Clinical correlates of occipital intermittent rhythmic delta activity (OIRDA) in children

PURPOSE: The clinical significance of occipital intermittent rhythmic delta activity (OIRDA) on the electroencephalogram has not been fully established. Recent studies suggest that this pattern occurs almost exclusively in children and is probably of epileptic origin in most cases. We sought to characterize the electrographic features and clinical correlates of occipital intermittent rhythmic delta activity. METHODS: A review of 697 consecutive pediatric electroencephalograms detected occipital intermittent rhythmic delta activity in 24 studies. Mean patient age was 7.96 years. RESULTS: Recent convulsions and absence seizures constituted the main indications for the study. Concomitant, independent epileptiform activity was noted in half of the cases. This activity was focal in all but one case. Conversely, in most cases of absence seizures, epileptiform activity intermixed with occipital intermittent rhythmic delta activity. Furthermore, the frequency of the occipital rhythmic discharges in studies of children with absences was generally faster (3-4 Hz) than in localization-related epilepsy (2-3 Hz). Most patients were awake when occipital intermittent rhythmic delta activity occurred. Chronic encephalopathy was seen in one child only. Analysis of neuroimaging studies in eight cases revealed no structural pathology associated with occipital intermittent rhythmic delta activity. CONCLUSIONS: Occipital intermittent rhythmic delta activity is probably an epileptiform pattern, although it is noted occasionally in encephalopathic children. Its electrographic characteristics appear to differ between localization-related epilepsy and primary generalized epilepsy, particularly absence seizures.     

The epileptiform significance of intermittent rhythmic delta activity in childhood.

Intermittent rhythmic delta activity is reported in various disorders and is classified as a nonspecific abnormal electroencephalographic pattern. We have investigated its clinical and electroencephalographic features in childhood. Intermittent rhythmic delta activity was identified in 54 children over a period of 48 months. Epilepsy was present in 81%, 4% had only a single generalized tonic-clonic seizure, and 15% had no seizures. Generalized seizures were more common than partial seizures (83% versus 13%; 4% were mixed). The largest group of patients had idiopathic epilepsy. Epileptiform features were present in 70%. No patient identified prospectively has had a space-occupying lesion. Intermittent rhythmic delta activity should be considered an epileptiform pattern in children, most commonly occurring as an interictal pattern in primary generalized epilepsy.     

Generalized absence seizures with 10-15 Hz fast discharges.

OBJECTIVE: To report clinical and EEG features in 5 adults with unusual, fast rhythmic discharges accompanying absence seizures. DESIGN AND METHODS: The 5 patients presented with uncontrolled seizures. All had EEG-video monitoring with recorded seizures. Video seizures were reviewed and ictal as well as interictal epileptiform activity was analyzed. The patients were followed up after appropriate therapy for a minimum of 6 months. RESULTS: There were 3 women and two men, with a mean age of 37 years (range: 23-59). Two patients had onset of absence seizures in childhood, one in adolescence and two after age 20. All patients also had generalized tonic-clonic seizures. Ictal EEG recordings showed generalized spike and wave (SW) discharges of variable dominant frequencies (2.5-6 Hz) and intermingled 10-15 Hz generalized rhythmic discharges which also occurred in isolation or as the dominant activity. Interictal recordings showed similar but shorter 2.5-6 Hz generalized SW discharges. The background activity was normal in 3 patients and mildly slow in two who had very frequent absence seizures during the recording period. Four patients became seizure free and one had 75% improvement on appropriate antiabsence therapy. CONCLUSIONS: The fast 10-15 Hz rhythmic discharges that we report appear to occur mostly in adult patients with absence, as well as, generalized tonic-clonic seizures. They can occur in isolation or be embedded in more typical SW discharges accompanying typical absence seizures. Their presence does not imply a poor prognosis for seizure control.     

Clinical correlation of occipital intermittent rhythmic delta activity.

