Some Clinical and EEG Aspects of Benign Juvenile Myoclonic Epilepsy

Asconapé, Jorge J.; Jk, Penry

doi:10.1111/j.1528-1157.1984.tb04163.x

Cited by 169 publications

(111 citation statements)

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“…The great majority of the patients with JME responded well to the treatment 3,17 . In our cohort, 39.3% of the patients qualify as resistant to the treatment.…”

Section: Discussionmentioning

confidence: 99%

Epileptiform Asymetries and Treatment Response in Juvenile Myoclonic Epilepsy

Létourneau

Cieuta‐Walti

Deacon

2010

Can. j. neurol. sci.

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Juvenile myoclonic epilepsy (JME) is a common idiopathic generalized and age-related epileptic syndrome. This syndrome is distinctively characterized by myoclonic jerks often associated with generalized tonic-clonic seizures (GTCS) and, less often, absence seizures 1,2 . Myoclonic seizures occur often shortly after awakening and can be precipitated by sleep deprivation and also by alcohol and photic stimulation. Usually, patients with JME respond well to appropriate treatment with valproate 3,4 . Other useful anti-epileptic drugs (AEDs) also used with other generalized epilepsies include: lamotrigine, levetiracetam and topiramate 5 .The interictal electroencephalogram (EEG) shows diffuse bilateral polyspike and slow waves (PSW) and spike and wave complexes (SWC) on a normal background 2 . The usual ABSTRACT: Background: Epileptiform electroencephalogram (EEG) asymmetries are not uncommon in juvenile myoclonic epilepsy (JME) and can contribute to the misdiagnosis of this syndrome. The objective of this study is to further characterize patients with focal or asymmetric epileptiform electroencephalographic abnormalities and more specifically in terms of response to treatment. Controversial data exists in the literature concerning this issue. Methods: We retrospectively reviewed clinical and EEG data of a group of consecutive JME patients followed at our Epilepsy Service. The first EEG available for each patient was reviewed blindly by two independent electroencephalographers. Results: Twenty-eight patients with JME were identified: 11 (39.3%) were resistant to at least one appropriate anti-epileptic drug (AED), including valproate, lamotrigine, topiramate or levetiracetam. All patients except two had generalized epileptiform abnormalities. Overall, EEG asymmetries were detected in 57.1% of the cases. The proportion of EEG asymmetries between AED-sensitive group (52.9%) and AED-resistant group (63.5%) did not reach statistical significance. Concordance between examiners for identification of EEG asymmetries was good. Analysis of patients with and without asymmetries showed no statistically significant differences in comparisons of age, family history of seizure, presence of polyspike and slow wave, photosensitivity and timing of EEG related to the onset of treatment. Conclusion: Asymmetric electroencephalographic abnormalities are frequent in patients with JME. These features should not be misinterpreted as being indicative of partial epilepsy. In our group, asymmetries were not associated with resistance to treatment.RÉSUMÉ: Asymétries épileptiformes et réponse au traitement dans l'épilepsie myoclonique juvénile. Contexte : Les asymétries épileptiformes à l'EEG sont fréquentes dans l'épilepsie myoclonique juvénile (EMJ) et peuvent contribuer à un diagnostic erroné de ce syndrome. Le but de cette étude était de mieux caractériser les patients qui ont des anomalies focales ou des anomalies asymétriques épileptiformes à l'EEG, plus particulièrement en ce qui concerne la réponse au traitement. Il existe dans la littéra...

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“…The great majority of the patients with JME responded well to the treatment 3,17 . In our cohort, 39.3% of the patients qualify as resistant to the treatment.…”

Section: Discussionmentioning

confidence: 99%

Epileptiform Asymetries and Treatment Response in Juvenile Myoclonic Epilepsy

Létourneau

Cieuta‐Walti

Deacon

2010

Can. j. neurol. sci.

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“…Some authors 5,21 described focal discharges in JME, in this study present in 9 patients in routine EEG compared to 12 in the sleep-deprived EEG. In the series of Aliberti et al 1 4 , besides the typical generalized abnormalities, focal alterations were seen in at least one EEG in more than half of the patients and asymmetry of generalized discharges in an e v e n a greater proportion.…”

Section: Discussionmentioning

confidence: 99%

EEG recording after sleep deprivation in a series of patients with juvenile myoclonic epilepsy

Sousa

Garzón

et al. 2005

Arq. Neuro-Psiquiatr.

