Electrolyte therapy for refractory seizures in familial dysautonomia

PURPOSE: Video-EEG in a family of three patients with slow development and familial dysautonomia demonstrated absence seizures associated with 3-Hz generalized spike-and-wave discharges. The seizures were refractory to antiepileptic drugs (AEDs). METHODS: Treatment was given with rice-based cereal electrolyte oral solution. RESULTS: Treatment induced seizure freedom and normalization of EEG in all three patients. Repeated video-EEG monitoring with discontinuation of AEDs and maintenance of the oral hydration therapy was associated with recurrence of epileptic activity. All three patients have remained seizure free (approximately 1 year) with a combination of topiramate and electrolytic therapy. CONCLUSIONS: Rice-based oral electrolyte hydration therapy may play a role in prevention and control of seizures in patients with familial dysautonomia.     

Generalized onset seizures with focal evolution (GOFE) — A unique seizure type in the setting of generalized epilepsy

PurposeWe report clinical and electrographic features of generalized onset seizures with focal evolution (GOFE) and present arguments for the inclusion of this seizure type in the seizure classification.MethodsThe adult and pediatric Epilepsy Monitoring Unit databases at Vanderbilt Medical Center and Children's Hospital were screened to identify generalized onset seizures with focal evolution. We reviewed medical records for epilepsy characteristics, epilepsy risk factors, MRI abnormalities, neurologic examination, antiepileptic medications before and after diagnosis, and response to medications. We also reviewed ictal and interictal EEG tracings, as well as video-recorded semiology.ResultsTen patients were identified, 7 males and 3 females. All of the patients developed generalized epilepsy in childhood or adolescence (ages 3–15 years). Generalized onset seizures with focal evolution developed years after onset in 9 patients, with a semiology concerning for focal seizures or nonepileptic events. Ictal discharges had a generalized onset on EEG, described as either generalized spike-and-wave and/or polyspike-and-wave discharges, or generalized fast activity. This electrographic activity then evolved to focal rhythmic activity most commonly localized to one temporal or frontal region; five patients had multiple seizures evolving to focal activity in different regions of both hemispheres. The predominant interictal epileptiform activity included generalized spike-and-wave and/or polyspike-and-wave discharges in all patients. Taking into consideration all clinical and EEG data, six patients were classified with genetic (idiopathic) generalized epilepsy, and four were classified with structural/metabolic (symptomatic) generalized epilepsy. All of the patients had modifications to their medications following discharge, with three becoming seizure-free and five responding with > 50% reduction in seizure frequency.ConclusionGeneralized onset seizures may occasionally have focal evolution with semiology suggestive of focal seizures, leading to a misdiagnosis of focal onset. This unique seizure type may occur with genetic as well as structural/metabolic forms of epilepsy. The identification of this seizure type may help clinicians choose appropriate medications, avoiding narrow spectrum agents known to aggravate generalized onset seizures.     

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.     

Absence seizures aggravated by valproic acid

PURPOSE: To report on pediatric patients with absence epilepsy who experienced absence seizure aggravation while receiving valproic acid (VPA). METHODS: The charts of all children from four pediatric epilepsy clinics receiving VPA for absence epilepsy were reviewed. Patients were evaluated and followed up between 1994 and 2000. RESULTS: Eight cases (six boys) of absence seizure aggravation were detected. Mean age at seizure onset was 5.8 years (range, 3-12 years). Six patients had simple absence seizures, one had myoclonic absences, and one had absences with automatisms. The electroencephalogram in all cases depicted generalized 3-Hz spike-and-wave activities. All eight patients experienced an increase in the frequency of absence seizures within days of VPA introduction. Dose increments resulted in further seizure aggravation. Serum levels of VPA were within therapeutic range in all patients. No case was attributed to VPA-induced encephalopathy. All patients improved on VPA discontinuation. In five children, VPA was reintroduced, resulting in further seizure aggravation. CONCLUSIONS: VPA can occasionally provoke absence seizure aggravation in patients with absence epilepsy.     

