摘要:

Summary:Epileptic syndromes are clusters of signs and symptoms regularly occurring together. These may include type of seizure(s), time and circumstances of occurrence, and neurological and EEG findings. Some syndromes have common signs and a predictable course (e.g., benign rolandic epilepsy). Others, such as absence seizures, are less specific and may include several subgroups with different outcomes and different associated features. Still others are rather loose collections of a few common characteristics irregularly linked together. Widely accepted syndromes in childhood include the West syndrome and Lennox‐Gastaut syndromes, several myoclonic syndromes, febrile seizures of infancy, absence epilepsy, benign partial epilepsy, and juvenile myoclonic epilepsy. If the concept of epileptic syndromes is to be practically useful, it should be limited to clusters that are unequivocally identifiable. Heterogeneous epileptic syndromes such as West syndrome or absence epilepsies give only a limited guide to prognosis but may be important in determining investigations and treatment.A classification based on syndromes has the advantage of dispensing with most assumptions required by other systems. However, it cannot cover all the clinical aspects of epilepsy and gives no guide to the pathophysiology, genetics, and etiology of a seizure disorder. The ultimate goal remains the delineation of disease entitie

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05803.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:William G. Lennox, author ofEpilepsy and Related Disorders, had a lasting effect on our understanding of this illness. He postulated that epilepsy was not a unitary condition and that neuronal chemistries differed from one form of the disease to another. A leader in the use of electroencephalography in epilepsy, he described the first nearly pathognomonic EEG pattern and demonstrated specific features for each of the three most common types of seizure. His pioneering investigations into the biochemical basis of epilepsy helped to identify pathological mechanisms in epileptic attacks. Lennox stood alone in his belief, now generally accepted, that the genetics of epilepsy could be understood only through a multifactorial mode of inheritance. The author presents an affectionate portrait of the physician, the teacher and the man, the founder of the Seizure Unit and the unifying force in the study of epilepsy by both professionals and lay persons.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05798.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:In both adults and children, the seizure type and frequency of occurrence largely determine the burden of epilepsy, for the patient and his family. Epilepsy in children can range from the severe to the relatively benign, and it is important to make the correct diagnosis as early as possible. In those under 5 years of age, epilepsy often presents as an age‐related syndrome, e.g. West and Lennox‐Gastaut syndromes. Later, typical absences and generalized tonic‐clonic convulsions are more usual. Drug therapy should take account of the different pharmacodynamics of antiepileptic drugs in children, and phenobarbital should be avoided. Rectal diazepam has an important role in the emergency management of childhood seizures. Psychological difficulties may be manifested by both the child and the parents. They should be prevented if possible by active intervention at an early stage after diagnosis, or managed on a continuing basis by expert medical and paramedical advice. The aim of such counseling is to ensure, so far as is possible, that the epileptic child develops normally in a normal family enviro

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05807.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:Several factors are known to modulate the occurrence of interictal spike‐wave complexes (SWC). During performance of a recent memory task by a patient with uncontrolled complex partial seizures and implanted electrodes, visually presented stimuli consistently evoked focal medial temporal lobe interictal SWC with an average latency of 228 ± 29 ms. The SWC were more often lateralized to the left medial temporal lobe. No consistent change in interictal spike rate was observed during performance of several other cognitive tasks during which visual or auditory stimuli were presented. This suggests that performance of specific cognitive tasks–in this case, recognition memory of words—can trigger interictal SWC.RÉSUMÉOn connait plusieurs facteurs qui modulent la survenue de pointes‐ondes (PO) intercritiques. Pendant l'éxecution d'un test de mémoire récent, la présentation de stimuli visuels a entraîné de façon reproductible l'a survenue de complexes de PO focales médio‐temporales après une latence moyenne de 228 ± 29 mse‐condes. Les PO étaient le plus souvent latéralisées à la partie moyenne du lobe temporal gauche. Nous n'avons pas observé de modification notable de la fréquence des PO intercritiques pendant l'exécution de plusieurs autres tests cognitifs qui comprenaient une présentation de stimuli visuels ou auditifs. Ceci suggère que l'exécution de tâches cognitives précises, dans notre cas la mémoire de reconnaissance des mots, peut déclencher l'apparition de PO intercritiques.RESUMENEs conocido que varios factores modulan la frecuencia de los complejos punta‐onda lenta interictales. Durante la realizacion de pruebas para memoria recienle con un paciente con ataques parciales complejos sin controlar y con elcctrodos implantados, los estímulos visuales presentados consistentemente ante él. evocaro n complejos punta‐onda lenta interictales focales en la porción medial del lóbulo temporal (SWC) con una latencia promediada de 228 ± 29 mseg. Los SWC aparecián más frecuentemente lateralizados hacia la porción medial del lóbulo temporal izquierdo. No se observó ningún cambio con respecto a la frecuencia de las punlas interictales duranle la realización de varios tests para medir la functión cognitiva. durante los cuales se presentaron ante el enfermo estímulos visuales o auditivos. Estos hallazgos sugieren que la realización de pruebas para medida de función cognitiva, en este caso el reconocimient

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05090.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:: Limited quantitative research has been undertaken on the social accompaniments of severe epilepsy. In this study we present new data from 92 patients with uncontrolled seizures necessitating admission to a special assessment unit in the United Kingdom. Comprehensive information on the medical, educational, and social history of each subject was obtained; moreover, they completed the Social Problems Questionnaire, which examines the respondent's level of satisfaction with various aspects of living including employment, finances, housing, etc. Thirty‐nine percent of the sample reported moderate or severe dissatisfaction in four problem areas. The most frequently reported problems were with social contacts (73%) and employment (71%). The significance of these findings is discussed and some remedial strategies outline

