Atypical Benign Partial Epilepsy Research Articles

Benign Childhood Partial Seizures and Related Epileptic Syndromes

In the last quarter of the twentieth century, epileptology made considerable progress thanks to new neuroimaging techniques, better antiepileptic drugs, but probably above all more accurate definition and description of the epileptic disorders. ‘Epilepsy’ is not an entity: because epilepsies encompass many and various conditions, accurate diagnosis is essential for management and prognosis. This is the view of Dr Panayiotopoulos, who declares, ‘Dramatic progress in the management of epilepsies can be expected if more time and emphasis are given on how to diagnose epilepsies rather then the current theme how to treat epilepsy’. Commissions of the International League against Epilepsy proposed categorizations, a classification of epilepsy syndromes in 1989, and an epidemiological classification in 1992. However, such proposals have a short half-life and must be updated and revised. Dr Panayiotopoulos' book is both an extensive review of established facts and also a critical view of current knowledge, based on his personal experience. In chapter 1, under the heading The Diagnosis of Epilepsies the syndromic concept is convincingly championed. Chapter 2 demonstrates that the electroencephalogram (EEG) is not an obsolete tool but is indispensable for a correct diagnosis (magnetic resonance imaging is normal in some two-thirds of seizure patients). Chapter 3 offers a nice summary of these disorders, and also suggests that the term ‘seizures’ or ‘seizure susceptibility’ should be used instead of ‘epilepsy’. Apart from the pathophysiological arguments, an objection to this proposal is that the best way to draw epilepsy out of the shadows and abolish its stigma is to accept the term. Some syndromes are well defined and easy to diagnose if the necessary details are ascertained—as in most cases of benign childhood epilepsy with centrotemporal spikes (chapter 4) and early-onset idiopathic childhood occipital epilepsy (chapter 8). They are astutely and thoroughly described here. The borders of benign childhood epilepsy with centrotemporal spikes and other conditions with a similar EEG pattern are detailed in chapter 5. Chapter 6 describes occipital seizures and chapter 7 shows that they occur in various epileptic disorders, symptomatic as well as idiopathic. The fact that, whereas rolandic seizures are mainly a manifestation of an idiopathic epilepsy, the same is not true in occipital seizures, is clearly shown. Correct diagnosis of late-onset benign childhood epilepsy is far more difficult (chapter 9). The pitfalls of a hasty interpretation of occipital epileptiform abnormalities are discussed at length in chapter 10. After these updated descriptions, subsequent chapters place them in an historical frame. The obvious relationship between occipital seizures, occipital EEG foci and the role of cortex in visual functions leads to a discussion of seizures with specific modes of precipitation and mainly photosensitive epilepsies, illustrated with the author's personal studies. Differentiation of occipital epilepsies from migraines is justified because of the complex relation between migraine and epilepsy and the fact that, in 1978, cases of idiopathic occipital epilepsy were published as basilar migraine. Last, the author addresses the problem of the borders of the so-called benign partial seizure susceptibility syndrome, with other clinical phenotypes or other locations of EEG epileptiform abnormalities, and of what are called ‘related epileptic syndromes’ such as Landau—Kleffner syndrome, epilepsy with continuous spikes and waves during slow sleep and atypical benign partial epilepsy of childhood. Since the proposal of a classification of epilepsies and epileptic syndromes in 1989, epileptologists have divided themselves into splitters, admitting a host of specific syndromes, and lumpers, finding a continuum between epileptic events. Panayiotopoulos is both a splitter (read this book) and a lumper (read chapter 18). He is also a precursor, adding personal and astute views to an exhaustive review of the published work.