Does Hyperventilation Elicit Epileptic Seizures?

PURPOSE: Voluntary hyperventilation has been advocated for many decades as an "activating" procedure to provoke clinical seizures and epileptiform discharges in subjects with suspected epilepsy who undergo standard EEG recordings. This study was undertaken to determine the effects of hyperventilation in patients with proven epilepsy. METHODS: We examined the records of 433 consecutive patients with proven epilepsy, as documented by long-term video-EEG studies. The patients underwent 5 min of voluntary hyperventilation during standard EEG recordings. All EEGs were interpreted by board-certified electroencephalographers. The patients ranged in age from 10 to 64 years; 384 (88.7%) had localization-related and 48 (11.3%) generalized epilepsy syndromes. RESULTS: Hyperventilation was associated with a clinical seizure in two (0.46%) of the subjects (partial seizures in both cases). Interictal epileptiform discharges were interpreted as showing in increase in frequency during hyperventilation in 19 (4.4%) patients, when compared with the baseline EEG. CONCLUSIONS: Voluntary hyperventilation in patients with unequivocal epilepsy is rarely associated with either clinical seizures or an increase in frequency of epileptiform discharges.     

Can absence status epilepticus be of frontal lobe origin?

Five women with an unclassifiable nonconvulsive status epilepticus (NCSE) characterized by young age at onset, prolonged confusions, focal motor seizures, and both generalized spike-and-wave discharges and focal epileptic discharges on the EEG were studied with video-EEG monitoring. Electrographically, the NCSE originated from the left frontal lobe in 4 patients, and the left hemisphere with multifocal seizure discharges in 1 patient. Focal motor seizures seemed to originate from the left hemisphere in all 5 patients, particularly from its anterior part in 3 of them. Results show that the NCSE is complex partial status epilepticus of frontal lobe origin electroclinically mimicking absence status epilepticus once it reaches a full-blown phase.     

Idiopathic generalized epilepsies with versive or circling seizures

OBJECTIVES: To describe the electroclinical features of the idiopathic generalized epilepsies (IGEs) with versive or circling seizures. METHODS: Sixteen patients with versive or circling seizures and interictal electroclinical features of IGE were studied. Patients with insufficient clinical or imaging data, with a follow-up period less than 1 year or with partial seizures in addition to the versive or circling ones were excluded from the study. All patients underwent full interictal clinical and neurophysiological studies. The EEG patterns of 13 versive or circling seizures from 4 patients were also analyzed. RESULTS: A specific IGE syndrome was recognized in 9 out of the 16 patients (56%). More specific, 1 patient had childhood absence epilepsy (CAE), 4 had juvenile absence epilepsy (JAE), and 4 had juvenile myoclonic epilepsy (JME). No specific IGE syndrome was recognizable in the remaining 7 patients (44%). These 7 patients had a juvenile epileptic syndrome (mean age at onset of seizures was 15.7 years) characterized by versive or circling seizures followed or not by generalized tonic-clonic fits. Three main EEG patterns were identified during versive or circling seizures: 1) generalized spike-and-wave discharges at 3-4 cps; 2) generalized polyspike-and-wave discharges at 1 to 2.5 cps beginning with generalized fast activity at 12-14 cps, and 3) generalized spike-and-wave discharges at 3-4 cps intermingled with fast activity at 12-14 cps. Most patients had good response to treatment on a single drug regimen (mainly valproic acid). CONCLUSIONS: Versive or circling seizures may occur in the context of an IGE. Although many individuals share the features of different IGE syndromes including CAE, JAE and JME, a consistent number of patients, who show circling or versive seizures solely, remain without a specific syndromic diagnosis. When occurring in the context of IGE, circling or versive seizures do not worsen the prognosis.     

