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[A case of bilateral perisylvian polymicrogyria with epileptic attacks of focal inhibitory seizure followed by complex partial seizure].

A 20 year-old woman began to have epileptic attacks of focal inhibitory seizure with paralysis and hypesthesia of her left or right upper limb followed by complex partial seizure several times a week since age 19. She was born by breech presentation and umbilical cord was coiling around her neck at birth. EEG showed spike foci on P 3, O1 and T5. Cerebral MRI with 4 mm-section inversion recovery image revealed bilaterally symmetrical polymicrogyria involving the posterior portion of sylvian fissure extending posteriorly to the inner cortex of the postcentral gyrus and the supramarginal gyrus, and she was diagnosed as bilateral perisylvian polymicrogyria. 99mTc-ECD SPECT showed increased cerebral blood flow over the bilateral polymicrogyric lesion. On cerebral MRA, bilateral middle cerebral arteries were narrow all way through. Epileptic attacks were controlled with zonisamide and carbamazepin. This is a rare case of bilateral perisylvian polymicrogyria because epileptic attacks were the only manifestation and the patient showed neither mental retardation nor neurological abnormality.

Adult↗

[Topiramate--a new antiepileptic agents].

BACKGROUND: Topiramate is a novel antiepileptic drug that was licensed in Norway in 1997 as adjunctive treatment for patients with partial seizures. Metaanalysis of randomized controlled studies suggest that topiramate may be the most potent of the new antiepileptic drugs and have a favourable pharmacokinetic profile. At the National Center for Epilepsy we have used topiramate since 1990. We present our clinical experience with the drug. MATERIAL AND METHODS: We have, retrospectively, assessed 114 adult patients, mainly with intractable partial seizures, who received topiramate as add-on treatment. Average follow-up was 2.3 years. RESULTS: Four patients (3.5%) became seizure free. The overall seizure frequency was reduced by > 50% in 56 patients (49%). Adverse effects were observed in 82 patients (72%); in 54 patients (47%) the drug had to be withdrawn due to unacceptable side effects and/or lack of clinical effect. The most frequent side effects were weight loss, fatigue, behavioural and cognitive problems. INTERPRETATION: Our results corroborate an impression of topiramate being an effective drug used as adjuntive therapy in patients with refractory epilepsy. Side effects can be a problem, but a low starting dose, a slow dose escalation, and topiramate used alone may reduce this problem.

Adolescent↗

Status epilepticus and tiagabine therapy: review of safety data and epidemiologic comparisons.

PURPOSE: To determine whether an increased risk of status epilepticus (SE) and complex partial status epilepticus (CPSE) is associated with tiagabine (TGB) therapy. METHODS: Thirteen cases in which an EEG, performed on patients with altered mental status taking TGB, was reported to demonstrate spike-and-wave discharges (SWDs) were reviewed by a panel of experts. In addition, all cases of suspected SE from TGB clinical trials were reviewed. The occurrence of SE in four epidemiologic cohorts from Rochester, Minnesota, Turku, Finland, Bronx, New York, and New Haven, Connecticut was analyzed as an external comparison. RESULTS: Review of the 13 cases with reported SWDs found that the majority had had prior EEGs with similar findings, and only three were thought to have electrographic evidence of SE. There was no difference in the frequency of SE or CPSE in the placebo-controlled clinical trials between the TGB-treated (1.0% SE, 0.8% CPSE) and placebo-treated (1.5% SE, 1.5% CPSE) groups. The 5% frequency of SE and 3% frequency of CPSE in the TGB-treated patients in the long-term safety studies, which included 2,248 patients, were very similar to the rates of occurrence of SE and CPSE in the four external cohorts. The major risk factor for the occurrence of SE and CPSE in all groups was a prior episode of SE (p < 0.0001). CONCLUSIONS: Over a 3-year period, SE will occur in 5-10% of patients with epilepsy not in remission. At highest risk are those who have had a prior episode of SE. Treatment with TGB in recommended doses does not increase the risk of SE in patients with partial seizures.

Adolescent↗

Pattern visual evoked potentials recorded from human occipital cortex with chronic subdural electrodes.

Pattern evoked potentials to full- and partial-field stimulation were recorded simultaneously from scalp electrodes and from subdural electrodes located over the temporal and occipital cortex, including electrodes placed over or close to the lower lip of the calcarine fissure. High-amplitude pattern evoked potentials were recorded exclusively from electrodes localized in the vicinity of the calcarine fissure and showed a positive-negative deflection in phase with surface recordings, followed by a second negative peak phase reversed with respect to the major surface positive peak ("P100"). The findings suggest that the initial component is an expression of the afferent volley and that the second component (equivalent of the surface "P100") is most probably generated as a dipole strictly localized to the visual cortex in close proximity of the calcarine fissure (area 17 and/or area 18).