Frontal intermittent rhythmic delta activity is associated with encephalopathy, and temporal intermittent delta activity is associated with epilepsy, but the importance of OIRDA (OIRDA) is less well defined.The authors reviewed retrospectively EEGs and medical records of 77 patients with OIRDA to determine whether they had epilepsy, acute encephalopathy, or another diagnosis. They compared the incidence of epilepsy in this population with a control group of 77 patients referred for EEG, matched for age, gender, and year of EEG. OIRDA was most commonly generalized, high amplitude, saw toothed, and reactive to eye opening, and with mean frequency of 2.89 +/- 0.50 Hz. Mean age was 8.1 +/- 4.5 years. Seventy-six of 77 patients were 相似文献   

Seizure-like phenomena and propofol: a systematic review

Data on seizure-like phenomena (SLP) in patients receiving propofol were systematically reviewed. Reports had to provide detailed information on SLP in individual patients who received propofol. Phenomena were classified according to the time point of their occurrence during anesthesia or sedation (induction, maintenance, emergence, delayed [>30 minutes after emergence]) and their clinical presentation (generalized tonic-clonic seizures, focal motor seizures, events presented as increased tone with twitching and rhythmic movements not perceived as generalized tonic-clonic seizures, opisthotonos, involuntary movements). In 70 patients without epilepsy, SLP happened during induction in 24 (34%), during maintenance in two (3%), during emergence in 28 (40%), and was delayed in 16 (23%). Most frequent clinical presentations of SLP were generalized tonic-clonic seizures in 30 patients (43%), events presented as increased tone with twitching and rhythmic movements not perceived as generalized tonic-clonic seizures in 20 (36%), and involuntary movements in 11 (16%). Of 11 patients with epilepsy, seven (64%) had generalized tonic-clonic seizure during emergence. Of all 81 patients, 26 (32%) only had an EEG, and 12 (15%) only a neurologic consultation. SLP may happen in patients with or without epilepsy receiving propofol. The time point of the occurrence of SLP suggests that a change in cerebral concentration of propofol may be causal. To confirm this hypothesis, to estimate the prevalence of propofol-related SLP, and to identify patients at risk, data of higher quality are needed.     

Electroencephalographic correlate of juvenile Huntington's disease

The spectrum of clinical disease in juvenile Huntington's disease differs from that seen in adults. Younger patients often present with seizures, dystonia and rigidity. The mechanism and type of seizures, timing of onset and electrographic features have not been well characterized in either adults or children. We describe the electroencephalographic findings observed in a young child with Huntington's disease who presented with motor regression and seizures. Recordings demonstrated bilateral posterior quadrant epileptiform discharges and occipital intermittent rhythmic delta activity, generally considered a nonspecific abnormality. These findings have not been reported in adult or juvenile Huntington's disease. Its presence in proximity to bilateral posterior spikes suggests that occipital intermittent rhythmic delta activity is an epileptiform abnormality, on a continuum with the posterior spike-and-wave discharges. Intermittent rhythmic delta activity can occur secondary to either the subcortical gray-matter disease or the associated seizure disorder. Huntington's disease should be considered in the differential diagnosis of young children who present with seizures and developmental regression.     

Idiopathic childhood occipital epilepsy of Gastaut: report of 12 patients

This study sought to present clinical and outcome data of patients with idiopathic childhood occipital epilepsy of Gastaut, to validate previously reported characteristics of this epilepsy. The study group was comprised of 12 affected children (three boys and nine girls), with a median age of onset at 10.3 years. Common ictal manifestations included elementary visual hallucinations (75.0%), blindness or blurring of vision (50.0%), headache (50.0%), and secondarily generalized tonic-clonic seizures (58.3%). Interictal electroencephalography revealed occipital spike-wave paroxysms reactive to eye closure and opening in all patients, accompanied by spike-wave activity in the extra-occipital areas in four (33.3%), and by generalized spike-wave discharges in two (16.7%). One patient exhibited the onset of occipital lobe seizures 1 year after manifesting absence epilepsy. Seizure remission occurred in 81.8% of cases, in half of which medication was discontinued by late adolescence. This study confirmed the previously delineated electroclinical features of epilepsy syndrome, with additional aspects including the frequent association of generalized tonic-clonic seizures and atypical evolution from childhood absence epilepsy.     

