1/115. periventricular nodular heterotopia and childhood absence epilepsy.A young female presented with an epileptic syndrome resembling childhood absence epilepsy, a normal neurologic examination, generalized 3-Hz spike-and-wave discharges, and clinical absences. Her seizures responded to treatment with valproic acid. Other abnormalities in her electroencephalogram prompted neuroimaging studies, which demonstrated periventricular nodular heterotopia. review of published reports confirmed this presentation to be atypical of this developmental lesion. The authors describe their patient and discuss this unexpected association and the relevant reports briefly.- - - - - - - - - - ranking = 1keywords = epilepsy, childhood (Clic here for more details about this article) |
2/115. Nonconvulsive status epilepticus in childhood localization-related epilepsy.PURPOSE: To report on three children with localization-related epilepsy who exhibited minor seizures (atypical absences, brief atonic, and myoclonic) and nonconvulsive status epilepticus (NCSE) consisting of these minor seizures, and to elucidate their significance. methods: We studied the electroclinical characteristics of these children. Ictal electroencephalograms (EEGs) of NCSE were evaluated by using simultaneous video-EEG-electromyogram (EMG) polygraphic recordings. RESULTS: All patients began to have partial seizures between the ages of 6 months and 2 years 7 months, with minor seizures appearing later, between the ages of 1 year 11 months and 6 years 6 months. These minor seizures evolved into NCSE. Complex partial seizures remained after suppression of the minor seizures. Interictal EEGs taken when the minor seizures appeared showed excessive diffuse epileptic discharges in addition to multifocal spike-waves. Before and after suppression of the minor seizures, focal epileptic discharges predominated on the EEGs. On ictal EEGs of brief atonic and myoclonic seizures, diffuse spike-wave and polyspike-wave bursts were detected. Ictal EEGs of the atypical absences revealed diffuse spike-wave bursts mixed with irregular high-voltage slow waves, often interspersed with brief atonic and myoclonic seizures. When atypical absences lasted for a long time, patients manifested NCSE. Polytherapy might be related to the occurrence of minor seizures and NCSE, because all patients were treated with polytherapy at their appearance, and simplification of antiepileptic drug (AED) therapy seemed to be effective. CONCLUSIONS: We concluded that this NCSE is a type of atypical absence status which is an age-dependent, transient, electroclinical condition. The mechanism of occurrence of these minor seizures might be related to secondary bilateral synchrony.- - - - - - - - - - ranking = 0.99996126767792keywords = epilepsy, childhood (Clic here for more details about this article) |
3/115. Primary ciliary dyskinesia syndrome and primary generalised epilepsy.A child with primary ciliary dyskinesia syndrome (PCDS) developed primary generalised epilepsy (PGE). The PGE had characteristics of both childhood absence epilepsy (CAE) and juvenile myoclonic epilepsy (JME). The association of PCDS and PGE has not previously been recorded.- - - - - - - - - - ranking = 1.3997676060675keywords = epilepsy, childhood (Clic here for more details about this article) |
4/115. Tiagabine-induced absence status in idiopathic generalized epilepsy.Several medications such as baclofen, amitriptyline and even antiepileptic drugs such as carbamazepine or vigabatrin are known to induce absence status epilepticus in patients with generalized epilepsies. Tiagabine (TGB) is effective in patients with focal epilepsies. However, TGB has also been reported to induce non-convulsive status epilepticus in several patients with focal epilepsies and in one patient with juvenile myoclonic epilepsy. In animal models of generalized epilepsy, TGB induces absence status with 3-5 Hz spike-wave complexes. We describe a 32-year-old patient with absence epilepsy and primary generalized tonic-clonic seizures since 11 years of age, who developed her first absence status epilepticus while treated with 45 mg of TGB daily. Administration of lorazepam and immediate reduction in TGB dosage was followed by complete clinical and electroencephalographic remission. This case demonstrates that TGB can induce typical absence status epilepticus in a patient with primary generalized epilepsy.- - - - - - - - - - ranking = 1.5996901414234keywords = epilepsy (Clic here for more details about this article) |