Adult↗

The ontogeny of hippocampal local circuits and focal epileptogenesis.

Our understanding of the pathophysiological mechanisms underlying the childhood epilepsies is rudimentary at this time. However, results of recent studies in animal models have lead to a number of important hypotheses concerning age-dependent alterations in seizure susceptibility. In area CA3 of hippocampus it appears that during a critical period when this brain area is particularly prone to electrographic seizures, an overabundance of recurrent excitatory synapses may exist. At the same time the synapses themselves appear to be functionally different than their mature counterpart. Age-dependent differences in the properties of postsynaptic NMDA receptors seem to contribute to enhanced seizure susceptibility. In recent years significant progress has been made in unravelling the fundamental processes that underlie the formation of connections between developing neurons. Over-production of early-formed axon collaterals appears to be common-place in the CNS. Moreover, the selection of appropriate patterns of connectivity appear to be dependent in large part on the patterning of neurophysiologic activity. In this regard the NMDA receptor seems to play a pivotal role in synapse consolidation. Based on these observations and the central role recurrent excitation appears to play in hippocampal seizures, it seems entirely plausible that excessive abnormal neuronal discharging that occurs during seizures early in life could result in the consolidation of abnormal numbers of recurrent excitatory synapses. This miswiring of hippocampal networks might be responsible for the marked seizure susceptibility into adulthood and might even contribute to complex partial epilepsy in individuals with a history of childhood seizures.

Adult↗

Neonatal seizures and limb malformations associated with liver-specific complex IV respiratory chain deficiency.

An eight-week-old infant, the fourth child of consanguineous parents presented with intractable neonatal seizures. The mother had two previous miscarriages. The infant initially presented on day one with multifocal myoclonus, complex partial and generalised tonic-clonic seizures. On examination, there were dysmorphic hands and feet, with absent nails and terminal phalanges of the fingers and toes, hepatomegaly, marked axial and peripheral hypotonia and severe global developmental delay. Ophthalmological assessment showed 'salt and pepper' pigmentary retinopathy. The urinary organic acid profile revealed a marked increase in tricarboxylic acid metabolites. Urinary phosphate reabsorption was reduced at 84%. Type I fibre atrophy was seen on muscle histology, and a cytochrome c oxidase deficiency was found only on enzymology of liver tissue. Limb malformations associated with respiratory chain defects have rarely been reported. To our knowledge, this child has the most severe limb anomaly associated with a tissue-specific complex IV respiratory chain defect.

Abnormalities, Multiple↗

The relationship between seizure subtype and interictal personality. Results from the Vietnam Head Injury Study.

A wide variety of interictal personality traits has been associated with complex partial (CP) epilepsy. However, the specificity of these behavioural changes to CP epilepsy has been questioned in several controlled studies. To address this issue, we examined personality and behavioural characteristics in 467 Vietnam veterans 15 years post penetrating head injury. Of this sample, 238 (51%) had developed seizure disorders which were classified as follows: 39 simple partial (SP), 59 CP, 76 partial with secondary generalization (PG), and 64 generalized (G). Seizure patients were compared with two demographically matched control groups: 229 penetrating head-injured Veterans without seizures (PHI-C) and 84 uninjured veterans (UC). Dependent measures included self-report and examiner rating scales, and history of psychiatric treatment. Pre-injury intelligence, brain volume loss (CT scan), seizure frequency and duration of epilepsy served as covariates. We found statistically significant increases in interictal psychopathology in the CP, PG and G groups when compared with the two control groups (PHI-C and UC). No group differences were observed across seizure subtypes. These results suggest that interictal personality and behavioural abnormalities are not specific to individuals with CP seizures. Furthermore, the increased psychopathology in seizure groups when compared with the PHI-C group suggests that personality changes cannot be accounted for by structural brain damage alone.

Adult↗

Auditory event-related potentials (P300) in the identification of psychogenic nonepileptic seizures.