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.     

Absence Seizure with Generalized Rhythmic Delta Activity

In seven children, 5-11 years old, who presented with spells of staring or unresponsiveness, hyperventilation reproduced clinical spells that were accompanied on the EEG by generalized, rhythmic, delta activity. The discharges were usually of abrupt onset, extremely high amplitude, recurrent, and prolonged. One spontaneous staring spell, not provoked by hyperventilation, was also accompanied by generalized rhythmic delta activity. All patients had improvement or complete control of their spells and abolition of the EEG changes with ethosuximide or valproate. Delta activity during hyperventilation in children may not always be benign and may be associated with clinical absence seizures.     

Seamless Transition from Recurrent Absence Seizures to a Generalized Tonic-Clonic Convulsion with Focal Onset

Abstract: An unclassified seizure characterized by a seamless transition from a series of absence seizures to a generalized tonic-clonic convulsion with an apparent focal onset was described in a 15-year-old male patient. At the beginning of a lengthy series of recurrent absences, the ictal EEGs showed diffuse 3 Hz spike-and-wave rhythms with a frontal dominancy. However, the frequency of the spike-wave rhythms varied from 2 to 3 Hz 9 minutes after the onset. The intervals between each absence gradually shortened until the last absence abruptly developed into a versive movement of the head. While this transition occurred, the spike-wave complexes became less rhythmic and synchronized and an abrupt occurrence of repetitive spiking ensued in the right frontal region immediately prior to the left-sided head turning. The frontal focal spiking progressed to a high-voltage spiking activity over the entire right and then both hemispheres corresponding to the appearance of a generalized tonic-clonic convulsion. From the clinical and electrographic points of view, it was concluded that a secondarily generalized tonic-clonic convulsion with a focal motor onset took place immediately following the lengthy series of absences. There were no focal epileptic abnormalities in the interictal EEGs before and after this ictal event. One explanation of this rare ictal phenomenon is the possibility that a dormant frontal epileptogenic focus was transiently activated through the continuous bombardment of generalized spike-wave discharges.     

Reflex occipital lobe epilepsy.

Photosensitivity is a typical feature of photosensitive epilepsy which is usually considered a form of idiopathic generalized epilepsy. Partial seizures featuring visual symptoms are rarely reported in photosensitive epilepsy. In this study, we describe 13 neurologically normal patients in whom daytime seizures were always induced by television and began with elementary visual hallucinations, followed frequently by vomiting, headache and then secondary generalization. Three patients additionally reported nocturnal seizures, which have not been described in previous studies. Two of these latter patients had generalized tonic-clonic seizures, the other always awoke from sleep and could describe typical visual hallucinations at the beginning of the seizure. EEG features included normal background activity and occipital spikes or spike-waves in all but two patients. Eight patients also showed generalized epileptiform activity during intermittent photic stimulation. Seizure frequency was low in all. Apart from two patients, who refused treatment, all patients received antiepileptic drugs. Only one patient continued to have rare seizures after treatment; in the others seizure control was achieved with monotherapy. We conclude that reflex occipital lobe epilepsy is an idiopathic form of the benign partial epilepsies, which may overlap with one another.     

Angelman's syndrome: clinical and electroencephalographic findings

Angelman's syndrome is a rare genetic disorder characterized by developmental delay, craniofacial abnormalities, ataxia, paroxysmal laughter, and seizures. The diagnosis is suspected in infants who have the characteristic clinical features and electroencephalographic (EEG) abnormalities and is confirmed by the genetic identification of a maternally derived 15q11–13 deletion. We report on 3 patients with genetically confirmed Angelman's syndrome who had the characteristics clinical and EEG features. The EEGs demonstrated high-amplitude 2- to 3-Hz delta activity, with intermittent spike-and-slow-wave discharges maximal in the occipital region in 2 patients and generalized sharp-and-slow-wave discharges, occipital spikes, and electrographic status epilepticus during slow-wave sleep in the other patient. The findings of generalized high-amplitude delta slowing and occipital spike-and-wave discharges, facilitated by eye closure, in children with developmental delay and seizures suggest the diagnosis of Angelman's syndrome and should lead to genetic testing.     