5/115. Emotion-induced myoclonic absence-like seizures in a patient with inv-dup(15) syndrome: a clinical, EEG, and molecular genetic study.We have described a clinical EEG and molecular genetic study of a 9-year-old boy with inv-dup(15) syndrome in whom seizures were induced by emotionally gratifying stimuli. The reflex seizures began 5-20 s after the onset of repeated cheek-kissing from his mother or after viewing of pleasant or funny events. They were characterized by bilateral discharges involving mainly the temporal regions and evolving into myoclonic absence-like seizures. Nonemotional stimuli, such as a pinch, sucking or rubbing his cheeks, or the sound of the kiss alone, failed to provoke seizures. The seizures were resistant to antiepileptic (AED) treatments. Molecular genetic investigations revealed a correct methylation pattern of the chromosomes 15, and three copies (two maternal and one paternal) of the segment 15q11-q13, including the GABRb3 gene. We hypothesize that an overexpression of cerebral gamma-aminobutyric acid (GABA)-mediated inhibition accounts for the severe epilepsy that we observed in this patient.- - - - - - - - - - ranking = 0.19996126767792keywords = epilepsy (Clic here for more details about this article) |
6/115. Paradoxic reaction to lamotrigine in a child with benign focal epilepsy of childhood with centrotemporal spikes.A girl aged 5 years developed nocturnal seizures (mouth twitching, salivation, anarthria, with right arm jerking and occasional secondary generalisation), with frequent focal sharp waves over the left centrotemporal region in her EEG, suggesting benign childhood epilepsy with centrotemporal spikes (BECTS). seizures became diurnal and frequent, not modified by carbamazepine (CBZ) or valproate (VPA) but responding to VPA and lamotrigine (LTG) with recommended dosage schedules for this combination. Her school performance then deteriorated insidiously, with poor memory and concentration, clumsiness, stuttering, and emotional lability. After 4 months, new episodes, < or =10 per day, occurred. These lasted a few seconds; she stared into space, her jaw dropped, her head dropped to the right, and her eyelids flickered. She usually maintained awareness. Attacks were often provoked by blowing or sneezing. Ictal EEG showed anterior-predominant 3/s sharp-slow wave complexes lasting < or =8 s, with bilateral rolandic discharges interictally. Withdrawal of LTG resulted in rapid improvement in cognitive function and gradual remission of the new attacks. CONCLUSIONS: This appears to be a paradoxic reaction to LTG in the setting of BECTS.- - - - - - - - - - ranking = 1keywords = epilepsy, childhood (Clic here for more details about this article) |
7/115. transcranial magnetic stimulation induces 'pseudoabsence seizure'.OBJECTIVE: Several studies support the hypothesis of an antidepressive or mood-enhancing effect of repetitive transcranial magnetic stimulation (rTMS) on depressive patients. The most acute concern regarding rTMS is possible seizure induction; therefore, reports on seizure during rTMS are of special significance. METHOD: We describe a case in which high frequency rTMS over the left dorsolatero-prefrontal cortex (DLPC) applied as an add-on antidepressive strategy may have induced a frontal lobe complex partial seizure in a female patient affected by drug-resistant depression. RESULTS: The epileptic seizure was self-limited, and the patient did not report any physical sequelae. The psychopathological improvement, observed immediately after the incident in question, did not last. CONCLUSION: In this case train duration in rTMS, combined with drugs modulating the norepinephrine turnover, may have contributed to the occurrence of this complex partial seizure, which neuroanatomically seems to be localized in the DLPC.- - - - - - - - - - ranking = 0.0003008401331107keywords = frontal lobe, frontal, lobe (Clic here for more details about this article) |