The feasibility and conceivable value of postictal event-related potential (ERP) recordings were studied in patients with nonepileptic seizures (NES) admitted for long-term video/EEG monitoring. Ten patients with NES underwent preictal (on hospital admission) and postictal (< or =6 hours after seizure) ERP recordings of an auditory oddball paradigm. Additionally, 10 temporal lobe epilepsy (TLE) patients with partial seizures and secondary generalization underwent preictal, postictal (< 6 hours after seizures), and interictal (7-48 hours after seizure) ERP recordings. We recently reported that ERPs recorded in TLE patients with partial epilepsy undergo a temporary change postictally, while returning to their preictal state during interictal recordings. In the current study intraclass correlations, transformed into z scores, are used to determine test-retest validity of repeated ERP recordings. An independent sample t test with z scores for the comparison of preictal and postictal recordings showed that ERP activation differed between NES and TLE patients (P=0.009). More specifically, ERP recordings in the preictal and postictal states were similar in NES patients, but dissimilar in TLE patients. On the other hand, this dissimilarity in ERPs disappeared when comparing z scores for the preictal and postictal recordings in NES patients with z scores for the preictal and interictal recordings in TLE patients. This further supports the notion that identical waveforms during preictal and postictal recordings in NES patients reflect nonepileptic seizure activity. The current findings suggest that postictal ERP recordings are useful in the diagnosis of NES and differentiate TLE from NES.

Acoustic Stimulation↗

Epilepsy in middle-aged and elderly people: a three-year observation.

An analysis of the medical documentation and investigation of 130 cases of epilepsy diagnosed in a group of people over 50 years of age (average: 65.4 years) revealed that the most common type of seizure in the group studied was partial (66.2%), followed by seizures with secondary generalization (33.8%). Epilepsy was caused by cerebrovascular disease (50.8%) considerably more often in patients over 74 years of age, craniocerebral trauma in patients addicted to alcohol (13.1%), especially those under 65 years of age, primary or metastatic neoplastic disease (10.7%), and others. The authors wish to draw attention to the leukoaraiosis factor, which might be the proepileptogenic cause of epilepsy recognized in the group of patients over 74 years of age (56.5%) and is much more frequent in this group than in the group of patients under 65 years of age (1.6%). Moreover, some drugs, such as L-dopa and Baclofen, might have been related to the epileptic seizures. In 29 patients (22.3%) the definite cause of late-onset epilepsy was unknown. The authors suggest in such cases, both follow-up tomographic examination and careful clinical examinations. In the study group of patients with initially unknown seizure etiology, some diseases, such as cerebral tumor or colon and pancreatic neoplasm, were diagnosed during follow-up examination. These processes were revealed several months after the first epileptic seizure.

Aged↗

Lamotrigine: an antiepileptic agent for the treatment of partial seizures.

OBJECTIVE: To review the current literature on lamotrigine and its use as an antiepileptic drug (AED). DATA SOURCES: MEDLINE and bibliographic literature searches pertaining to lamotrigine were performed. Additionally, Burroughs Wellcome provided a comprehensive bibliography, data on file, and investigator's brochure. DATA SELECTION: The selection of reported data for this review includes both controlled and uncontrolled studies as well as case reports and unreported data from both European and US trials. DATA SYNTHESIS: Lamotrigine is effective as an adjunctive agent in the treatment of complex and simple partial seizures with or without secondary generalization. Anecdotal reports suggest that the spectrum of activity may include other seizure types, but controlled studies substantiating these reports are needed. Lamotrigine has a favorable pharmacokinetic profile, including a long half-life, low serum protein binding, and lack of mixed-function oxidase enzyme induction. It is likely that the drug induces metabolism through the glucuronidation pathway, although probably not to a clinically significant extent. Concurrent use of enzyme-inducing AEDs increase lamotrigine's clearance, whereas valproic acid decreases it. Adverse effects are primarily central nervous system-related, with dizziness, diplopia, ataxia, and somnolence reported in at least 10% of the patients treated. The incidence of these effects is higher in patients treated concomitantly with carbamazepine and may represent a pharmacodynamic interaction. The occurrence of rash may limit lamotrigine's use and was the most common cause for discontinuation in clinical trials (2.3%). The incidence of rash is higher in patients comedicated with valproic acid. CONCLUSIONS: Lamotrigine appears to be a safe and effective new AED for patients with refractory partial seizures when used as an adjunctive agent. It has a favorable pharmacokinetic profile allowing for once- or twice-daily dosing and adverse effects appear mild and transient. Additional studies are required to confirm efficacy in other seizure types.

Adolescent↗

Differential effects of left and right anterior temporal lobectomy on verbal learning and memory performance.

