Cases reported "Epilepsies, Myoclonic"

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1/73. Long-term follow-up of an individual with vitamin B6-dependent seizures.

    We report on a 31-year-old female with vitamin B6-dependent seizures whose seizure onset was in the neonatal period. Her elder brother had the same disorder and died in infancy. Administration of vitamin B6 was initiated in the postnatal period. At the age of 12 years 1 month, 2 months after withdrawal of vitamin B6, visual seizures began to occur frequently. Myoclonic seizures and occasional generalized convulsive seizures were also observed. At the same time, photoparoxysmal response and spontaneous diffuse spike-wave bursts were seen on her EEG. Myoclonic seizures were provoked by intermittent photic stimulation during the EEG. It is distinctive that visual seizures were one of the main seizure types in this patient, that her clinical course was relatively benign, and that she has normal intellectual outcome.
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2/73. 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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3/73. 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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4/73. 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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5/73. Epileptic negative myoclonus induced by carbamazepine in a child with BECTS. Benign childhood epilepsy with centrotemporal spikes.

    A 7-year-old female with benign childhood epilepsy with centrotemporal spikes developed epileptic negative myoclonus (ENM) seizures during carbamazepine (CBZ) treatment. She had experienced nocturnal partial seizures since 5 years of age. Interictal electroencephalography demonstrated typical rolandic discharges. Valproate was first initiated at 6 years of age, but the seizures were uncontrollable. carbamazepine was added and valproate withdrawn. The frequency of partial seizures did not decrease. Moreover, she had brief episodes of tone loss in each or both arms and eye blinking several weeks after CBZ introduction. Unilateral loss of arm tone corresponded to spike-and-wave discharges in the contralateral centrotemporal region, and a loss of tone in arms was associated with bilateral synchronous discharges. eye blinking was also related to bilateral synchronous discharges and classified as a myoclonic seizure. The ENM and myoclonic seizures disappeared soon after CBZ withdrawal. Therefore the authors concluded that CBZ induced the ENM and myoclonic seizures in this patient. CBZ sometimes induces generalized seizures in the treatment of partial epilepsy and generalized epilepsy. CBZ-induced ENM seizures should be considered when a brief lapse of tone appears during CBZ treatment.
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6/73. reading epilepsy in a patient with previous idiopathic focal epilepsy with centrotemporal spikes.

    RATIONALE: reading epilepsy (RE) is a form of reflex epilepsy currently classified as an idiopathic localization-related epilepsy (ILAE, 1989). methods: We describe a 30 year-old right-handed male who suffered since the age of 8 from nocturnal partial motor seizures. Clinical features were typical of BECT. We reviewed the EEG recorded at that time which showed centrotemporal spike and waves. He was seizure-free from the age of 12 to the age of 17 when seizures evoked only by reading appeared. No other stimuli provoked seizures. Neurological and neuroradiological (CT and MR) investigations were normal. Baseline video-polygraphic EEG recordings were normal while reading aloud provoked myoclonic jerks in the facial muscles related to bilateral spike and wave discharges. Therapy with carbamazepine and valproic acid strongly reduced seizure frequency. CONCLUSION: Recent papers have debated the difficulties in classifying RE among the generalized or focal syndromes. literature reports describe an association with RE and juvenile myoclonic epilepsy, supporting the hypothesis of an idiopathic generalized form. We report the first documented case with a clear-cut idiopathic localization-related epilepsy evolving to a primary reading epilepsy.
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keywords = wave, frequency
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7/73. Is it juvenile myoclonic epilepsy?

    A 21-year old man with marked developmental delay was referred for the diagnosis of myoclonic jerks (MJ), which were sometimes responsible for sudden falls without loss of consciousness, that had begun 2 years before, and for a recent generalized tonic-clonic seizure preceded by a cluster of MJ. physical examination revealed a small stature, bilateral pyramidal signs, severe mental retardation, and retinis pigmentosa. Etiological factors for this encephalopathy were not found (muscle and skin biopsies, karyotype and extensive blood chemistry). Waking interictal EEG showed a normal background activity and generalized poly-spike-and wave (PSW) discharges. photic stimulation disclosed a marked photoparoxysmal response, sometimes associated with myoclonic jerks. Three spontaneous jerks accompanied by a burst of generalized PSW were recorded on awakening from a nap. The MRI disclosed wide ventricles, a thin corpus callosum, brainstem atrophy and a so-called "redundant gyration"; these changes were evocative of acquired perinatal damage. Juvenile myoclonic epilepsy (JME) was diagnosed and valproate was started resulting in complete control of seizures. During a 5-year follow-up, the patient has remained seizure-free and the EEG consistently normal. In our opinion, JME can be diagnosed in very uncommon settings, including patients with significant brain damage, as long as all the other criteria for the diagnosis are present.
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8/73. Late-onset myoclonic epilepsy in Down's syndrome (LOMEDS).

    The aim of this paper is to report a patient with late-onset myoclonic epilepsy in Down's syndrome (LOMEDS) as a differential diagnosis of adult-onset progressive myoclonic epilepsies. A 55-year-old male with Down's syndrome (DS) is described who developed progressively frequent myoclonus and generalized myoclonic-tonic seizures (GMTSs) at the age of 52. EEG recordings demonstrated background slowing and generalized polyspike-wave discharges occasionally associated with myoclonic jerks, leading to the classification of primary generalized epileptic myoclonus. Descriptions of late-onset epilepsy in DS patients are rare. However, a review of the pertinent literature revealed at least two other cases of elderly DS patients developing progressive myoclonic epilepsy after the onset of dementia. We suggest that late-onset myoclonic epilepsy in Down's syndrome as characterized here should be considered in the differential diagnosis of adult-onset myoclonic epilepsies. LOMEDS apparently shares features with myoclonic epilepsy in Alzheimer's disease (AD) and Unverricht-Lundborg disease (ULD) caused by a mutation on chromosome 21. Since life expectation of DS patients has markedly increased, LOMEDS may be more frequent than currently acknowledged.
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9/73. Neurocardiogenic syncope in a 10-year-old boy.

    We present a case with a suspected epileptic disorder. This may be a result of a neurocardiogenic syncope leading to seizures. A 10-year-old boy suffered two episodes of sudden loss of consciousness after getting injections. electrocardiography (ECG) and electroencephalography recordings during a venipuncture showed asystole of 6 seconds followed by a generalized seizure with clonic jerks of the right arm and leg while theta waves in the EEG were noted. Tilt-table testing could not provoke a pathological reaction.
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10/73. Aucte intermittent porphyria and epilepsy.

    A 14-year-old boy had suffered from intermittent acute hepatic porphyria, myoclonic convulsions and mental retardation (Lennox-Gastaut syndrome). The porphyria was treated by stopping the administration of phenobarbitone and phenytoin. sodium valproate at a dose of 70 mg/kg per day lessened the severity and frequency of convulsive crises.
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keywords = frequency
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