Ictal single photon emission computed tomography in absence seizures: apparent implication of different neuronal mechanisms

Absence seizures represent a complex group of epilepsy, characterized by lapse of consciousness with staring. Bilateral, synchronous, and symmetric bursts of 3-Hz spike-and-wave discharges are observed on the electroencephalogram, whereas interictal background activity is normal. This kind of epilepsy has to be differentiated from other generalized epilepsies such as juvenile absence epilepsy and juvenile myoclonic epilepsy. Moreover, absence seizures, together with generalized spike-and-wave discharges, may coexist with other types of epilepsy such as frontal lobe epilepsy, temporal lobe epilepsy, benign epilepsy with centrotemporal spikes, and childhood epilepsy with occipital paroxysms. We have carried out ictal single photon emission computed tomography (SPECT) in 10 patients with clinical evidence of absence seizures with the aim to better understand and to distinguish this kind of seizure as primarily or secondarily generalized to a specific area and to obtain more information on the neuronal mechanisms involved in the different types of seizures, usually not identifiable at the first appearance. During the long follow-up period (9 months to 14 years), 7 of the 10 examined patients underwent interictal SPECT when they became seizure free. Our data permitted, in two patients, the diagnosis of childhood absence seizures; in three patients, they suggested the possibility of later appearance of other seizure types, on the basis of focal hyperperfusion indicating a possible focal firing. In three of the examined patients, the diagnosis of idiopathic localization-related epilepsies mimicking childhood absence seizures could be performed. In the last two patients, the hypothesis of a coexistence of absences with partial and generalized seizures was considered. From our results, it can be presumed that ictal SPECT findings may contribute to the physiopathologic classification of the different types of epilepsies. Moreover, anticonvulsant treatment more appropriate to the different forms of seizures can be used.     

A clinical and electroencephalographic study of juvenile myoclonic epilepsy: its pathophysiological considerations based on the findings obtained from neuropsychological EEG activation

Juvenile myoclonic epilepsy (JME) is one of the idiopathic generalized epilepsies with age-related onset. Myoclonic seizures of JME often occur on awakening. On the other hand, patients with reflex epilepsy, whose seizures are particularly induced by activating higher cognitive functions, are thought to belong to the same category. As to the seizure precipitating factors of these patients with JME, however, few studies have ever been made. In this study, clinical and electroencephalographic findings of 25 patients with JME, on whom "neuropsychological EEG activation" (NPA) was performed, were carefully analyzed. After a routine EEG examination, NPA was carried out; this is a special method for EEG activation that requires the patients to speak, read, write, calculate and perform constructive acts (drawing figures and doing a block design test); in addition to EEG recording, simultaneous video-EEG monitoring was also made during these tasks. In the patients with JME, the sex ratio was about equal (11 males and 14 females). Eight patients (32%) had a family history of epilepsy but none of the patients had a positive past history responsible for epilepsy. The age at the onset of myoclonic seizures ranged between 10 and 20 years (mean: 15.3 years). A total of 18 patients (72%) had another type of seizures; 3 had absence seizures, 9 had generalized tonic-clonic seizures and 6 had both absence and generalized tonic-clonic seizures. Myoclonic seizures often occurred in the morning on awakening in 17 (68%) of 25 patients. As for EEG findings (all but one patient were receiving antiepileptic drugs when EEG was examined), paroxysmal discharges in a resting state were found in 12 patients (48%); most of them were either very brief generalized spike and wave complexes or bursts of generalized 3-5 Hz spike and wave complexes lasting for 1-3 seconds; generalized multiple spikes were observed only in 5 patients. These clinical and EEG findings were in accordance with those reported previously. Careful investigation of the histories obtained from the patients disclosed the following: Myoclonic seizures were induced (1) by mental activity associated with the use of hands in 20 patients (80%), (2) in a situation with psychic tension in 12 (48%), and (3) by decision making in 3 (12%). Interestingly, these findings could be confirmed by NPA with simultaneous video-EEG monitoring.(ABSTRACT TRUNCATED AT 400 WORDS)     

Focal semiologic and electroencephalographic features in patients with juvenile myoclonic epilepsy