ESES Syndrome with Acquired Aphasia

Case Report: A 7‐year‐old boy presented with a 2‐year history of convulsions. Birth history was unremarkable. His motor and mental development were normal until the onset of partial seizures of his face at the age of 5.3 years, usually upon awakening. CBZ failed to control seizures. At 6.3 years he began to display slow speech, drooling, and reading difficulties. Absence attacks began at age 7. Upon examination, muscle weakness and cerebellar ataxia and paresis were absent. He showed sudden loss of tone in the arms when eating or writing, considered to represent negative myoclonus. Laboratory examination including CBC, blood chemistry, CSF studies, and MRI all were normal. EEG showed continuous diffuse spike and wave bursts associated with focal spikes at mid‐temporal and central area in the sleep record. Overnight sleep EEG showed continuous diffuse spike and wave bursts during slow wave sleep. The spike wave index was about 30% among slow wave sleep stages. During REM sleep, rapid eye movements disturbed the discharges of the diffuse spike and wave complex on EEG. His intelligent quotient was about 73 in the Tanaka‐Binnet test. Attention was limited and he could not copy figures accurately due to pour eye‐hand coordination. Speech therapy evaluation found word finding difficulty, paraphasia, slow speech, misuse of different consonants, distortion of vowels, and motor articulatory disturbance or motor aphasia. He lacked oral dyspraxia. During a hospital admission, ACTH‐2 was administered intramuscularly 2 I times in addition to oral administration of valproate, phenytoin, and carbamazepine. Convulsions were controlled, and he gradually recovered from the motor aphasia. Diffuse abnormalities of EEG resolved, but focal spikes remained over centro‐temporal areas during sleep. Cognitive function and vocabulary improved. Negative myoclonus of his hand disappeared. Several months after ACTH therapy, however, he suffered from moderate difficulties with word finding and the EEG deteriorated. Conclusions: ESES syndrome consists of “electrical status epilepticus during sleep”, “nonconvulsive status epilepticus with epileptic negative myoclonus”, “atypical benign partial epilepsy”, and “LandauKleffner syndrome“. According to Ohtahara, Landau‐Kleffner syndrome is characteristic of auditory agnosia. There have been no reports of motor aphasia associated with this syndrome. The present case does not meet criteria for “electrical status epilepticus during sleep” because of the low spike‐wave index but may be considered a variant of ESES. The distinctive features of this case are motor aphasia and negative myoclonus, as well as diffuse spike‐waves during slow wave sleep. Long‐term prognosis of this patient is not presently known.

Foix‐Chavany‐Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster‐Drought syndrome

Foix‐Chavany‐Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster‐Drought, who described the clinical entity in children 40 years ago, the term Worster‐Drought syndrome is proposed for this unique disorder in children.

Evoluciones atípicas de la epilepsia parcial benigna de la infancia con espigas centrotemporales

To stress that different atypical evolutions occur in the course of disease in some patients with the so called benign partial epilepsies of childhood (BPEC). To promote interest in finding clinical and/or electroencephalographic clues to recognize which patients might be prone to present these risks. Follow up of 26 pediatric patients who started with typical clinical and EEG features of benign childhood epilepsy with centro-temporal spikes (BCECTS) but presented reversible or persistent serious epileptic events including status epilepticus and language, cognitive or behavioral impairments. Repeated neurological examinations, EEG records and neuropsychological evaluations were done in the course of up to 14 years. Brain imaging studies (CAT and/or MRI) were obtained in all patients. Four groups of patients were recognized: 11 children with atypical benign partial epilepsy of childhood (ABPEC) followed during 4 to 13 years. All have finally recovered and attend normal schools, although five have learning difficulties. Three patients met diagnostic criteria for Landau-Kleffner syndrome (LKS). Two of them recovered from aphasia and in one, some language difficulties persist. Seven children showed status epilepticus of BCECTS but all of them are now normal after 3 to 14 years of follow-up. Five children showed mixed features of the three groups mentioned above and three of them fulfilled the criteria for diagnosis of the syndrome of continuous spike-wave during slow sleep (CSWS). A small proportion of cases starting with BCECTS evolve into either ABPEC, LKS, status of BCECTS or the syndrome of CSWS. Then, BCECTS is not always benign. Clinical and EEG markers should be sought to predict these atypical evolutions.