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05791.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:About 75% of patients with epilepsy have seizures during childhood, often requiring antiepileptic therapy. Children possess all the drug‐specific pharmacokinetic features of adults (e.g., nonlinearity of phenytoin elimination and autoin‐duction of carbamazepine metabolism), plus other factors (e.g., age, intercurrent illness, comedication) that influence dosage. Kinetic differences are maximal in newborns and infants, with limited drug elimination in premature and full‐term babies, soon followed by accelerated elimination during infancy and childhood, before lower adult elimination rates develop during late childhood or early adolescence. Most children with epilepsy require two‐ to fourfold larger doses relative to bodyweight than adults, to achieve comparable drug levels and therapeutic effects. Although rapid growth may require increased dosage, the need is limited as relative clearance declines with age. Children of any age, but particularly premature and newborn babies, show greater individual variability in drug handling and therefore in dose requirements than adults. Clinical response and antiepileptic drug concentrations should both be monitored carefully in c

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05804.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:The Wechsler Adult Intelligence Scale–Revised (WAIS‐R), the newest version of the Wechsler Scales, has had several factor analytic studies, but none have been done in a sample of seizure‐disorder patients. The factor structure of the WAIS‐R was examined in this study in a sample of 107 patients with various seizure disorders. The results of this study revealed the 3‐factor solution, as has been reported in many previous studies. The most notable difference was the relatively greater prominence of the Freedom from Distractibility factor. These results are consistent with studies of the factor structure of previous editions of the Wechsler Scales in samples of epileptic patients. The relative prominence of the Distractibility factor could suggest that attentional factors are a more important influence on performance than in other patient groups. Further studies are needed to clarify the effect of various seizure‐related variables on patterns of intelligence.RÉSUMÉL'échelle d'intelligence adulte de Wechsler révisée (WAIS‐R) a fait l'objet de plusieurs analyses factorielles. mais jamais chez les patients épileptiques. Nous avons étudié la structure factorielle du WAIS‐R chez 107 patients présentant divers types d'épilepsie. Nous avons constaté, comme les autres études, une solution de 3 facteurs. La différence la plus notable a été la prééminence relative du facteur “distractibilityé”. Nos résultats corroborent ceux des études de structure factorielle ayant porté sur les anciennes versions du WAIS chez les épileptiques. La prééminence relative du facteur distractibilityé pourrait signifier que les facteurs liés a l'attention ont plus d'influence sur les performances que dans d'autres groupes de patients. D'autres travaux devront clarifier l'influence de diverses variables liées aux crises sur les processus de l'intelligence.RESUMENLa escala revisada de Wechsler para medida de inteligencia de adultos (WAIS‐R). (la nueva versión de la escala de Wechsler) ha sido sometida a varios estudios analíticos de factor pero ninguno ha sido realizado en una muestra de enfermos con ataques epiléplicos. La estructura factorial del WAIS‐R ha sido examinada en este estudio en una muestra de 107 enfermos con varios tipos de ataques. Los resultados han revelado las 3 condiciones factorials tal como se han publicado en muchos estudios previos. La diferencia más notable ha consislido en la prominencia relativamente mayor del factor de libertad sobre el de distractibilidad. Los resultados son consistentes con estudios de estructura factorial, realizados con ediciones previas de la escala de Wechsler, en muestras de enfermos epilépticos. La prominencia relativa del factor de distractibilidad podría sugerir que los factores de atención conllevan una influencia más importante sobre las actividades totales que en otro grupo de enfermos. Estudios adicionales son necesarios para clarificar el efecto de varias variables relacion

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05091.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:The role of antiepileptic drugs in behavior and cognitive function in children is well documented in the literature. In general, behavioral problems occur most frequently with phenobarbital and clonazepam, and appear least often with valproate and carbamazepine. Cognitive impairments occur with phenytoin, are less evident with valproate, and minimal with carbamazepine. Monotherapy, as with adults, leads to improvements in both cognitive abilities and behavior.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05805.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:: The relationship of a number of epilepsy variables to cognitive decline is reviewed. Underlying brain damage, seizure age of onset, type and frequency, and anticonvulsant drugs are all considered. Two investigations in which subgroups of patients with intellectual deterioration have been compared to those without such deterioration are presented, and certain conclusions are reached with respect to the most important factors that relate to decline. Recurrent tonicclonic seizures, head injury, the prescription of phenytoin, and low folic acid levels were all related to cognitive decline in one or both of the studies, and the implications of this for the management of epilepsy are noted.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05787.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

Summary:Despite the current trend toward monotherapy, polytherapy in children with epilepsy is still common. A drug combination is advantageous only if it achieves a higher efficacy: toxicity ratio (therapeutic index) or if its antiepileptic spectrum is wider. Studies of brain concentrations of antiepileptic drugs have so far shown that a higher efficacy toxicity ratio is not achieved by most combinations. Problems are associated with drug combinations. First, numerous pharmacokinetic interactions are documented. These interactions, which can be associated with significant changes in blood levels at a given dose, make frequent measurements and dosage readjustments necessary. They can also alter the concentration of active metabolites or the free fraction of a drug. Second, toxicity can be assumed to be at least partially cumulative, since reduction in polytherapy has been shown to be associated with a reduction in side effects. Third, the therapeutic range appears to depend on whether a drug is taken alone or in combination, so that polytherapy confuses the interpretation of serum drug measurements. Fourth, the presence of more than one drug will add to the difficulty in evaluating the efficacy or side effects of any single drug. Finally, a pharmacodynamic interaction between valproate and several other antiepileptics, particularly the barbiturates, can lead to a stuporous state. Transition from polytherapy to monotherapy is much more difficult to achieve than the opposite.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1988.tb05806.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY