Cases reported "Epilepsy, Absence"

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1/176. Epileptogenic mineralization: pathological variants with good prognosis.

    Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy.
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ranking = 1
keywords = seizure
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2/176. 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.
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ranking = 48.709786754411
keywords = epilepsy, seizure
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3/176. Prolonged psychic epileptic seizures: a study of the absence status.

    Long-lived epileptic seizures associated with spike-and-wave complexes are presently considered to be the absence status, i.e., the generalized nonconvulsive status. EEG radiotelemetry allowed us to record three prolonged seizures of 3 epileptic patients. Clinical manifestations included selective rather than global impairment of higher cortical functions. Clinical impairment appeared only when patients were in a state of activity and if those altered functions were used. EEG abnormalities were diffuse, but among them spike-and-wave complexes were never diffuse. It was impossible to establish close electroclinical correlation. However, the clinical and electrical evolution was roughly isomorphic, i.e., cyclic. Major clinical manifestations were associated with spikes rather than with slow waves. Lastly, patients showed common ictal psychopathological symptoms. The problem of classifying such seizures in either the generalized or partial status is discussed. The role of selective impairment of mental functions in psychopathological symptoms is also dealth with.
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ranking = 3.5
keywords = seizure
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4/176. 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.
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ranking = 54.709786754411
keywords = epilepsy, seizure
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5/176. 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.
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ranking = 67.493701456175
keywords = epilepsy
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6/176. Nonconvulsive status epilepticus in eyelid myoclonia with absences--evidence of provocation unrelated to photosensitivity.

    A 10-year old girl with eyelid myoclonia with absences (EMA) in whom nonconvulsive status epilepticus developed shortly after awakening is described. A video-polygraphic recording during the status showed the characteristic eye-closure provocation of eyelid myoclonia with upward deviation of the eyeballs and brief absences. Ictal EEG showed generalized polyspikes concomitant with eyelid myoclonia, while absences were accompanied by 3.5 Hz polyspike-wave complexes on EEG. This condition occurred even in total darkness as well as even after seizures precipitated by bright sunlight had been eliminated by medication. The present case suggests that the eye closure mechanism could be a more potent precipitating factor than photosensitivity in the pathophysiology of EMA.
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ranking = 0.5
keywords = seizure
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7/176. 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.
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ranking = 77.635658807057
keywords = epilepsy, seizure
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8/176. 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.
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ranking = 14.141957350882
keywords = epilepsy, seizure
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9/176. Myoclonic status epilepticus following high-dosage lamotrigine therapy.

    An 8-year-old girl with Lennox-Gastaut syndrome showed a partial reduction in seizure frequency when lamotrigine (LTG), 15 mg/kg per day, was added to clobazam (CLB) and vigabatrin (VGB). An increase in LTG dosage to 20 mg/kg per day produced no further improvement and was followed by myoclonic status epilepticus. The condition developed insidiously and ultimately became stable. Video-EEG polygraphy and jerk-locked back-averaged EEG demonstrated continuous myoclonus of cortical origin. Discontinuation of LTG resulted in rapid disappearance of clinical and electrophysiological manifestations of myoclonic status epilepticus. No episodes of myoclonus occurred in the subsequent 2 years, during which CLB and VGB were kept unchanged. The striking response to drug discontinuation suggests that LTG may have played a role in the precipitation of status, possibly within the context of paradoxical intoxication.
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ranking = 0.5
keywords = seizure
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10/176. 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.
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ranking = 48.709786754411
keywords = epilepsy, seizure
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