063 Familial sibling cases of atypical benign partial epilepsy of childhood and benign epilepsy of childhood with centrotemporal spikes

063 Familial sibling cases of atypical benign partial epilepsy of childhood and benign epilepsy of childhood with centrotemporal spikes

Enfoque diagnóstico del niño con epilepsia y deterioro neuropsicológico

Symptomatic epilepsy secondary to hereditary metabolic or degenerative disorders, is usually associated to neurological deterioration. Though epilepsy by itself does not induce neurological deterioration, we should remind that some epileptics encephalopathies, such as the West or Lennox-Gastaut syndromes, do actually induce limited neurological deterioration. Furthermore, in some forms of complex partial epilepsy, motor problems and behavior disorders can be observed, specially in adolescents with temporary lobe epilepsy. Other forms of epilepsy, such as the atypical benign partial epilepsy or the Landau-Kleffner syndrome, can present a certain degree of cognitive deterioration in the evolution, although they can recover later lost functions, totally or partially. The evolution of some refractory epilepsy, as patients are submitted to a multiple treatments, can make us suspect a degenerative disease. In some cases, the diagnosis of the hereditary metabolic and heredodegeneratives diseases can be made by the characteristics of the seizures but in most cases the diagnosis will be established by the symptoms of the basic disease and the lab data.

Atypical benign partial epilepsy of childhood (ABPEC): a novel therapeutic strategy

I progressi compiuti in questi ultimi anni nella gestione degli infarti cerebrali in fase acuta hanno permesso di ridurre significativamente la morbilità e la mortalità legate a questa grave patologia. Dopo la conferma della diagnosi grazie alle neuroimmagini, le indagini eziologiche vengono condotte parallelamente alle misure terapeutiche. La terapia comprende alcuni provvedimenti non specifici: mantenimento delle funzioni respiratoria, cardiaca ed emodinamica e mantenimento dell’equilibrio idroelettrolitico e glicemico. Le complicanze neurologiche o sistemiche (edema cerebrale, trasformazione emorragica, epilessia, infezioni, malattia tromboembolica venosa) peggiorano la prognosi dell’infarto cerebrale e devono essere prevenute e/o trattate. Le misure terapeutiche specifiche comprendono la messa in atto precoce di una terapia antitrombotica o una ricanalizzazione con trombolisi se il trattamento può essere somministrato entro le 4 ore e 30. Il tempo è il principale fattore che limita l’uso della trombolisi, tanto più che i segni di allarme dell’infarto cerebrale sono largamente misconosciuti dal pubblico. I trattamenti endovascolari possono essere proposti per alcune indicazioni specifiche in centri specializzati. I neuroprotettori non hanno, ad oggi, dato prova della loro efficacia. Il ricovero in unità specializzate (stroke unit) costituisce parte integrante della terapia. L’infarto cerebrale deve ormai essere considerato un’urgenza trattabile come l’infarto del miocardio. Una migliore informazione del pubblico e l’adattamento del sistema assistenziale, specialmente durante la fase preospedaliera, fanno parte degli obiettivi da raggiungere per far sì che il maggior numero di pazienti possa beneficiare di questi progressi.

Symptomatology in children with focal sharp waves of genetic origin

The purpose of the study was to identify the clinical symptomatology of children with focal sharp waves of genetic nature. Genetic determination was assumed if the EEG of at least one sib revealed typical focal sharp waves. Forty-one probands (27 boys, 14 girls) and their 44 sibs showed a broad spectrum of different seizure types of focal origin including so-called atypical benign partial epilepsy (pseudo-Lennox-syndrome) and febrile convulsions. Numerous cases showed psychomental retardation or selective deficits of development. The classical syndrome of rolandic seizures with normal psychomental development and normal neurological findings was present in only a few cases. Similarly, EEG findings were quite variable. Rolandic sharp wave foci alone were found in only 22% of the probands. The remainder had foci in other regions (17%), or multiple foci (61%). Depending on age at first seizure, 42%-79% of the seizure-affected probands and 50% of the affected sibs showed generalized heritable EEG traits such as 4-7/s rhythms, generalized irregular spikes and waves, and/or photoparoxysmal response. Three pairs of monozygotic twins were concordant for the special type of EEG findings, two of them also for clinical symptoms.