PURPOSE: A few reports have described focal electroencephalographic or clinical features or both of juvenile myoclonic epilepsy (JME), but without video-EEG documentation. We examined focal clinical and EEG features in patients with JME who underwent video-EEG monitoring. METHODS: Twenty-six patients (nine males and 17 females) who had seizures recorded during video-EEG monitoring were included. Age at seizure onset was 0 to 22 years (mean, 12.3 years), and age at monitoring was 12 to 44 years (mean, 26.5 years). In one patient with left parietooccipital epilepsy, primary generalized tonic-clonic seizures developed after resection of the parietal tumor. Two patients had both temporal lobe epilepsy and JME. Videotaped seizures in each patient were analyzed. Interictal and ictal EEG also were analyzed for any focal features. RESULTS: Focal semiologic features were observed in 12 (46%) of 26 patients. Six patients had focal myoclonic seizures, and two had Figure 4 sign: one with version to the left, and another had left version followed by Figure 4 sign, and left arm clonic seizure. Their ictal EEGs were generalized at onset but with a lateralized evolution over the right hemisphere. The patient who had both JME and left parietooccipital epilepsy, right arm clonic seizure, and Figure 4 sign was seen during a generalized EEG seizure. Interictally, one patient had temporal sharp waves, and another had run of spikes in the right frontal region. CONCLUSIONS: Fourteen (54%) of 26 patients with JME exhibited focal semiologic or electroencephalographic features or both. Video-EEG was essential in reaching a correct diagnosis and choosing an appropriate antiepileptic drug regimen.     

Idiopathic generalized epilepsy presenting with hemiconvulsive seizures

PURPOSE: Unilateral seizures, or hemiconvulsive attacks, are motor seizures with tonic and/or clonic phenomena that involve only one side of the body. METHODS: We describe three adolescents who presented with hemiconvulsive seizures and were found to have 3-cps generalized spike-and-wave discharges on ictal and/or interictal EEG. All had normal neuroimaging studies. Two patients had been previously treated with carbamazepine, which led to a partial response in one patient. RESULTS: All three patients, however, are now seizure free on either sodium valproate or a combination of sodium valproate and lamotrigine. We believe the electroclinical diagnosis is that of idiopathic generalized epilepsy. CONCLUSIONS: Idiopathic generalized epilepsy presenting with hemiconvulsive seizures has not, to our knowledge, been previously described. However, the correct diagnosis of an idiopathic generalized seizure disorder, as opposed to a partial seizure disorder, has important treatment implications. The possible mechanism of hemiconvulsive seizures in idiopathic generalized epilepsy is discussed.     

Generalized paroxysmal fast activity in EEG: An unrecognized finding in genetic generalized epilepsy

ObjectiveTo study generalized paroxysmal fast activity (GPFA) in patients with genetic generalized epilepsy (GGE).IntroductionGPFA is an electroencephalographic (EEG) finding in patients with symptomatic generalized epilepsy consisting of 15-25 Hz bifrontally predominant generalized fast activity seen predominantly in sleep. Historically GPFA is linked to epileptic encephalopathy with drug resistant epilepsy and intellectual disability. However, GPFA has been rarely described as an atypical finding in patients with GGE without negative prognostic implication. We report cognitive profile and seizure characteristics in seven patients with GGE and GPFA.MethodsThe Vanderbilt EMU and EEG reports were searched for the keywords “idiopathic generalized epilepsy”, “GPFA”and “generalized spike and wave discharges (GSWD)”. We reviewed the EEG tracings and the electronic medical records of patients thus identified. The seizure type, frequency, neurological work-up, clinical profile and imaging data were recorded.ResultsAwake and sleep states were captured on EEGs of all patients. On EEG tracing review six patients were confirmed to have GSWD and GPFA; one patient had GPFA but no GSWD. All patients had normal cognitive function. Four had a normal brain MRI and one a normal head CT (two were never imaged). None of the patients had tonic seizures. The main seizure type was generalized tonic-clonic seizures (GTCS) in five patients, absence in two. Age at onset of epilepsy ranged from 4 to 24 years. The mean GTC seizure frequency at the time of EEG was 3; two patients were seizure free on two antiepileptic drugs (AEDs).ConclusionsGPFA can be an unrecognized electrographic finding in patients with genetic generalized epilepsy. While GPFA remains an important diagnostic EEG feature for epileptic encephalopathy (Lennox-Gastaut syndrome) it is not specific for this diagnosis. Thus, GPFA may have a spectrum of variable phenotypic expression. The finding of GPFA is not necessarily indicative of unfavorable outcome.     

