Cases reported "Epilepsy, Benign Neonatal"

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1/4. 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 carbamazepine was added and valproate withdrawn.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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2/4. Benign partial epilepsy in infancy and early childhood with vertex spikes and waves during sleep: a new epileptic form.

    International epilepsy classification includes different epileptic syndromes with favourable outcomes in paediatric age, both partial and generalised. This is true in childhood while no partial benign forms are accepted in infancy. In 1987, Watanabe first described a new entity and he defined it as 'benign complex partial epilepsies in infancy'. In 1992, Vigevano referred similar but familial cases whose seizures had secondary generalisation. Both these forms had no interictal EEG abnormalities neither awake nor during sleep. This article presents a survey of 12 cases of partial epilepsy with favourable outcome differing from Watanabe and Vigevano's cases, both for the presence of interictal EEG abnormalities only during sleep and for seizure picture. All our patients are neurologically and neuroradiologically normal. Psychomotor development is unremarkable. Age onset range is 13-30 months. All cases present characteristic spikes and waves during slow-sleep in vertex cerebral areas. Awake EEG is always normal, at follow-up too. Our cases have such homogeneous electroclinical features as to hypothesise a new partial idiopathic epileptic syndrome with favourable outcome in infancy and early childhood. We propose to define it as 'benign partial epilepsy in infancy and early childhood with vertex spikes and waves' (BVSE).
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3/4. Epileptic falls and gait disturbance in two young children with a sharp wave focus at the vertex: a variant of benign partial epilepsy of childhood?

    We describe two young children who presented with frequent falls and myoclonic jerks affecting the trunk and legs associated with a sharp and slow wave epileptic focus at the vertex. The initial neurological examination and brain magnetic resonance imaging were normal. Both patients had a persistent gait dysfunction, sometimes asymmetrical, fluctuating with the intensity of the epilepsy and the electroencephalogram abnormalities. The localization of the epileptic focus at the vertex, corresponding to the motor control of the legs and trunk, can explain this peculiar semiology. The seizures were difficult to treat, but one patient is currently in remission. Although epileptic falls are most often a feature of severe epilepsies of childhood, we think that these two patients present a variant of benign partial epilepsy of childhood.
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keywords = wave
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4/4. Benign familial infantile convulsions: phenotypic variability in a family.

    Benign familial infantile convulsion is an autosomal dominant epilepsy syndrome characterized by seizures starting from 3 to 12 months and a favorable outcome. We present a Turkish family with benign familial infantile convulsions and report the clinical variability associated with this syndrome in three generations. All 11 affected members had benign infantile seizures, which were primarily generalized in all but one patient, who had partial seizures with secondary generalization. The seizures started within the first year and were accompanied by normal neurologic development and a good response to treatment with phenobarbital. In this family, the phenotype extended beyond infancy. The index patient had unilateral occipital spike and waves on electroencephalography (EEG), although he had no clinical seizures at 4 years of age. Follow-up EEG of this patient 1 year later showed that the discharges shifted to the occipital lobe of the other hemisphere. The grandmother of this patient had temporal lobe seizures as an adult, years after the remission of infantile convulsions. One of the patients experienced paroxysmal choreoathetosis during adolesence. Our findings highlight the intrafamilial phenotypic variability of benign familial infantile convulsions in a large pedigree with long-term follow-up.
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