Epileptic spasms and partial seizures as a single ictal event

PURPOSE: To investigate the phenomenon of epileptic spasms (ESs) associated with other seizure types in a single ictal event and to study the predictive value of this phenomenon regarding etiology and prognosis. METHODS: We selected retrospectively eight female and five male patients, who had ESs and other seizure types within a single seizure event and for whom a video-EEG recording of the phenomenon was performed in at least one situation. RESULTS: The seizure type associated with ESs was a partial seizure in all patients. We identified three groups with different seizure patterns regarding the temporal association of ES and partial seizures (PSs): (a) PS followed by ES; (b) PS appearing during a cluster of ESs without interrupting the cluster; and (c) complex seizure interaction with a succession of ESs and PSs in a close but variable temporal association. Underlying disorders included cortical dysplasia (three patients), complex cerebral malformations (two patients), and perinatal anoxic-ischemic injuries (two patients); four cases were classified as cryptogenic, and in two children, etiology was unknown, but prenatal origin was suspected. Outcome was poor in nine cases with intractable epilepsy; four cases had a favorable outcome, defined as complete cessation of epileptic seizures. CONCLUSIONS: The phenomenon of associated ESs and PSs as a single ictal event can be related to different etiologies and should not be considered distinctive for cortical malformations or severe brain damage. Different seizure patterns of associated ESs and PSs provide no hint for etiology or prognosis. Outcome is prevalently but not constantly unfavorable in patients with the phenomenon.     

Hyperventilation-induced high-amplitude rhythmic slowing with altered awareness: a video-EEG comparison with absence seizures

PURPOSE: Hyperventilation-induced high-amplitude rhythmic slowing (HIHARS) in children may be associated with clinical episodes of altered awareness. The presence of automatisms has been proposed as a distinguishing feature that helps to differentiate absence seizures from nonepileptic causes of decreased responsiveness. This retrospective, controlled, video-EEG study compared the clinical characteristics of episodes of HIHARS with loss of awareness with those of absence seizures. METHODS: The database of a tertiary Children's Hospital was searched for patients studied between April 1993 and April 1997 who had at least one episode of HIHARS with loss of awareness. The absence control group was obtained by selecting the next patient, after an HIHARS study subject, who met the following criteria: (a) had at least one absence seizure occurred during hyperventilation in the EEG recording, and (b) had a diagnosis of idiopathic generalized epilepsy. The video-EEG and medical histories of all patients were reviewed and summarized. RESULTS: We reviewed video-EEG recordings of 77 episodes of HIHARS with loss of awareness from 22 children and 107 absence seizures during hyperventilation from 22 children. Eye opening and eyelid flutter were seen more frequently in absence seizures, whereas fidgeting, smiling, and yawning occurred more frequently during HIHARS episodes. Arrest of activity, staring, and oral and manual automatisms were observed in both groups. CONCLUSIONS: Automatisms are common in both HIHARS and absence seizures. Yawning, smiling, and particularly fidgeting occur more commonly and eye opening and eyelid flutter less commonly in HIHARS. However, episodes of HIHARS with loss of awareness clinically mimic absence seizures, and these conditions can be distinguished reliably only by EEG.     

The effect of lamotrigine on epileptiform discharges in young patients with drug-resistant epilepsy

PURPOSE: In a double-blind crossover study with lamotrigine (LTG), we investigated a possible relationship between the clinical responses and changes of the amount of epileptiform activity in EEG. METHODS: Twelve patients, aged 4-21 years, with generalized drug-resistant epilepsy who had responded to LTG, completed the study. Twenty-four-hour video-EEGs were taken during control, placebo, and drug phases. The amounts of epileptiform discharges were estimated and compared with the clinical effects. RESULTS: The duration of periods of repeated epileptiform discharges was significantly reduced during the LTG phase compared with the placebo phase (n = 12, p = 0.04). Ten patients showed a reduction of the amount of epileptiform discharges in the LTG phase by a mean of 81% (range, 17-100%). Periods of repeated epileptiform discharges with duration longer than 30 s were significantly reduced in length (p = 0.03) and number (p = 0.04) during the LTG phase compared with the placebo phase. Shorter periods of epileptiform discharges and the numbers of single epileptiform discharges were not affected. In five patients there was a seizure reduction (>/=50%) concomitant with the reduction of epileptiform discharges in the EEG. The behavior improved during LTG treatment in all patients. The patients became more alert, and their concentration and performance improved, according to their parents and the medical personnel. CONCLUSIONS: LTG in dosages of 1-8 mg/kg body weight was found to depress relatively long episodes of interictal, repetitive, epileptiform activity in young patients with drug-resistant epilepsy, whereas short episodes were not affected. It depressed seizures in about half of the patients studied but gave improvements of behavior in all patients.     