Benign versive or circling epilepsy with bilateral 3-cps spike-and-wave discharges in late childhood

Twenty-eight of 920 patients seen between the ages of 8 and 20 years for a first seizure manifested versive or circling events associated with bilateral rhythmic 3-cps spike-and-wave discharges. In 21 (75%), the seizures appeared between the ages of 10 and 14 years. Paroxysmal adversion or gyration was always toward the same side in a given patient. "Break of contact" during the ictus was found in 70% of the seizures, and secondary generalization occurred in half. Ictal electroencephalographic recordings failed to show a focal hemispheric origin. Additional features were: absence of prior major diseases; normal results of neurological and neuroradiological examinations; excellent response to anticonvulsant treatment with sodium valproate and/or phenobarbital; frequent family history of epileptic seizures (25% of patients); and association with generalized seizures (57.2% of patients). We conclude that versive or circling epilepsy with bilateral rhythmic 3-cps spike-and-wave discharges represents a benign form of primary generalized epilepsy in late childhood.     

Hypomotor seizures in infants and children

PURPOSE: Hypomotor seizures (characterized by diminished behavioral activity with indeterminate level of consciousness) have been identified as an important seizure type in infants. Our goal was to investigate further the clinical and EEG features of hypomotor seizures. METHODS: We retrospectively reviewed 110 hypomotor seizures from 34 patients recorded with video-EEG. RESULTS: Twenty-seven (79%) patients were younger than 48 months, and seven (21%) were aged 4 to 15 years. Seventy-one (64%) seizures had regional or lateralized EEG onset, arising predominantly from temporal or parietal lobe regions. The other 39 (35%) seizures had generalized onset, usually with abrupt onset of diffuse rhythmic high-amplitude theta activity or diffuse electrodecrement and only rarely (two patients) with slow spike-wave complexes or 3-Hz spike-wave complexes. Hypomotor seizures with generalized EEG onset were significantly shorter than those with regional or lateralized onset (p = 0.01, GEE model). Unsustained head or eye movements and subtle mouth automatisms were commonly seen in hypomotor seizures with either focal or generalized onset. Seventeen percent of hypomotor seizures with focal onset evolved to include version of head and eyes or jerking of one arm, whereas 2% of generalized hypomotor seizures evolved to a cluster of spasms. CONCLUSIONS: Hypomotor seizures may be either focal or generalized. Regional EEG onsets were most often temporal or parietal, suggesting that focal hypomotor seizures may be a bland form of "complex partial" seizures with no or minimal automatisms, seen predominantly in infants. Generalized hypomotor seizures were rarely associated with an ictal pattern of generalized spike-wave complexes, suggesting a different mechanism from absence seizures seen later in life.     