The California Verbal Learning Test (CVLT) was utilized to identify the quantitative and qualitative alterations in verbal learning and memory performance that discriminated between patients following partial resection of the left (dominant) (n = 26) or right (nondominant) (n = 31) temporal lobe. Patients were administered the CVLT preoperatively and 6 months postoperatively, and the differential effects of laterality of resection on verbal learning and memory performance were determined. Following left temporal resection, patients showed significantly more serial clustering, a lower proportion of words recalled from the middle of the list, and more intrusion errors in free recall. Patients who underwent right temporal resection showed significantly greater recall of words from the middle and fewer words from the end of the list, more semantic clustering, and greater ability to recall verbal material after a short delay. These findings suggest that anterior temporal lobectomy (ATL) results in changes in the way verbal material is acquired, and affects the rate of forgetting. Patients who undergo left ATL become more dependent on less effective and efficient learning strategies, and forget the material that they have acquired at a faster rate. The opposite tendencies characterize patients who undergo right ATL.

Adult↗

Time-frequency analysis using the matching pursuit algorithm applied to seizures originating from the mesial temporal lobe.

OBJECTIVES: The ability to analyze patterns of recorded seizure activity is important in the localization and classification of seizures. Ictal evolution is typically a dynamic process with signals composed of multiple frequencies; this can limit or complicate methods of analysis. The recently-developed matching pursuit algorithm permits continuous time-frequency analyses, making it particularly appealing for application to these signals. The studies here represent the initial applications of this method to intracranial ictal recordings. METHODS: Mesial temporal onset partial seizures were recorded from 9 patients. The data were analyzed by the matching pursuit algorithm were continuous digitized single channel recordings from the depth electrode contact nearest the region of seizure onset. Tine frequency energy distributions were plotted for each seizure and correlated with the intracranial EEG recordings. RESULTS: Periods of seizure initiation, transitional rhythmic bursting activity, organized rhythmic bursting activity and intermittent bursting activity were identified. During periods of organized rhythmic bursting activity, all mesial temporal onset seizures analyzed had a maximum predominant frequency of 5.3-8.4 Hz with a monotonic decline in frequency over a period of less than 60 s. The matching pursuit method allowed for time-frequency decomposition of entire seizures. CONCLUSIONS: The matching pursuit method is a valuable tool for time-frequency analyses of dynamic seizure activity. It is well suited for application to the non-stationary activity that typically characterizes seizure evolution. Time-frequency patterns of seizures originating from different brain regions can be compared using the matching pursuit method.

Algorithms↗

Electrocortical and behavioral responses produced by acute electrical stimulation of the human centromedian thalamic nucleus.

Incremental, desynchronizing and spike-wave electrocortical responses and concomitant symptoms to acute electrical stimulation of the centromedian thalamic nucleus (CM) were studied in 12 patients with intractable complex partial and tonic-clonic generalized seizures. Low-frequency (6/s), 320-800 microA stimulation of the caudal-basal and central portions of CM elicited incremental recruiting and augmenting-like responses with a bilateral regional scalp distribution, with emphasis at the ipsilateral frontal (recruiting) and central (augmenting) regions, while ventral-basal CM stimulation elicited primary-like responses with a focal distribution at the ipsilateral parietal region. High-frequency (60/s), 320-800 microA stimulation of caudal-basal and central, but not ventral-basal CM, elicited EEG desynchronization and a slow negative shift of the EEG baseline with scalp distribution similar to that showed by recruiting- and augmenting-like responses. Neither incremental nor desynchronization EEG responses were accompanied by evident patient sensory or motor responses. Low-frequency (3/s), high-intensity (30 V = 2400 microA) combined stimulation of the right CM and left non-specific mesencephalic ascending pathways elicited a response similar to the typical absence attack with all EEG and clinical ingredients: S1, S2, P1 and W components of the individual spike-wave complex and generalized spike-wave discharges followed by sleep spindle EEG afterdischarges, accompanied by motionless stare, 3/s eye blinking, lip smacking and total failure to respond to visual stimuli in patients under a simple responding task.

Behavior↗

FDG-positron emission tomography and invasive EEG: seizure focus detection and surgical outcome.

