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[The postictal state. A clinically oriented observation of patients with epilepsy].

UNLABELLED: Epileptic seizures are followed by dynamic alterations in neurologic function in the postictal period which have received little attention by clinicians over a long period of time. We therefore retrospectively studied videotapes of 160 patients with focal epilepsy who underwent presurgical evaluation, for the occurrence of postictal symptoms to determine whether these phenomena have any localizing or lateralizing value in defining the seizure onset zone. RESULTS: (1) We found postictal paresis in 22 of 160 patients (18.8%) in each case contralateral to the hemisphere of seizure onset. (2) 'Perservative' automatisms which start during the ictus and continue in the postictal period occurred in 25.2% of 135 patients with temporal lobe epilepsy but not in patients with frontal lobe epilepsy. (3) Sexual automatisms defined as manipulations of the genitals were found exclusively in patients with temporal lobe epilepsy (in 5.9% of 135 patients). (4) Postictal 'Nose-wiping' was evident in 51.3% of 76 temporal lobe epilepsy patients but only in 12.0% of 25 extratemporal lobe epilepsy patients and was performed with the hand ipsilateral to the hemisphere of seizure onset in 86.5% of all temporal lobe seizures. (5) Postictal language disturbances were observed only in patients with temporal lobe epilepsy (34% of 97 patients) and pointed to a seizure onset in the dominant hemisphere in 80.8%. We conclude that postictal phenomena can provide reliable information for the localization of the seizure onset zone in patients with complex partial seizures. Thus, more attention should be given to the postictal state during presurgical epilepsy monitoring.

Aphasia↗

Vigabatrin in intractable childhood epilepsy: a retrospective study.

The effects of vigabatrin were studied over a 6-month period in 43 patients with intractable epilepsy. Children with complex partial seizures, with or without secondary generalization, responded best with more than one-half achieving a greater than 50% reduction; generalized tonic-clonic seizures also improved but there was no significant change in absence or myoclonic seizures. Four patients are seizure-free on monotherapy with vigabatrin. The drug was well tolerated with few side effects.

Adolescent↗

De novo epileptic confusional status in a patient with cobalamin deficiency.

A 80-year-old man with cobalamin deficiency and no history of epilepsy developed a partial complex epileptic confusional status (ECS) unresponsive to acute i.v. diazepam. Brain CT scan and MRI investigation ruled out a focal cerebral lesion. Therapy with high doses (10,000 micrograms i.m. daily) of cobalamin alone was started, and the patient fully recovered in the following 72-hour. Control EEGs repeatedly performed days and weeks later showed progressive disappearance of the frontal interictal spiking, while the patient was on monotherapy with cobalamin (5,000 micrograms i.m. weekly). A month later the patient unfortunately discontinued replacement therapy and 13 weeks later he developed a fatal convulsive epileptic status. To our knowledge the association of ECS and cobalamin deficiency has not been previously reported.

Aged↗

Visual field constriction is not limited to children treated with vigabatrin.

Vigabatrin (GVG) is widely used in the treatment of complex partial seizures and infantile spasms. Persistent visual field constriction associated with GVG therapy in adults was reported as a rare but serious side-effect. Visual field examination in children is more difficult because of a lack of cooperation among very young or mentally handicapped patients. We performed Goldmann perimetry in 12 of 153 patients treated with GVG as mono- or as add-on therapy. The others would not cooperate, and two adolescents refused the examination. For comparison, we examined 12 age-matched patients with complex partial or generalized epilepsy who had never taken GVG. In five of 12 GVG-treated patients, and in one of the control group, we found a concentric visual field constriction. All patients were subjectively asymptomatic. The GVG-treated patients had taken the drug in combination with valproic acid (VPA) or oxcarbazepine (OCB). In four patients, GVG treatment was already stopped at the time of the ophthalmologic examination. Three patients had intracerebral lesions that could not account for the pathologic perimetric findings. The single patient from the control group with concentric visual field constriction had an absence epilepsy, treatment being performed with VPA and lamotrigine (LTG). In conclusion, GVG has a causal but not unique connection with visual field constriction in pediatric patients.

