Early-onset absence epilepsy: clinical and electroencephalographic features in three children

To describe the clinical and electroencephalographic features of three infants diagnosed as having early-onset absence seizures. Two males and one female, aged 21-29 months were seen in our neuropaediatric outpatient clinic because of daily episodes of motor arrest and loss of contact. Neurological examination and mental development was considered normal in all of them. Two out three had first-degree relatives with seizures with onset in the childhood and favourable evolution in the adulthood. A video-electroencephalogram was requested. Ictal EEG revealed a normal background and generalised spike-and-wave complexes at 3-3.5 Hz accompanied by disruption of ongoing activity in keeping with absence seizures. The duration of seizures ranged from 2 to 10s. One child (patient 2) experienced rhythmic myoclonic jerks in upper limbs and head as those described in myoclonic absences. Clinical and electroencephalographic follow-up ranged from 8 months to 4 years. Two children were on treatment with valproate and in the case of the patient 3, the combination of valproate and ethosuximide was necessary. Control of absence seizures was achieved in all our cases. Absence seizures should be considered as a possible cause of transient impairment of consciousness even among infants minor than 3 years of age. A video-electroencephalogram is the method of choice in the diagnostic evaluation and syndromic classification of these paroxysmal events.     

Supplementary motor seizures: clinical and electroencephalographic findings

The clinical and EEG features of 11 patients with seizures arising in the supplementary motor area (SMA) were reviewed. All patients underwent prolonged EEG with simultaneous video recording. Three patients had recordings and electrical stimulation of the SMA using subdural electrode arrays. All patients had preservation of consciousness during the seizure unless it became secondarily generalized. Tonic posturing of the extremities was present in all patients, and in seven it was present bilaterally. Adversive movements were not seen unless the seizure became secondarily generalized. Interictal and/or ictal abnormalities were present at or adjacent to the midline in ten patients. Seizures arising from the supplementary motor region are clinically distinct, and the diagnosis can almost always be verified with prolonged EEG/video recording.     

Idiopathic childhood occipital epilepsies: clinical and electroencephalographic features in 63 children

We studied clinical-EEG features of 63 children, age range of 2-14 years, with occipital epileptiform activity in the EEG, with epileptic seizures and no evidences of brain damage. Age at onset was between 1-12 years. In 15 cases the seizures last for more than 30 min up to 6 h. Seizures occurred during sleep in 28 children. Autonomic symptoms were apparent in 25, head deviation in 24 and hemiconvulsion or generalization in 17 cases. Ten children reported visual symptoms. In 14 children the seizures were exclusively generalised convulsions. Spikes were observed in 27 cases and spike and slow-wave complex in 36. Discharges blocking by eyes opening were confirmed in 15 cases. Somatosensory evoked spikes by foot stimulation were observed in 9.5% cases. The cases were classified in the following idiopathic epileptic syndromes: early-onset occipital -- Panayiotopoulos-type (EOO), 32 cases; late-onset occipital -- Gastaut type (LOO), 8; rolandic epilepsy in 7. CONCLUSION: EOO occurred more frequently than LOO. Idiopathic occipital syndromes diagnosis was possible in 63.4% of the cases.     

Absence seizures with evolution into generalized tonic-clonic activity: clinical and EEG features

PURPOSE: To report the clinical and electrographic features of absence seizures evolving into generalized tonic-clonic (GTC) activity in six patients with idiopathic generalized epilepsy. METHODS: All patients were referred for evaluation of refractory seizures and underwent video-EEG monitoring after discontinuation of their antiepileptic drugs (AEDs). We analyzed the video-EEG recordings for seizure semiology as well as ictal and interictal activity. We also reviewed the initial clinical data in all patients. RESULTS: All patients were women, with a mean age of 27 years (range, 14-43 years). The mean age at seizure onset was 12 years (range, 5-15 years). Family history was positive for epilepsy in four patients. All patients had recorded seizures with an onset that was characteristic of generalized absence clinically and electrographically, with evolution into GTC activity. The EEG onset was with generalized 2.5-to 5-Hz spike-and-wave discharges, with evolution into faster rhythmic activity. Interictal EEG recordings showed generalized 2-to 5-Hz spike-and-wave discharges. All had normal background activity. All patients were treated with divalproex monotherapy. Five patients have been seizure free, and one had a single breakthrough GTC seizure during a follow-up period of 12-36 months. CONCLUSIONS: GTC activity may evolve from typical absence seizures. This seizure type should be included in the International Classification of Seizures. Its recognition and distinction from complex partial seizures with secondary generalization are important for appropriate therapy.     

