin Holtmann MD Department of Child and Adolescent Psychiatry and Psychotherapy, JW Goethe-University, Frankfurt/MainSearch for more papers by this author Katja Becker MD, Corresponding Author Katja Becker MD Department of Child and Adolescent Psychiatry and Psychotherapy, Central Institute of Mental Health, Mannheim Department of Child and Adolescent Psychiatry and Psychotherapy Central Institute of Mental Health Mannheim, Germany E-mail: kbecker@zi-mannheim.deSearch for more papers by this authorJudith K Sinzig MD, Judith K Sinzig MD Department of Child and Adolescent Psychiatry and Psychotherapy, University of CologneSearch for more papers by this authorMartin Holtmann MD, Martin Holtmann MD Department of Child and Adolescent Psychiatry and Psychotherapy, JW Goethe-University, Frankfurt/MainSearch for more papers by this author First published: 13 February 2007 https://doi.org/10.1111/j.1469-8749.2004.tb00514.xCitations: 5AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1 Dunn DW., Austin JK., Harezlak J., Ambrosius WT. (2003)ADHD and epilepsy in childhood. Dev Med Child Neurol 45: 50– 54. 2 Noeker M., Haverkamp F. (2003) Neuropsychological deficiencies as a mediator between CNS dysfunction and inattentive behaviour in childhood epilepsy. Dev Med Child Neurol 45: 717– 718.(Letter). 3 Holtmann M., Becker K., Kentner-Figura B., Schmidt MH. (2003) Increased frequency of rolandic spikes in ADHD children. Epilepsia 44: 1241– 1244. 4 Hemmer SA., Pasternak JF., Zecker SG., Trommer BL. (2001) Stimulant therapy and seizure risk in children with ADHD. Pediatr Neurol 24: 99– 102. 5 Szatmari P., Offord DR., Boyle MH. (1989) Ontario Child Health Study: prevalence of attention deficit disorder with hyperactivity. J Child Psychol Psychiatry 30: 219– 230. 6 Halperin JM. (1991) The clinical assessment of attention. Int J Neurosci 58: 171– 182. 7 Dulcan M. (1997) Practice parameters for the assessment and treatment of children, adolescents, and adults with attention-deficit/hyperactivity disorder. American Academy of Child and Adolescent Psychiatry. J Am Acad Child Adolesc Psychiatry 36 (Suppl. 10): 85– 121. 8 Deonna T., Zesiger P., Davidoff V., Maeder M., Mayor C., Roulet E. (2000) Benign partial epilepsy of childhood: a longitudinal neuropsychological and EEG study of cognitive function. Dev Med Child Neurol 42: 595– 603. 9 Weglage J., Demsky A., Pietsch M., Kurlemann G. (1997) Neuropsychological, intellectual, and behavioral findings in patients with centrotemporal spikes with and without seizures. Dev Med Child Neurol 39: 646– 651. 10 Baglietto MG., Battaglia FM., Nobili L., Tortorelli S., De Negri E., Caleva MG., Veneselli E., De Negri M. (2001) Neuropsychological disorders related to interictal epileptic discharges during sleep in benign epilepsy of childhood with centrotemporal or Rolandic spikes. Dev Med Child Neurol 43: 407– 412. Citing Literature Volume46, Issue7July 2004Pages 501-502 ReferencesRelatedInformation

Epileptic negative myoclonus in a case of atypical benign partial epilepsy of childhood: a detailed video-polygraphic study

Purpose: To describe the clinical and electroencephalographic features of a child diagnosed as having atypical benign partial epilepsy (ABPE) who suffered from frequent lapses of postural tone in the right lower limb that were considered to represent a focal epileptic negative myoclonus (ENM). Material and methods: Electrophysiological evaluation included four serial waking and two sleep EEGs. Moreover, the investigation included a polygraphic recording with simultaneous video-EEG monitoring performed in rest, during hyperventilation, and while standing up with the aim of capturing patient’s typical seizures. Results: During awake the EEG showed frequent bilateral centrotemporal discharges maximal over the left central area. Moreover, we recorded short generalised paroxysms of spike-and-wave discharges accompanied by a clear transient cognitive impairment that were in keeping with absences. We also captured several episodes of sudden and unexpected loss of postural tone in the right lower limb. These focal inhibitory seizures were associated with brief diffuse but asymmetrical paroxysms of irregular spike-and-wave discharges maximal over the left side. EMG flattening of the right quadriceps muscle was time-locked to the midline of the slow-wave component of the diffuse spike-and-wave discharges. Conclusions: ENM leading to focal lapses of postural tone in lower extremities may be a predominant type of seizures in ABPE. A polygraphic recording with video-EEG monitoring is essential to confirm the diagnosis, and to detect other concomitant seizures, such as atypical absences. In our case, ENM could be more likely due to a transient disruption of cortical function.