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-Seizures in Juvenile Myoclonic Epilepsy (JME) are dependent on the sleep-wake cycle and precipitant factors, among which sleep deprivation (SD) is one of the most important. Still an under diagnosed syndrome, misinterpretation of the EEGs contributes to diagnostic delay. Despite this, a quantitative EEG investigation of SD effects has not been perf o rmed. We investigated the effect of SD on EEGs in 41 patients, aged 16-50 yr. (mean 25.4), who had not yet had syndromic diagnosis after a mean delay of 8.2 yr. Two EEG recordings separated by a 48-hour interval were taken at 7 a.m. preceded by a period of 6 hours of sleep (routine EEG) and after SD (sleep-deprived EEG). The same protocol was followed and included a rest wakefulness recording, photic stimulation, hyperventilation and a post-hyperventilation period. The EEGs were analyzed as to the effect of SD on the number, duration, morphology, localization and pre d ominance of abnormalities in the diff e rent stages. A discharge index (DI) was calculated. Out of the 41 patients, 4 presented both normal EEG recordings. In 37 (90.2%) there were epileptiform discharges (ED). The number of patients with ED ascended from 26 (70.3%) in the routine EEG to 32 (86.5%) in the sleep-deprived exam. The presence of generalized spike-wave and multispike-wave increased from 20 (54.1%) and 13 (35.1%) in the first EEG to 29 (78.4%) and 19 (51.4%) in the second, respectively (p<0.05 and p<0.01). As to localization, the number of generalized, bilateral and synchronous ED increased from 21 (56.8%) to 30 (81.1%) (p<0.01). The DI also increased; while 8 patients (21.6%) presented greater rate in the routine EEG, 25 (67.6%) did so in the sleep-deprived EEG mainly during somnolence and sleep (p<0.01). Moreover, the paroxysms were also longer in the sleep-deprived EEG. Sleep-deprived EEG is a powerful tool in JME and can contribute significantly to the syndromic characterization of this syndrome.KEY WORDS: sleep deprivation, EEG diagnosis, juvenile myoclonic epilepsy.Registros eletrencefalográficos após privação de sono em uma série de pacientes com epilepsia mioclônica juvenil RESUMO -Na epilepsia mioclônica juvenil (EMJ), uma síndrome epiléptica ainda subdiagnosticada, as crises são dependentes do ciclo vigília-sono e de fatores precipitantes, entre os quais a privação de sono (PS) é um dos mais importantes. A interpretação inadequada dos EEGs contribui para atraso no diagnóstico. Ainda não foi realizada investigação quantitativa sobre os efeitos da PS. Avaliamos o efeito da PS nos EEGs de 41 pacientes entre 16 e 50 anos (média 25,4) com EMJ em dois registros eletrencefalográficos, separados por intervalo de 48 horas. Os exames foram realizados às 7 horas da manhã, precedidos por um período de 6 horas de sono (EEG de rotina) e após PS (EEG com PS). Seguimos o mesmo protocolo que incluiu o registro em vigília em repouso, fotostimulação, hiperventilação e pós hiperventilação. O efeito da PS foi analisado sobre o número, duração, morfologia, localização e predominância das anor...

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“…This is especially evident in the myoclonic jerks and tonic-clonic seizures in juvenile myoclonic epilepsy. 3 It is also striking that, in some people, seizures can occur exclusively at night, an observation mentioned in ancient texts. Indeed, Aristotle stated that "the beginning of this malady (epilepsy) takes place with many during sleep, and their subsequent habitual seizures occur in sleep, not in waking hours".…”

Section: Temporal Association Of Epilepsy With Sleepmentioning

confidence: 99%

Epilepsy and Sleep Disorders

Walker¹,

Eriksson²

2011

US Neurology

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Throughout the ages, sleep has been considered akin to death; indeed, Homer referred to sleep as the 'brother of death'. However, the advent of electroecephalography (EEG) has expelled this misconception and sleep is now recognized as consisting of active brain states during which many biologic processes occur, such as synaptic plasticity and memory consolidation.1 Sleep can be broadly divided into rapid eye movement (REM) sleep and non-REM (NREM) sleep. These sleep states cycle over 90 minutes throughout the night. NREM can be subdivided into light (stages I/II) and deep (stages III/IV) sleep (see Figure 1). Temporal Association of Epilepsy with SleepPerhaps the best established relationship between epilepsy syndromes and sleep is in idiopathic generalized epilepsy syndromes, in which seizures occur shortly after waking. This is especially evident in the myoclonic jerks and tonic-clonic seizures in juvenile myoclonic epilepsy. 3 It is also striking that, in some people, seizures can occur exclusively at night, an observation mentioned in ancient texts. Indeed, Aristotle stated that "the beginning of this malady (epilepsy) takes place with many during sleep, and their subsequent habitual seizures occur in sleep, not in waking hours". 4 Later studies from the 19th and early 20th centuries determined that 20 % of patients with epilepsy have nocturnal seizures only. 5,6 Importantly, the occurrence of seizures predominantly during sleep is a characteristic of specific epilepsy syndromes. In particular, nocturnal seizures are typical of frontal lobe epilepsy, in which seizures usually cluster and can occur many times in a night. 7 Nocturnal seizures occur throughout all stages of NREM sleep and are distributed proportionately, so that they occur more frequently during light stages rather than deep stages (see Figure 2).Similarly, seizures in benign focal epilepsy with centrotemporal spikes (BECTS) show a predilection for sleep or drowsiness. 10 Although temporal lobe epilepsy does not show this diurnal variation, if temporal lobe seizures occur during the night, they are more likely to be secondary generalized. 7,9 Certain epileptic encephalopathies also show marked diurnal variation in seizure manifestation and electrographic activity. This is a particular feature of electrical status epilepticus during sleep (ESES), which is characterized by spike and wave discharges in 85-100% of NREM Table 1). 34 Nocturnal Frontal Lobe Epilepsy and Non-rapid Eye Movement Parasomnia Diagnosis and Differential DiagnosisNocturnal frontal lobe seizures can manifest as paroxysmal arousals, which consist of brief, sudden eye opening, head raising or sitting up in bed, a frightened expression and, sometimes, vocalization; or nocturnal paroxysmal dystonia, which involves dystonic posturing and hypermotor (complex motor) phenomena, and episodic nocturnal wanderings, which are longer in duration (one to three minutes), with associated stereotyped Figure 3). 41 Nevertheless, even with access to good-quality video-EEG telemetry, the d...

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Some Clinical and EEG Aspects of Benign Juvenile Myoclonic Epilepsy

Cited by 169 publications

References 2 publications

Epileptiform Asymetries and Treatment Response in Juvenile Myoclonic Epilepsy

Epileptiform Asymetries and Treatment Response in Juvenile Myoclonic Epilepsy

EEG recording after sleep deprivation in a series of patients with juvenile myoclonic epilepsy

Epilepsy and Sleep Disorders

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