Clinical and electroencephalographic features of simple partial seizures

The clinical and electroencephalographic features of 87 simple partial seizures in 14 patients were studied with video-EEG telemetry. The patients were able to respond to verbal stimuli during all seizures and, later, could clearly recall ictal events. To determine whether the EEG changes in simple partial seizures could be reliably observed, a reader blindly reviewed four EEGs of equal duration for each seizure. These EEGs consisted of one ictal and three nonictal recordings obtained at predetermined times before the seizure. There were 27 motor seizures (mean duration, 86 seconds; range, 2 to 250 seconds), all involving clonic movements of the head and/or upper extremities; 8 (30%) of these had a sensory component (pain in 6, paresthesia in 2). An EEG change, usually localized spikes or sharp waves over the contralateral or both rolandic regions, was identifiable in nine (33%) of the motor seizures. The 60 nonmotor seizures (mean duration, 63 seconds; range, 8 to 375 seconds) involved a variety of symptoms, including somatosensory/special sensory (3 seizures), autonomic (26 seizures), cognitive (1 seizure), affective (14 seizures), and mixed, or more than one category of nonmotor symptoms (16 seizures). In only nine (15%) of the nonmotor seizures was there an ictal EEG change, usually localized spikes or paroxysmal theta activity over the temporal region. Overall, among the 87 simple partial seizures, only 18 (21%) revealed ictal EEG changes. Thus, a normal EEG is common during simple partial seizures and does not exclude the diagnosis.     

Benign familial neonatal seizures: clinical and electroencephalographic characteristics

Two families with benign familial neonatal seizures evident over three generations are presented with emphasis on clinical and electroencephalographic features. Benign familial neonatal seizures represent a disorder with the onset of frequent generalized seizures during the first weeks of life. Family history reveals autosomal dominant inheritance of this disorder. The neonates are neurologically normal and outcomes are usually benign. In our families, none of the patients had seizures after the first ten months of life, with long-term follow-up ranging from 10 months to 56 years. The use of antiepileptic medication seems to have little effect on course or eventual outcome. The electroencephalograms, both initially and subsequently, ranged from normal to epileptiform and did not correlate with risk for further seizures in infancy or subsequent epilepsy. Benign familial neonatal seizures are a distinct clinical entity whose key features and favorable prognosis should be appreciated by all those caring for sick neonates. This recognition would result in an early diagnosis and conservative treatment.     

Bilateral akinetic seizures: A clinical and electroencephalographic description

Purpose: The main feature of akinetic seizures is the inhibition of voluntary movements without impairment of awareness. Most clinical information about akinetic seizures has been obtained from cortical electrical stimulation studies, whereas clinical and video–electroen‐cephalography (EEG) features have not been described thoroughly. We aimed to analyze clinical and EEG characteristics of bilateral akinetic seizures (BAS). Methods: Patients with BAS were retrospectively identified from 1,858 consecutive video‐EEG studies. All patients had ictal video‐EEG, comprehensive clinical evaluation, neuropsychological testing, and brain magnetic resonance imaging (MRI). Results: Ten patients (nine men) were identified; mean age was 22.5 years (range 0.3–71 years) at the time of epilepsy onset and 34.9 years (range 5–73 years) at the time of evaluation. BAS was the only seizure type in four patients. BAS consisted of sudden speech and motor arrest in eight patients, whereas in two patients seizures were characterized by abrupt freezing precipitated by gait initiation. Startle precipitated BAS in four patients. Magnetic resonance imaging (MRI) showed mesial frontal lobe lesions in six patients. Epileptiform activity was restricted to the frontal midline electrodes in all patients, with variable extension to frontal regions. In five patients, BAS were initially misdiagnosed as generalized seizures or nonepileptic events. Discussion: BAS should be considered in the differential diagnosis of patients reporting paroxysmal inability to move with preservation of awareness, bearing in mind that these seizures can occur spontaneously or be precipitated by startle. The diagnosis can be achieved with video‐EEG monitoring, showing stereotyped semiology and distinctive EEG abnormalities, and is often supported by the presence of lesions involving the frontal lobes.     