Positron emission tomography in generalized seizures

We used 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) to study nine patients with clinical absence or generalized seizures. One patient had only absence seizures, two had only generalized tonic-clonic seizures, and six had both seizure types. Interictal scans in eight failed to reveal focal or lateralized hypometabolism. No apparent abnormalities were noted. Two patients had PET scans after isotope injection during hyperventilation-induced generalized spike-wave discharges. Diffusely increased metabolic rates were found in one compared with an interictal scan, and in another compared with control values. Another patient had FDG injected during absence status: EEG showed generalized spike-wave discharges (during which she was unresponsive) intermixed with slow activity accompanied by confusion. Metabolic rates were decreased, compared with the interictal scan, throughout both cortical and subcortical structures. Interictal PET did not detect specific anatomic regions responsible for absence seizure onset in any patient, but the results of the ictal scans did suggest that pathophysiologic differences exist between absence status and single absence attacks.     

EEG and Seizures in Children with Hemolytic–Uremic Syndrome

The EEG results of 11 children, ages 1-15 years, who presented with hemolytic-uremic syndrome complicated by seizures 3-10 days after the prodrome were studied. In four children who experienced generalized tonic-clonic seizures, the EEGs demonstrated diffuse delta slowing with no focality. All recovered without neurological deficit or a residual seizure disorder. Of seven children who experienced partial seizures, six had structural lesions on cranial computed tomography and residual focal neurological deficits with epilepsy. The EEGs in two patients revealed focal spikes and slowing consistent with the lateralization of the partial seizures, in four it was characterized by atypical "burst suppression," and in one showed epochs of high-amplitude delta slowing alternating with generalized suppression. Although episodic and generalized burst suppression is usually regarded as a grave prognostic indicator, all four subjects recovered.     

Symptomatology of Epileptic Seizures in the First Three Years of Life

PURPOSE: Few data are available concerning symptomatology of epileptic seizures in infants. METHODS: We reviewed 296 videotaped seizures from 76 patients aged 1-35 months (mean, 15.1 months) who underwent video-EEG monitoring at our institution from 1988 to 1998. Seizure symptomatology was first classified based on observable behavioral and motor manifestations and then correlated with ictal EEG. RESULTS: Four seizure types accounted for 81% of all seizures seen in this group: epileptic spasms (24%), clonic seizures (20%), tonic seizures (17%), and hypomotor seizures (20%; characterized by arrest or significant decrease of behavioral motor activity with indeterminate level of consciousness). The remaining seizures included small numbers of myoclonic, atonic, and versive seizures. All 12 focal motor seizures and all five versive seizures were associated with focal EEG seizure patterns, seen in the contralateral hemisphere in all but one patient with versive seizures. Generalized motor seizures (clinically generalized at onset) were accompanied either by focal (19 of 51; 37%) or generalized (32 of 51; 63%) EEG seizures. Hypomotor seizures also were associated with focal (14 of 20; 70%) or generalized (six of 20; 30%) EEG seizures. Four patients with generalized epileptic spasms had generalized EEG seizures in the setting of focal epilepsy based on neuroimaging, interictal EEG, and in two cases also on postresection seizure freedom. Seizure types not seen in this age group included auras, seizures with prominent automatisms (except in one case), and classic generalized tonic-clonic seizures. CONCLUSIONS: The repertoire of seizure manifestation in the first 3 years of life appears to be limited. In infants, focal motor seizures are reliably associated with focal EEG seizures in the contralateral hemisphere, whereas generalized motor and hypomotor clinical seizures may be either focal or generalized on EEG. Epileptic spasms may be seen in focal as well as generalized epilepsies. Video-EEG monitoring and neuroimaging may be critical for clarifying the focal or generalized nature of the epilepsy in infants.     