Myoclonic Absence-Like Seizures and Chromosome Abnormality Syndromes

Summary: Purpose: We explored the relationship between myoclonic absence seizures (MAS) and underlying chromosome disorders.
Methods: Among 14 patients with MAS observed in three centers, 5 had typical cryptogenic myoclonic absence epilepsy (MAE), 2 had MAS associated with other seizure types (1 with signs of a neuronal migration abnormality and 1 with signs of a metabolic disorder), and 7 had MAS, with or without other seizure types, complicating a chromosome abnormality syndrome-2 with trisomy 12p, 4 with Angelman syndrome, and 1 with inv dup (15).
Results: In the 7 patients with chromosomopathy, MAS appeared at a mean age of 2.9 years (range 4 months to 6 years 6 months), had a duration of 4–20 s, and were accompanied by reduced awareness and rhythmic myoclonic jerks involving proximal limb muscles. Ictal EEG showed 2- to 3-Hz generalized spike-and-wave discharges.
Conclusions: In these patients, MAS differed slightly from those of typical MAE: age of onset was earlier, absences were of shorter duration, and no clear increase in muscular tone was noted. Abnormal expression of genes codifying for the subfamily of K⁺ channels and for γ-aminobutyric acid-3 subunit receptors (GABRBS), both located in the chromosome segments involved in the chromosomopathies presented by our patients, could be responsible for the same generalized seizure type. Chromosome analysis should be performed in patients with mental retardation and MAS, especially when the ictal pattern does not completely overlap that observed in MAE.     

Ambulatory cassette EEG in absence epilepsy

Absence epilepsy is associated with bursts of generalized 3 Hz spike-and-wave activity. Even very brief bursts may cause an alteration in the level of consciousness and impaired reaction time. We studied 15 patients, ages 5-16 years, with absence epilepsy using 8 channel ambulatory cassette electroencephalography. All patients demonstrated multiple paroxysms of generalized spike-and-wave discharges, most of which were asymptomatic. Because patients with absence epilepsy may not accurately report seizure frequency, periodic long-term monitoring may be required which is accomplished easily on an outpatient basis with ambulatory cassette electroencephalography.     

Myoclonic-astatic epilepsy of early childhood--clinical and EEG analysis of myoclonic-astatic seizures, and discussions on the nosology of the syndrome.

Purpose: The aim of this study is to elucidate the clinical and neurophysiological characteristics of the myoclonic, myoclonic-astatic, or astatic seizures in patients with myoclonic-astatic epilepsy (MAE) of early childhood, and to discuss on the nosology of this unique epileptic syndrome.Subjects: The subjects included 30 patients, who fulfilled the following modified International League Against Epilepsy (ILAE) criteria for MAE, and whose main seizures were captured by video-electroencephalographs (EEG) or polygraphs. The modified ILAE criteria includes: (1) normal development before onset of epilepsy and absence of organic cerebral abnormalities; (2) onset of myoclonic, myoclonic-astatic or astatic seizures between 7 months and 6 years of age; (3) presence of generalized spike- or polyspike-wave EEG discharges at 2-3 Hz, without focal spike discharges; and (4) exclusion of severe and benign myoclonic epilepsy (SME, BME) in infants and cryptogenic Lennox-Gastaut syndrome based on the ILAE definitions.Results: The seizures were investigated precisely by video-EEG (n=5), polygraph (n=2), and video-polygraph (n=23), which identified myoclonic seizures in 16 cases (myoclonic group), atonic seizures, with or without preceding minor myoclonus, in 11 cases (atonic group), and myoclonic-atonic seizures in three cases. All patients had a history of drop attacks, apart from ten patients with myoclonic seizures. Myoclonic seizures, involving mainly the axial muscles were classified into those with mild intensity not sufficient to cause the patients to fall (n=10) and those that are stronger and sufficient to cause astatic falling due to flexion of the waist or extension of the trunk (n=6). Patients in the atonic group fell straight downward, landed on their buttocks, and recovered immediately. Analysis of the ictal EEGs showed that all attacks corresponded to the generalized spike or polyspikes-and-wave complexes. In the atonic form, the spike-and-wave morphology was characterized by a positive-negative-deep-positive wave followed by a large negative slow wave. In two patients, the intensity of the atonia appeared to correspond to the depth of the positive component of the spike-and-wave complexes. We did not detect any significant differences in the clinical and EEG features and prognosis, between the atonic and myoclonic groups.Conclusions: Although the determination of exact seizure type is a prerequisite for diagnosing an epileptic syndrome, the strict differentiation of seizure type into either a myoclonic or atonic form, does not appear to have a significant impact on the outcome or in delineating this unique epileptic syndrome. At present, we consider it better to follow the current International Classification of Epileptic Syndromes and Epilepsies until a more appropriate system than the clinico-electrical approach for classifying patients with MAE is available.     