PURPOSE: To study the value of [18F]2-deoxyglucose (FDG)-positron emission tomography when surface ictal EEG is nonlocalizing. METHODS: FDG-PET scans were performed in 46 patients with complex partial seizures (CPS) not localized by ictal surface-sphenoidal video-EEG (VEEG) telemetry. Interictal PET was performed with continuous EEG monitoring, and images were analyzed with a standard template. Forty patients subsequently had subdural and 6 had depth electrodes (invasive EEG, IEEG); 22 had bilateral implants. A focus was detected in 40, and 35 had temporal lobectomy based on IEEG localization. RESULTS: There was a close association between IEEG and PET localization (p < 0.01): 26 patients had relative unilateral temporal FDG-PET hypometabolism, all had congruent IEEG, and 18 of 23 were seizure-free after temporal lobectomy. Five patients had unilateral frontotemporal hypometabolism (3 of 5 were seizure-free), 1 had frontal hypometabolism, and 14 had no lateralized PET abnormality (4 of 7 were seizure-free). Patients who became seizure-free had significantly higher lateral temporal asymmetry index (AI). PET showed > or = 15% relative temporal hypometabolism (AI) in 12 of 22 patients with nonlateralized surface ictal VEEG and was capable of distinguishing between frontal and temporal foci in 16 of 24 patients with lateralized, but not localized, surface ictal video-EEG. CONCLUSIONS: FDG-PET provides valuable data in patients with unlocalized surface ictal EEG and can reduce the number of patients who require IEEG studies. Quantitation is necessary for optimal PET interpretation.

Brain↗

The amygdala and temporal lobe simple partial seizures: a prospective and quantitative MRI study.

PURPOSE: To determine whether specific temporal lobe simple partial seizures (SPSs) are associated with an abnormal amygdala T2 (AT2) ipsilateral to the seizure focus in patients with intractable unilateral temporal lobe epilepsy (TLE). AT2 relaxation time mapping is a sensitive method for the detection of abnormal tissue in the amygdala in patients with refractory TLE. The relation between an abnormal AT2 in the epileptic temporal lobe and amygdala seizure onset has not been established. METHODS: Fifty patients with intractable unilateral TLE and concordant data during presurgical evaluation were included. Patients with a foreign-tissue lesion on standard magnetic resonance imaging (MRI) were excluded. All had AT2 mapping. Fifteen types of SPSs were ascertained prospectively, systematically, and blinded to the results of AT2 mapping. The SPSs of patients with a normal AT2 (n = 25) were compared with those of patients with an abnormal AT2 ipsilateral to the seizure focus (n = 25). RESULTS: The group of patients with an abnormal AT2 reported a median of six types of SPSs (range 1-11), in comparison with a median of three types of SPSs (range, 0-7) for the group with a normal AT2 (p<0.01). Déjà vu, a warm sensation, an indescribable strange sensation, a cephalic sensation, and fear were associated with an abnormal AT2. The combination of déjà vu, a cephalic sensation, a warm sensation, a gustatory hallucination, and an indescribable strange sensation discriminated best between the 25 patients with a normal and the 25 patients with an abnormal AT2. CONCLUSIONS: A high number and the types of different SPSs provide clinical evidence for early involvement of the amygdala during seizures in patients with refractory unilateral TLE and an abnormal AT2 in the epileptic temporal lobe

Adolescent↗

Treatment of epileptic seizures with clonazepam. A reappraisal.

The therapeutic efficacy of orally administered clonazepam has been evaluated in 32 epileptic patients, with substantial improvement in 22. The drug is active in all types of seizures, particularly in myoclonus, petit mal absences, and partial complex epilepsy; it seems the drug of choice in generalized infantile organic epilepsy, although the treatment of these patients is still unsatisfactory. In some patients, the drug seemed less effective after months of therapy. Drowsiness is the main side effect.

Administration, Oral↗

Carbamazepine plasma concentration. Relationship to cognitive impairment.

Neuropsychological function was assessed before and after carbamazepine monotherapy in children with newly diagnosed complex partial epilepsy. Simultaneous video-electroencephalographic monitoring examined the influence of subclinical abnormal electrical discharges on performance. Total and unbound plasma carbamazepine concentrations were examined in relation to changes in performance at low (carbamazepine level, less than or equal to 32 mumol/L [less than or equal to 7.5 mg/L]) and moderate (carbamazepine level, greater than 34 mumol/L [greater than 8.0 mg/L]) drug levels. The data suggest a mild beneficial effect of carbamazepine on speeded eye-hand coordination and, at low drug levels, more rapid processing of items in memory. Efficiency of learning new information and memory-scanning rate displayed a concentration-dependent relationship with carbamazepine level, with poor performance significantly associated with higher carbamazepine plasma concentrations. Carbamazepine free levels were equivalent to total levels in predicting cognitive side effects.

Carbamazepine↗