Consistent EEG Focalities Detected in Subjects with Primary Generalized Epilepsies Monitored for Two Decades

Summary: Purpose: To describe the evolution of interictal findings in serial EEGs from patients with primary generalized epilepsy.
Methods: A cohort of 89 subjects with various primary generalized epilepsies were reviewed. Thirty-one did not meet a priori criteria. Of the 58 patients analyzed, 12 had only absence seizures, 28 had absence seizures followed by one or more generalized tonic-clonic seizures, 9 had generalized tonicclonic seizures followed by absence and/or myoclonic seizures, and 9 had juvenile myoclonic epilepsy. Patients were followed for a mean of 16 years. An average of 39 EEGs were obtained on each patient.
Results : Thirty-two patients (56%) had focal features present in up to 65% of the EEGs in each of the patients. Accepted focalities were only those that were consistent in lateralization, location and, often, morphology across the span of the study. Focal findings were most often temporal or frontal.
Conclusions : Patients with typical primary generalized epilepsies show a high incidence of focal EEG features that cannot be explained on the basis of structural lesions, coincidental factors, or to artifacts of the selection criteria. Although the data do not allow a definitive explanation, possible mechanisms include associated focal cortical pathology such as microdysgenesis, and development over time of localized, self-sustaining hyperexcitability in low-threshold cortical structures subjected to repeated generalized spike-wave activity. Either hypothesis implies the participation and interaction of genetic, ontogenic, and environmental factors.     

Generalized‐onset seizures with secondary focal evolution

The international seizure classification recognizes that partial‐onset seizures can become secondarily generalized, but generalized‐onset seizures are expected to remain generalized. We report six patients who had recorded seizures with generalized onset, but subsequent evolution into a focal discharge. The clinical seizure onset was generalized absence or myoclonic, and the most common subsequent clinical pattern was prolonged behavioral arrest with mild automatisms, and then postictal confusion. The ictal discharge started with generalized spike‐and‐wave activity and then acquired a focal predominance. Interictal epileptiform activity was generalized. There were no focal magnetic resonance imaging abnormalities. Four patients were misdiagnosed with complex partial seizures. All patients were initially refractory, but three became seizure‐free and three improved after treatment with antiepileptic medications appropriate for absence or myoclonic seizures. Generalized‐onset seizures that acquire focal features are easily misdiagnosed as complex partial. These seizures have a more favorable response to medications effective against generalized absence and myoclonic seizures.     

"Benign" form of myoclonic epilepsy in children

Among 62 children with myoclonic epilepsy who had first seizures between 1 and 10 years, without clinical or radiological evidence of brain lesion, we selected the 16 patients who had exhibited several types of fits and had stopped having seizures for over two years. First seizures occurred between 18 months and 4 years, and they were generalized clonic, tonic-clonic or tonic. After a mean 3 months' period, patients started also to have absence and myoclonic fits. During the period with various types of seizures, that lasted a mean 10 months, patients were ataxic and hyperkinetic, and 11 of them suffered myoclonic absence status for several hours or days. The EEG showed a high voltage rhythmic slow-wave activity with spikes, differing from the slow spike wave tracing of the Lennox-Gastaut syndrome, and there was no photosensitivity. The mean duration of the epilepsy was 1 year and 4 months and the last seizures were convulsive, occurring mainly during sleep. The clinical and EEG pattern, the high familial incidence are shared by the Doose syndrome, of which the present series seems to be a subgroup, as are other well-defined syndromes: benign and severe myoclonic epilepsies of infancy.     

颞叶外节细胞胶质瘤继发癫痫的手术治疗研究

目的 研究颞叶外节细胞胶质瘤(GG)继发癫痫的临床特点和手术治疗方式及疗效.方法 回顾性分析手术治疗并经病理证实的20例颞叶外节细胞胶质瘤继发癫痫患者的临床资料.术后癫痫控制效果按改良Engel分级标准评定;分析患者的发作表现、病变部位、影像学特点、病理改变、手术方式及病变切除程度与癫痫控制的关系.结果 节细胞胶质瘤位...