Adolescent↗

Classification of the epilepsies: an investigation of 945 patients in a developing country.

The present study classified epilepsies in Nigerians and compared them with the profile of epilepsies as found in other countries: India, France, and Denmark. Partial epilepsy formed the largest group (76.6%) in this study and in that in India (80%), but these incidences were higher than that found in France (62%), owing to a higher frequency of birth injury, CNS infections, and childhood febrile convulsions in developing countries. In contrast to our study and that in France, where partial epilepsy with complex symptomatology formed the largest subgroup, partial epilepsy with elementary symptomatology formed the largest subgroup in India. The reason for this is not totally clear, though etiological factors and criteria for categorization are contributory. The incidence of partial epilepsy was lower in children than in adults owing to a relatively lower incidence of partial epilepsy with elementary symptomatology in children in the present series and a lower incidence of complex symptomatology in children in France and Denmark. Nigerian children seem more vulnerable to complex symptomatology owing to a high incidence of febrile illness (e.g., from malaria) and febrile convulsions. The incidence of generalized epilepsies in children was higher than in adults. Grand mal formed the largest subgroup of generalized epilepsies in children in this series and in Denmark, whereas petit mal formed the largest subgroup in France and India. Petit mal was relatively rare in children in our series (2.5%) compared with children in the French study (17.5%). Secondary generalized epilepsy was peculiar to children in all the series.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Benign pediatric localization-related epilepsies. Part I. Syndromes in infancy.

There is currently increasing interest in identifying and classifying pediatric benign epilepsy syndromes and recently several new syndromes have been recognized. Benign epilepsy syndromes, by definition, occur in children with normal developmental history, respond well to therapy, and remit without sequelae. The large majority of children with benign epilepsy syndromes follow a truly benign course. The concept of benign epilepsy syndromes has, however, been challenged by the minority of patients who continue to have seizures despite therapy, develop new seizures after initial remission, or exhibit neuropsychological abnormalities. Without long-term follow-up, benignity can not be truly ascertained a priori. Thus it may be preferable to use the terms possible and probable before the name of a specific syndrome until such time that the diagnosis of a definite benign syndrome is confirmed on long-term follow-up. In this review of the pediatric benign localization-related epilepsy syndromes, we address the concept of benignity and the process of diagnosis of a benign epilepsy syndrome. In addition we review the epidemiology, clinical manifestations, EEG findings, work-up, diagnostic criteria, differential diagnosis, genetics, management and prognosis of benign infantile familial convulsions, benign partial epilepsy in infancy with complex partial seizures, benign partial epilepsy in infancy with secondarily generalized seizures, benign infantile convulsions associated with mild gastroenteritis, and benign infantile focal epilepsy with midline spikes and waves during sleep.

Child, Preschool↗

Patient awareness of complex partial seizures.

PURPOSE: To assess self-awareness of complex partial seizures (CPSs) in unselected epilepsy patients through a thorough interview. METHODS: The study comprised 134 patients at our epilepsy clinic, whose CPSs had been documented by the patient's family members. We investigated the proportion and characteristics of patients unaware of their CPSs compared to those who were, and we monitored the evolution of unawareness of CPSs during the follow-up. RESULTS: Thirty-one (23%) patients were assigned to the unawareness group (complete, 23; incomplete, 8) and 103 (67%) patients to the awareness group. Patients in the unawareness group were older and had a later age of onset than patients in the awareness group. Interictal epileptiform discharges (IEDs) localized primarily to the temporal region and were more frequently detected in the unawareness group (94%) than the awareness group (55%). Bilateral independent IEDs were found more frequently in the unawareness group than in the awareness group (48% vs. 13%). The bilateral presence of lesions was also more frequent in the unawareness group than the awareness group (16.1% vs. 4.9%). Six (26%) of 23 patients with complete unawareness of their CPSs had experienced awareness of CPSs during the follow-up. Two of these patients even experienced the emergence of de novo aura. CONCLUSIONS: Our results indicate that a significant number of epilepsy patients are not aware of their CPSs. Unawareness of CPSs may be related to bitemporal dysfunction and a rapid and complete loss of consciousness caused by rapid spread of ictal discharges to the contralateral hemisphere in association with bilateral independent IEDs and bilateral presence of lesions.