Ictal spitting: clinical and electroencephalographic features

PURPOSE: To identify clinical and EEG correlates of ictal spitting automatisms and to assess their reliability in indicating the hemisphere of seizure onset. METHODS: The epilepsy-monitoring database (1994-2002) of the Cleveland Clinic Foundation (CCF) was searched for patients with a definite history of ictal spitting. All available documents of the patients, particularly their original video and EEG data, were reviewed. RESULTS: Twelve (0.3%) of the approximately 4000 patients had a documented history of ictal spitting. In seven of them, 15 seizures with spitting automatisms were recorded. All of them started with an aura or arousal out of sleep. In six of the seven patients (12 of 15 seizures), EEG onset was clearly lateralized to the right, nondominant hemisphere. Spitting occurred at a median time of 21 s after EEG seizure onset. At that time, predominantly fast, high-amplitude theta (5-7 Hz) was seen in the hemisphere of seizure onset, maximum temporal. In all but one of the total 12 patients, the epileptogenic zone was in the temporal lobe. In nine of the 12 patients, seizure onset was in the non-language-dominant hemisphere. Two patients had seizures arising from the language-dominant hemisphere; in another patient, the side of the seizure onset could not be determined. CONCLUSIONS: Ictal spitting is an uncommon feature of epileptic seizures. Although the symptomatogenic area is probably outside the temporal lobe, it is most frequently seen in temporal lobe epilepsy of the right, nondominant hemisphere.     

癫痫伴肌阵挛-失张力发作的临床及脑电图特征分析

目的探讨癫痫伴肌阵挛-失张力发作(epilepsy with myoclonic-atonic seizures,EMAS)的临床及视频脑电图特点,以提高对该病的认识。方法对2017年12月-2018年12月吉林大学白求恩第一医院小儿神经科收治的6例EMAS患儿临床及脑电特征进行回顾性分析。结果 6例EMAS患儿中,男5例,女1例;发病前智力运动发育正常,影像学正常。发病年龄2岁2个月～6岁,确诊时间2个月～1年6个月。6例患儿至少有肌阵挛、肌阵挛-失张力、失张力发作、不典型失神发作中两种发作形式。其中4例在上述发作前或后出现强直-阵挛发作,1例在病程晚期有强直发作。6例患儿中5例背景活动正常; 1例背景活动偏慢。6例患儿的脑电图在清醒期及睡眠期均出现广泛性2～4 Hz棘慢波、多棘慢波不规则或节律性发放,睡眠期放电有时类似高度失律,均无局灶性发作。所有患儿影像学检查均正常。6例患儿均给予正规抗癫痫药物治疗,其中2例治疗反应良好,4例治疗无效后给予甲基强的松龙治疗,其中3例有效缓解,1例虽然没有发作仍有大量放电。结论 EMAS好发于学龄前期儿童,癫痫发作类型主要包括肌阵挛、失张力或肌阵挛-失张力发作,但不典型失神等,脑电图主要为广泛性棘慢波、多棘慢波发放,治疗以抗癫痫药物和激素治疗为主,预后相对较好。     

Clinical and electroencephalographic features of complex partial seizures in infants

The clinical and electroencephalographic (EEG) features were evaluated in a consecutive series of 50 infants with complex partial seizures. The age of onset of seizures showed a peak at age of 2 months. Significant development delay was seen in 60% of the infants. In 92% an underlying aetiological factor could be identified. Birth asphyxia was the commonest aetiological factor (30%). The seizure patterns were most frequently described as behavioural arrest, upward deviation of eyes, tonic posturing of the limbs, apnoea and cyanosis. Interictal EEG showed bilateral temporal lobe foci in 22%, unilateral foci in 78% and multiple foci in 46% of the cases. The response of the seizures to anticonvulsant drugs is discussed.     

Symptoms in focal sensory seizures. Clinical and electroencephalographic features.

PURPOSE: Aura is a brief subjective symptom that may represent the initial manifestation of a partial epileptic seizure with objective signs or constitute the entire epileptic attack (focal sensory seizure (FSS)). We studied the electro-clinical features of FSSs recorded in 28 patients. METHODS: Using long-term surface video-EEG recordings, we examined 28 patients (from a consecutive series of 64) with stereotyped FSSs and complex partial seizures (CPS) preceded in at least one instance by identical subjective manifestations (overall 255 FSSs and 39 CPS were recorded). FSSs were subdivided according to the type of sensation into somatosensory, visual or oculosensory, viscerosensory, experiential, cephalic and diffuse warm sensations. The EEG discharges accompanying FSSs were examined by two of the authors either blinded as to the type and timing of the seizure, or unblinded, i.e. after receiving complete clinical information including timing of the patient's warning. RESULTS: The ictal pattern accompanying FSSs was identified blind in 13 patients and unblind in 8 patients. In seven patients, the ictal discharge remained undetected. In the cases with recognizable ictal abnormalities, two main patterns could be distinguished, static and dynamic. FSSs whose ictal discharge could be recognized by blind EEG examination more frequently consisted of somatosensory and visual or oculosensory manifestations, and the discharge generally involved the centro-parieto-occipital regions. The ictal discharge of viscerosensory and experiential FSSs more easily remained undetected; when identified, it generally involved the fronto-temporal regions. CONCLUSIONS: FSSs are often accompanied by ictal abnormalities recognizable on surface EEG. A thorough knowledge of their EEG accompaniments may be a useful diagnostic aid in patients with partial epilepsy.     