额叶癫痫发作录像脑电图分析

目的通过录像脑电图观察额叶癫痫的临床特征、发作期及发作间歇期的脑电图特点。方法使用录像脑电图30例确诊为额叶癫痫的患者进行常规及长时间记录，对其中12例癫痫发作的临床表现及脑电图所见进行分析。结果额叶癫痫常见的发作形式有：姿势性发作，具有额叶癫痫特点的自动症；复杂部分性发作时伴发声、偏转或表情的变化，有时出现发作性情绪改变或强迫思维等少见症状。其发作特点为：持续时间短，发作相对较频繁，无明显发作后意识障碍。发作间歇期脑电图有时可无阳性所见，典型的临床发作及发作时VEEG记录到的额部爆发性节律有助于诊断。结论额叶癫痫是一组较为特征性的癫痫综合征，临床并不少见，录像脑电图有助于正确的诊断，以便及时治疗。     

Outpatient Video-EEG Monitoring in Children

Summary: Video-EEG monitoring enables correlation of behavioral activity with EEG activity, which is useful in recognition of pseudoepileptic seizures and in investigation of patients for epilepsy surgery. Because most patients are monitored for a prolonged time as in-patients, the cost of the procedure is high. We investigated the value of brief (2–3 h) outpatient video-EEG monitoring in 43 children with frequent seizures, most of whom had symptomatic generalized epilepsy. Indications for monitoring included differentiation of epileptic from nonepileptic behavior, seizure classification, and determination of seizure frequency. Clinical episodes were recorded in 36 of 43 children (83%). A definite diagnosis was established in 9 of the 17 patients investigated to determine the nature of the clinical behavior. Seizures were classified in 1.5 of the 25 patients investigated to determine seizure type, and classification was different from the original in 9 of the 15 children. A change in epilepsy syndrome classification was made in 9 children. The video-EEG allowed diagnosis in 25 of the 43 children (59.5%). Video-EEG appears to be an effective method for outpatient investigation of children with frequent seizures, particularly those with symptomatic géneralizéd epilepsy.     

Speech-induced aphasic seizures in epilepsy caused by LGI1 mutation

Summary:  Purpose: Patients with autosomal dominant lateral temporal lobe epilepsy (ADTLE) may have seizures precipitated by sound or speech. We have examined a patient with speech-induced seizures caused by an LGI1 mutation (C46R).
Methods: A clinical study and a video-EEG recording using interrogative speech as the activation procedure was performed in a 23-year-old man.
Results: He had experienced short episodes of sensory aphasia in situations in which he was suddenly verbally addressed. Voices became distorted, and he could not comprehend despite hearing words. The day after a late party, his girlfriend unexpectedly spoke to him. Her speech became unintelligible to him. He did not reply and had a generalized tonic–clonic (GTC) seizure. During an EEG, he was suddenly asked for the names of his siblings. He answered, but lost understanding of the further conversation and described how syllables floated together with an echoing character. With a versive movement to the right, another GTC occurred. In the EEG, rhythmic 6-Hz activity built up in the frontotemporal areas starting on the left side with bilateral and posterior spreading. Postictal slowing was symmetrical, and no aphasia was noted on awakening.
Conclusions: To our knowledge, this is the first video-EEG recorded seizure in LGI1 -caused ADTLE. This peculiar seizure semiology and precipitating effect of speech may serve as a marker for identifying further individuals with this particular phenotype and genotype and may indicate that the LGI1 gene may have a physiologic function connected to the human capacity for speech and language.     