Intracranial EEG Substrates of Scalp Ictal Patterns from Temporal Lobe Foci

Summary: Purpose: To determine the intracranial EEG features responsible for producing the various ictal scalp rhythms, which we previously identified in a new EEG classification for temporal lobe seizures. Methods: In 24 patients, we analyzed simultaneous intracranial and surface ictal EEG recordings (64 total channels) obtained from a combination of intracerebral depth, subd-ural strip, and scalp electrodes. Results: Four of four patients with Type 1 scalp seizure patterns had mesial temporal seizure onsets. However, discharges confined to the hippocampus produced no scalp EEG rhythms. The regular 5- to 9-Hz subtemporal and temporal EEG pattern of Type 1a seizures required the synchronous recruitment of adjacent inferolateral temporal neocortex. Seizure discharges confined to the mesiobasal temporal cortex produced a vertex dominant rhythm (Type 1c) due to the net vertical orientation of dipolar sources located there. Ten of 13 patients with Type 2 seizures had inferolateral or lateral, temporal neocortical seizure onsets. Initial cerebral ictal activity was typically a focal or regional, low voltage, fast rhythm (20–40 Hz) that was often associated with widespread background flattening. Only an attenuation of normal rhythms was reflected in scalp electrodes. Irregular 2- to 4-Hz cortical ictal rhythms that commonly followed resulted in a comparably slow and irregular scalp EEG pattern (Type 2a). Type 2C seizures showed regional, periodic, 1– to 4-Hz sharp waves following intracranial seizure onset. Seven patients had Type 3 scalp seizures, which were characterized by diffuse slowing or attenuation of background scalp EEG activity. This resulted when seizure activity was confined to the hippocampus, when there was rapid seizure propagation to the contralateral temporal lobe, or when cortical ictal activity failed to achieve widespread synchrony. Conclusions: Type 1, 2, and 3 scalp EEG patterns of temporal lobe seizures are not a reflection of cortical activity at seizure onset. Differences in the subsequent development, propagation, and synchrony of cortical ictal discharges produce the characteristic scalp EEG rhythms.     

Hyperorality in epileptic seizures: periictal incomplete Klüver-Bucy syndrome

PURPOSE: To analyze systematically hyperorality associated with epileptic seizures and its relation to the localization of epileptic activity. METHODS: To identify patients with periictal hyperorality, we reviewed video-recordings of 269 patients (aged 6-59 years) who had consecutively undergone presurgical evaluations including ictal video-EEG recordings and high-resolution magnetic resonance imaging (MRI) and had had epilepsy surgery because of intractable frontal (FLE) or temporal lobe epilepsy (TLE). Periictal hyperorality was defined if patients put or unambiguously intended to put nonfood items into their mouths during or after at least one of the reviewed seizures. For the further analysis, we included only patients with periictal hyperorality. We reviewed their medical records and reexamined their ictal video-EEG recordings. RESULTS: We identified eight patients (six women) aged 8-59 years who had hyperorality during or after seizures. Seven patients had TLE, and one patient had frontal lobe epilepsy (FLE). Three of these patients underwent right-sided surgery, whereas five patients had surgery on the left. Three patients exhibited ictal and five showed postictal hyperorality. Interictal EEG suggested bilateral interictal epileptiform discharges (IEDs) in three patients; in two other patients, no IEDs were detected. Ictal EEG suggested bilateral involvement in six cases. Patients with unilateral epileptiform activity had left TLE. CONCLUSIONS: Periictal hyperorality is a rare phenomenon occurring in 3% of the investigated epilepsy population. We suggest that periictal hyperorality is an ictal-postictal mental disturbance, an incomplete Klüver-Bucy syndrome. In most patients, bilateral seizure activity plays an important role in the pathomechanism, but it would appear that left-sided epileptic activity without contralateral involvement also can cause periictal hyperorality.