Nonconvulsive status epilepticus with continuous diffuse spike-and-wave discharges during sleep in childhood.

On three epileptic conditions with common characteristics of almost continuous diffuse spike-and-wave discharges during sleep in EEG, clinical and electroencephalographic studies were undertaken to elucidate their pathophysiologies and interrelationships; namely on five cases of epilepsy with electrical status epilepticus during slow sleep (ESES), seven cases of a peculiar type of nonconvulsive status epilepticus in childhood (PNSE) and three cases of atypical benign partial epilepsy (ABPE). The dominant seizure types were absences and/or GTC in ESES, whereas they were focal motor seizures in PNSE and ABPE with more focalized epileptic discharges on EEGs than those in ESES. All the three conditions showed both features of generalized and partial epilepsies, although the former features were more prominent in ESES and the latter in PNSE and ABPE.

Combined myoclonic-astatic and "benign" focal epilepsy of childhood ("atypical benign partial epilepsy of childhood"). A separate syndrome?

The authors have followed six children with atypical epilepsies but a favorable evolution, consisting in minor motor seizures of the myoclonic-astatic type (with diffuse slow spike-waves on the electroencephalogram) together with clinical and EEG features seen in benign focal epilepsy of childhood (BFEC), an association recently reported by Aicardi and Chevrie (1982). The maintenance of a normal neurological function despite severe epilepsy, the absence of tonic seizures and the marked activation of the spike discharges during sleep were described by these authors as important characteristics suggesting a good prognosis. The purpose of this study was to see if these children indeed represent a particular subgroup of idiopathic epilepsy and to draw attention to a special clinical and EEG combination indicating a possibly favorable ultimate outcome, in children usually diagnosed as suffering from Lennox-Gastaut syndrome. The six cases closely resembled the group described by Aicardi and Chevrie (1982), although the clinical and EEG features of BFEC were not as striking as in their cases. Also transient mental deterioration occurred during the active seizure periods. The therapeutic benefit of the various drugs tried was difficult to assess, but the behavior was often perturbed by medication. Although it is not possible to decide at the present time if these cases represent a particular epileptic syndrome, the special combination of clinical and EEG features seems characteristic enough to justify prospective studies of similar cases in the future.

Partial epilepsy in neurologically normal children: clinical syndromes and prognosis.

A clinical and electroencephalographic study of 107 neurologically normal children with partial seizures was undertaken to verify the existence and determine the frequency of epileptic syndromes reported in selected populations. Sixty-three children had simple partial seizures, 39 had complex partial seizures, and 5 children were unclassifiable. The syndrome of benign partial epilepsy of children with rolandic spikes (BPEC, 38 cases) was clearly identified and its uniformly benign final prognosis was confirmed even if some of these children had at times severe or poorly controlled seizures. Among the children with simple partial seizures outside the BPEC (25 cases) and complex partial seizures (39 cases), no homogeneous clinical or electroclinical subgroup could be found. Two children with benign partial epilepsy and myoclonic-astatic seizures ("atypical benign partial epilepsy of childhood") and one child with "benign epilepsy with occipital spike-waves" were identified. 74% of children with epilepsy with complex partial seizures (ECP) had a 1-year seizure-free interval, and many children with epilepsy with simple partial seizures outside the BPEC group (ESP) had no more than two seizures. A benign course is thus not limited to the BPEC but is difficult to predict. Prospective studies are necessary to confirm the existence of well-defined benign syndromes among the idiopathic partial epilepsies of childhood, which appear quite rare outside the BPEC.