Adult↗

Beta activity in status epilepticus.

Focal beta activity on scalp EEG is a rare seizure pattern that has most extensively been studied in patients undergoing evaluation for epilepsy surgery. However, ictal beta activity is uncommon beyond this population and has not been reported in human status epilepticus. We observed ictal focal beta activity as the predominant seizure pattern in a patient with refractory status epilepticus. Continuous left temporal beta activity clinically correlated with complex partial semiology on video-EEG.

Anticonvulsants↗

Brain diffusion after single seizures.

PURPOSE: Diffusion-weighted magnetic resonance imaging (DWI) after focal status epilepticus has demonstrated focal alterations of the apparent diffusion coefficient (ADC) in the epileptogenic zone. We hypothesized that localized dynamic alterations of brain diffusion during the immediate postictal state will be detectable by serial DWI and correlate with the epileptogenic zone. METHODS: Nine adult patients (four men, five women) with medically intractable epilepsy were prospectively examined with a total of 25 DWI scans taken 2-210 min after a seizure. RESULTS: The interictal ADC was significantly (p < 0.05) elevated in the ictogenic hippocampus in all patients with temporal lobe epilepsy. The following postictal changes of the ADC were seen: (a) decreases by maximally 25-31%, which were most pronounced in the epileptogenic zone (n = 2); (b) generalized ADC changes after generalized seizures (n = 1) or prolonged complex partial seizures (n = 2); (c) no major changes after short-lived seizures or if the time to first DWI scan was >15 min or both (n = 3); and (d) widespread bilateral ADC increases after a flumazenil-induced seizure (n = 1). CONCLUSIONS: ADC changes seen during serial postictal DWI are complex and appear to reflect origin and spread of the preceding seizure. A delineation of the epileptogenic zone appears to be possible only in complex-partial seizures of >60 s duration that do not secondarily generalize.

Adult↗

Stereotactically-guided craniotomy for cavernous angiomas presenting wit epilepsy.

With the wider availability of magnetic resonance imaging cavernous malformations are being recognised with increasing frequency in those patients presenting with intractable epilepsy. Surgical resection is the treatment of choice. However, because these lesions are usually small and may be located in eloquent areas stereotactic resection should be considered. Stereotactically-guided resection of pathologically verified cavernous angiograms was performed in 10 patients in this series presenting with epilepsy (8 males, 2 females, mean age 32 years). Eight patients presented with medically intractable epilepsy (5 complex partial seizures, 3 grand mal seizures). Of the remaining patients one experienced multiple episodes of haemorrhage and the other headaches (with a non-diagnostic scan) both in association with epilepsy. Pre-operative localisation of the motor strip was determined in one case by functional MRI. Following resection of these lesions all patients experienced improved seizure control with a mean follow-up period of 22 months. The mean postoperative hospital stay was 5.1 days with no surgical complications recorded. We conclude that stereotactically-guided resection offers significant advantages in the management of cavernous malformations. Surgical indications for operative resection would include medically refractory epilepsy, repeated haemorrhage and those cases where there is diagnostic uncertainty.

Adolescent↗

Epilepsy and medication effects on the pattern visual evoked potential.