Outcome of prolonged video-EEG monitoring at a typical referral epilepsy center

PURPOSE: When seizures do not respond to medications, video-EEG monitoring is the best available diagnostic tool and is the principal activity of epilepsy centers. The purpose of this study was to analyze the eventual disposition of patients who undergo video-EEG monitoring at a typical referral epilepsy center. METHODS: We reviewed the diagnoses and dispositions of all patients (adults and children) who underwent inpatient video-EEG monitoring (> or = 24 h) at our center (University of South Florida-Tampa General Hospital) over a 1-year period (2002). RESULTS: In total, 251 inpatient video-EEG monitoring sessions were performed. Nonepileptic seizures were diagnosed in 75 (30%); 58 (23%) were found to be surgical candidates; seven were implanted with the vagus nerve stimulator. In 47 (19%) patients, seizures were recorded, and the diagnosis of epilepsy was confirmed and clarified (symptomatic/cryptogenic generalized epilepsy, seven; localization-related epilepsy, 35; idiopathic generalized epilepsy, five). CONCLUSIONS: The eventual outcome of video-EEG monitoring is diverse. The largest groups, as expected, are psychogenic nonepileptic seizures (30%), and surgery (23%).     

Ictal paresis associated to PLEDS in two children: A video-EEG study

Ictal paresis (IP) is a rare negative motor phenomenon presenting challenging differential diagnostic problems with transient ischemic attacks, post-ictal paralysis, migraine and psychogenic paralysis. Video-EEG undoubtedly represents the essential mean for a proper diagnosis. Periodic lateralised epileptiform discharges (PLEDs) are a distinctive EEG pattern, consisting of periodic spike or sharp wave discharges, often associated with seizures. It is under debate if PLEDs should be considered only a peri-ictal or also an ictal EEG pattern. We describe two children with severe focal epilepsies, who presented IP recorded during video-EEG monitoring, associated to PLEDs. Clinical observation along with interictal and ictal scalp-EEG findings, suggested a fronto-temporal seizure onset in the first, and a temporo-insular onset in the second. We confirm that PLEDs may be an ictal pattern associated with negative motor phenomena.     

The Absence of Interictal Spikes with Documented Seizures Suggests Extratemporal Epilepsy

PURPOSE: To determine the demographic and clinical characteristics of patients who have documented epileptic seizures on long-term video-EEG monitoring who do not have interictal spikes. METHODS: The records of 1,223 monitoring studies from 919 patients who underwent noninvasive long-term video-EEG monitoring were reviewed. In 28 patients (3.0% of monitored patients, 4.4% of patients with electrographic evidence of epilepsy), no interictal spikes were found despite the occurrence of at least one recorded electrographic seizure. The demographic, medical, neuropsychological, and EEG data of these patients were compared with those of 28 matched control patients with documented interictal spikes. RESULTS: Extratemporal seizures were significantly more frequent in the patients with at least one recorded epileptic seizure but without interictal spikes compared with patients with epileptic seizures and interictal spikes (p = 0.031). The only other significant difference between the groups (p = 0.016) was a later age at seizure onset (18.3 vs. 10.7 years) for the patients without interictal spikes. Age at evaluation, gender, handedness, clinical seizure type, family history of epilepsy, history of febrile seizures, neuropsychological testing, and neurologic and psychiatric history did not differ between the two groups. CONCLUSIONS: In patients with documented epilepsy without interictal spikes on EEG monitoring, the possibility of an extratemporal focus should be considered.     

短程视频脑电图对非痫性发作性疾病的诊断价值

目的 探讨短程视频脑电图对门诊非痫性发作性患者的诊断价值.方法 回顾性分析我院门诊129例非痫性发作患者的临床和视频脑电图资料.结果 129例患者中,临床诊断为晕厥24例、头痛19例、发作性意识丧失15例、神经症15例、头晕13例、短暂性脑缺血发作6例、其它37例.脑电图阵发性异常16例,痫样放电3例,检测中出现临床发作9例.结论 短程视频脑电图对门诊非痫性发作性疾病的诊断具有重要的参考价值.