8/115. The spectrum of acquired cognitive disturbances in children with partial epilepsy and continuous spike-waves during sleep. A 4-year follow-up case study with prolonged reversible learning arrest and dysfluency.We report a longitudinal study (7-11 years) of a previously normal boy (MR) who presented from the age of 5 years with rare partial motor seizures and atypical 'absences'. The history revealed a stagnation in development and speech difficulties a few months before onset of his epilepsy. The first waking electroencephalogram (EEG) showed rare generalized discharges during hyperventilation. magnetic resonance imaging revealed an arachnoid cyst in the frontotemporal region. Although his epilepsy never became severe, he experienced important learning difficulties. Subsequent EEGs became increasingly active with left focal epileptic activity and continuous spike-waves during sleep (CSWS) present from the first sleep EEG. The first neuropsychological evaluation (7 years) showed a speech dysfluency, word finding and naming problems, inattention and low intelligence quotient. carbamazepine was changed to clobazam and later ethosuximide was added with a rapid improvement (within 1 month) in linguistic and cognitive performances as well as in behaviour. Furthermore, the patient showed considerable progress in acquisition over the next months whereas learning to read had previously been very difficult. The epileptic activity gradually disappeared and he was able to follow regular school at an age-appropriate level. This case adds a new facet to the already recognized more obvious acquired neuropsychological disturbances known to occur in some partial childhood epilepsy syndromes with CSWS (aphasia, dementia). It manifested as prolonged insidious stagnation in learning and subtle language disability. This study documents rapid specific language improvement with change in anti-epileptic drugs and a restored immediate and long-term learning capacity, suggesting a direct but 'hidden' role of epilepsy.- - - - - - - - - - ranking = 1.5997288737454keywords = epilepsy, childhood (Clic here for more details about this article) |
9/115. Distinct behavioral and EEG topographic correlates of loss of consciousness in absences.PURPOSE: To describe the behavioral and EEG topographic correlates of absences with 3-Hz generalized spike-waves and partitioned impairment of consciousness. methods: Two adult women had so-called "phantom" absences, characterized by brief and mild impairments of consciousness that were previously inconspicuous to both patient and physician. Neuropsychological examination was performed under video-EEG monitoring during absence status. EEG topographic mapping of spike-wave discharges was obtained in the two cases. RESULTS: Only mild attentional and executive disturbances were observed during absence status despite prolonged discharges. Spike-wave bursts were associated with selective impairment in the initiation of response and self-generated action, whereas short-term storage of external information during discharges was fully preserved. This is consistent with a predominant involvement of frontomesial cortex demonstrated by topographic mapping of spike-wave discharges in the two cases. By contrast, in two other patients with typical absences and a complete lack of retention for information given during the discharges, topographic mapping found a more lateral frontal involvement by spike-wave activity. CONCLUSIONS: Different types of absence seizures may impair distinct components of conscious behavior. A predominant involvement of frontomesial thalamocortical circuitry may underlie an "inconspicuous" disorder of consciousness as seen in phantom absences with selective loss of initiation and goal-oriented behavior, whereas involvement of more lateral frontal areas in typical absences may additionally disrupt working memory processes.- - - - - - - - - - ranking = 0.0001661794873126keywords = frontal (Clic here for more details about this article) |
10/115. Evidence that Alpers-Huttenlocher syndrome could be a mitochondrial disease.We report an 11-year-old boy with a slight developmental delay and epilepsy. After he was placed on valproate, he developed hepatic failure and increasing neurologic symptoms, including epilepsia partialis continua, and died. autopsy findings in liver and cerebrum were consistent with progressive neuronal degeneration of childhood with liver disease, also called Alpers-Huttenlocher syndrome. Ragged red fibers and cytochrome c oxidase negative fibers were present in muscle. These results suggest that Alpers-Huttenlocher syndrome, at least in some patients, is a mitochondrial disease.- - - - - - - - - - ranking = 0.2keywords = epilepsy, childhood (Clic here for more details about this article) |
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