Visual disruption in patients diagnosed with epilepsy may be attributable to either the disease itself or to the anti-epileptic drugs prescribed to control the seizures. Effects on visual function may be due to perturbations of the GABAergic neurotransmitter system, since deficits in GABAergic cortical interneurons have been hypothesized to underlie some forms of epilepsy, some anti-epileptic medications increase cortical GABA levels, and GABAergic neural circuitry plays an important role in mediating the responses of cells in the visual cortex and retina. This paper characterizes the effects of epilepsy and epilepsy medications on the visual evoked response to patterned stimuli. Steady-state visual evoked potentials (VEP) evoked by onset-offset modulation of high-contrast sine-wave stimuli were measured in 24 control and 54 epileptic patients. Comparisons of VEP spectral amplitude as a function of spatial frequency were made between controls, complex partial, and generalized epilepsy groups. The effects of the GABA-active medication valproate were compared to those of carbamezepine. The amplitude of the fundamental (F1) component of the VEP was found to be sensitive to epilepsy type. Test subjects with generalized epilepsy had F1 spatial frequency-amplitude functions with peaks shifted to lower spatial frequencies relative to controls and test subjects with complex partial epilepsy. This shift may be due to reduced intracortical inhibition in the subjects with generalized epilepsy. The second harmonic component (F2) response was sensitive to medication effects. Complex partial epilepsy patients on VPA therapies showed reduced F2 response amplitude across spatial frequencies, consistent with previous findings that showed the F2 response is sensitive to GABA-ergic effects on transient components of the VEP.

Adult↗

Gabapentin: discussion.

Gabapentin (GBP, Neurontin) is a novel antiepileptic drug (AED) that was shown to be effective against refractory partial seizures in five placebo-controlled trials. However, a number of patients with complex partial seizures experienced an increase in seizure frequency, suggesting that patients suffering from complex partial seizures are not a homogeneous group. In fact, we found that currently available AEDs are likely to be ineffective when staring is a prominent component of complex partial seizures. The poor response of this group of patients may reflect the fact that staring spells are inhibitory seizures and that the AEDs prescribed for partial seizures appear to facilitate inhibitory mechanisms. GBP resembles phenytoin (PHT) and carbamazepine (CBZ) in depressing segmental and reticular excitatory mechanisms and facilitating segmental inhibitory mechanisms, just as it resembles PHT and CBZ in efficacy against some partial seizures and against secondarily generalized seizures. Perhaps the patients in whom GBP increased seizure frequency had complex partial seizures with staring and were therefore unlikely to benefit from drugs such as GBP, CBZ, and PHT, which enhance inhibitory mechanisms in the brain. These findings suggest that future AED trials would greatly benefit from a categorization of complex partial seizures into nosologically distinct groups.

Acetates↗

Paroxysmal pain as sole manifestation of seizures.

A developmentally normal 4-year-old white female who presented with pain in the right hand as the only manifestation of epilepsy is reported. Two years later, she developed complex partial seizures following right-hand pain. Computed tomography and magnetic resonance imaging were unremarkable. Prolonged ambulatory electroencephalography (EEG) as well as video-EEGs with ictal pain episodes failed to reveal abnormalities. Only a full night video-EEG performed after antiepileptic drug withdrawal demonstrated 2 right-hand pain episodes followed by a complex partial seizure with ictal epileptiform activity on the scalp EEG in the left parasagittal area, rapidly generalized and interictal discharges in the C3-P3 area. This patient had a very unusual presentation of epilepsy.

Anticonvulsants↗

Localized cerebellar hypometabolism in patients with complex partial seizures.

PURPOSE: We sought to determine the cause of cerebellar dysfunction in epilepsy and whether this dysfunction was directly related to seizures. METHODS: Cerebellar metabolism was evaluated in 48 patients with a well-defined region of seizure onset and with corresponding hypometabolism. Regions of interest (ROI) were drawn according to a standardized template. If the ROI/nonepileptogenic cortex count rate ratio was outside the 95% confidence interval (CI) of controls, the ROI was defined as abnormal. The ratios from cerebellar hemispheres (defined as ipsi- or contralateral to the seizure onset region), were compared among controls (n = 8); patients who had seizure onsets and corresponding hypometabolism mesially in a temporal lobe (patient group 1, n = 19); patients whose seizures had onset mesially in a temporal lobe but spread rapidly to the ipsilateral frontal lobe and who had hypometabolism both in the affected temporal lobe and frontal lobe (patient group 2, n = 23); and patients who had seizure onsets and corresponding hypometabolism in the frontal lobe (patient group 3, n = 6). RESULTS: Significant hypometabolism was noted in the contralateral cerebellum of patients in group 2 and 3 [p = 0.007 and p = 0.008, respectively; two-way analysis of variance (ANOVA)]. In contrast, patients in group 1 tended to have lower values in the ipsilateral cerebellum (p = 0.057). CONCLUSIONS: The observed cerebellar changes are consistent with animal data showing that cerebellar connections to frontal lobes are numerous and crossed, whereas the connections to mesial temporal lobes are less abundant, bilateral, with an ipsilateral predominance. The difference between the two groups of patients with mesial temporal seizures suggests that cerebellar dysfunction in partial epilepsy, at least to a certain extent, is related to mechanisms involved in seizure generation and spread.