Cefepime-induced encephalopathy: clinical and electroencephalographic features in seven patients

Cefepime, a fourth-generation cephalosporin, with large antibacterial spectrum, is a commonly used antibiotic for the treatment of serious hospital infections. Its security report is considered favourable. Recently, many cases of a severe and reversible cefepime-induced encephalopathy were described. In this paper, we report seven patients with reversible cefepime-induced encephalopathy, with a peculiar EEG pattern, characterized by semiperiodic diffuse triphasic waves. We discuss the EEG abnormalities found and their association with nonconvulsive status epilepticus.     

Generalized paroxysmal fast activity: electroencephalographic and clinical features

We reviewed electroencephalographic and clinical features of 20 patients with generalized paroxysmal fast activity (GPFA). All had seizure disorders and all but 3 had more than one seizure type. Mental retardation was present in 17 patients. In most cases no cause for the seizures could be found. GPFA occurred almost exclusively in sleep. Fifteen patients had clinical seizures associated with this activity; in 8 the seizures were tonic. Additional generalized epileptiform abnormalities were present in 18 patients, 10 of whom had slow spike and wave complexes. Only 4 patients had normal background activity.     

Amphetamine-associated seizures: clinical features and prognosis

Forty-four patients presenting with first-ever seizure within 24 h of illicit use of amphetamine or related analogs (amphetamine-associated seizures, AAS) were identified over 8 years. Patients with AAS were compared to control groups of other first-ever seizure patients (provoked n = 126 and unprovoked n = 401). Cumulative probability of recurrence was calculated using Kaplan-Meier analysis. Seizure recurrence and development of epilepsy were less likely in patients with AAS compared to provoked or unprovoked controls. Forty percent of patients with AAS had clinical risk factors for epilepsy, epileptiform abnormalities on electroencephalography (EEG), or an epileptogenic lesion on neuroimaging. Sleep deprivation was more frequently present in those with AAS. AAS likely relate to an intrinsic proconvulsant effect of these drugs combined with patient susceptibility and environmental factors.     

Clinical and electroencephalographic characteristics of children with febrile seizures plus

OBJECTIVES: Febrile seizures plus (FS+) are attracting attention for their corresponding genetic abnormalities, and are defined as febrile seizures (FS) continuing beyond 6 years of age (late FS) or those associated with afebrile seizures. We tried to elucidate their clinical and EEG characteristics as compared with those of children having only FS. SUBJECTS AND METHODS: We reviewed clinical records in a pediatric neurology clinic to identify 31 patients with FS+ (group FS+) and 51 with only FS (group FS). Their family history of seizures, clinical features and EEG findings were compared. RESULTS: A family history of seizures was noted in 14 patients (45.2%) of group FS+ and in 24 (47.1%) of group FS. In group FS+, 19 patients had late FS, 11 had afebrile seizures, and the remaining one had both types of seizures. Two patients had seizures induced by TV/video-game as well, and another suffered from absences. Epileptic EEG abnormalities, which included diffuse spike-waves and focal spikes, were noted in 13 patients (41.9%) of group FS+ and 12 (23.5%) of group FS. CONCLUSIONS: The clinical and EEG characteristics of the children having FS+ were diverse, without significant differences from those with FS except for the seizures types.     

Psychogenic seizures: clinical features and psychological analysis

Approximately 25-30% of patients referred to epilepsy centers for refractory epilepsy are found to have nonepileptic seizures (NES). In many cases psychological assessments are performed to evaluate for underlying psychiatric disorders. The authors analyzed the clinical features of 23 patients with NES and correlated the features with underlying psychological status. Thirteen of the twenty-three patients (56.5%) had motor manifestations and 10 presented with limpness and unresponsiveness. There was no significant difference in the occurrence of depression, anxiety, posttraumatic stress disorder, or malingering between patients with limpness and those with motor manifestations. However, only patients with motor manifestations had a history of sexual and physical abuse. Those with limp and unresponsive presentations were less likely to have a history of sexual and/or physical abuse. This finding may help treating physicians to choose optimum psychiatric treatment for patients with NES. Additional studies are needed to clarify the association.