Cerebellum↗

Partial epilepsy in neurologically normal children: clinical syndromes and prognosis.

A clinical and electroencephalographic study of 107 neurologically normal children with partial seizures was undertaken to verify the existence and determine the frequency of epileptic syndromes reported in selected populations. Sixty-three children had simple partial seizures, 39 had complex partial seizures, and 5 children were unclassifiable. The syndrome of benign partial epilepsy of children with rolandic spikes (BPEC, 38 cases) was clearly identified and its uniformly benign final prognosis was confirmed even if some of these children had at times severe or poorly controlled seizures. Among the children with simple partial seizures outside the BPEC (25 cases) and complex partial seizures (39 cases), no homogeneous clinical or electroclinical subgroup could be found. Two children with benign partial epilepsy and myoclonic-astatic seizures ("atypical benign partial epilepsy of childhood") and one child with "benign epilepsy with occipital spike-waves" were identified. 74% of children with epilepsy with complex partial seizures (ECP) had a 1-year seizure-free interval, and many children with epilepsy with simple partial seizures outside the BPEC group (ESP) had no more than two seizures. A benign course is thus not limited to the BPEC but is difficult to predict. Prospective studies are necessary to confirm the existence of well-defined benign syndromes among the idiopathic partial epilepsies of childhood, which appear quite rare outside the BPEC.

Adolescent↗

Hippocampal neurons and glia in epileptic EL mice.

Reactive changes in hippocampal astrocytes are frequently encountered in association with temporal lobe epilepsy in humans and with drug or kindling-induced seizures in animal models. These reactive changes generally involve increases in astrocyte size and number and often occur together with neuronal loss and synaptic rearrangements. In addition to producing astrocytic changes, seizure activity can also produce reactive changes in microglia, the resident macrophages of brain. In this study, we examined the effects of recurrent seizure activity on hippocampal neurons and glia in the epileptic EL mouse, a natural model of human multifactorial idiopathic epilepsy and complex partial seizures. Timm staining was used to evaluate infrapyramidal mossy fiber organization and the optical dissector method was used to count Nissl-stained neurons in hippocampus of adult (about one year of age) EL mice and nonepileptic C57BL/6J (B6) and DDY mice. Immunostaining for glial fibrillary acidic protein (GFAP) and Iba1, an actin cross-linking molecule restricted to macrophages and microglia, was used to evaluate astrocytes and microglia, respectively. The EL mice experienced about 25-30 complex partial seizures with secondary generalization during routine weekly cage changing. No significant differences were found among the mouse strains for Timm staining scores or for neuronal counts in the CA1 and CA3 pyramidal fields or in the hilus. However, the number of GFAP-positive astrocytes was significantly elevated in the stratum radiatum and hilus of EL mice, while microglia appeared hyper-ramified and were more intensely stained in EL mice than in the B6 or DDY mice in the hilus, parietal cortex, and pyriform cortex. The results indicate that recurrent seizure activity in EL mice is associated with abnormalities in hippocampal astrocytes and brain microglia, but is not associated with obvious neuronal loss or mossy fiber synaptic rearrangements. The EL mouse can be a useful model for evaluating neuron-glia interactions related to idiopathic epilepsy.

Animals↗