Cases reported "Epilepsy, Tonic-Clonic"

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1/5. Reduction of epileptic seizures by reinforcement of bladder continence.

    Epileptic seizures and resulting incontinence of urine were effectively reduced by reinforcement of incompatible behaviour with individual programming within a general token economy system. Two treatment phases, including token and social reinforcement, during an 11 week period resulted in significant seizure reduction. Therapeutic gains were maintained in a 6 month follow-up.
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ranking = 1
keywords = behaviour
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2/5. Neurological findings in patients with the fragile-X syndrome.

    We report two brothers with previously unexplained mental retardation and seizures who had dysmorphic facial features, macro-orchidism, and a fragile site at the x chromosome. This recently described syndrome is the second most common chromosome aberration associated with mental retardation after Down's syndrome. In order to determine the prevalence of seizures and the frequency of specific neurological features, we studied a total of 17 patients with the fragile x syndrome. 41% had grand mal seizures; 41% had extensor plantar responses; 47% had hyperactive behaviour and 65% exhibited stereotypics; 59% had incoordination and 35% had blepharospasm. We emphasise the need for chromosome analysis of patients with unexplained mental retardation, specific phenotypic abnormalities, and large testes.
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ranking = 1
keywords = behaviour
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3/5. Emotional and behavioural aspects of rett syndrome.

    A survey of 107 families of children with rett syndrome was conducted to further define the behavioural phenotype. There was a high prevalence of behavioural and emotional problems, including episodes of anxiety (76 per cent), low mood (70 per cent) and self-injurious behaviour (49 per cent). Although there was no significant difference across the age-ranges studied, there was a tendency towards a reduction in these problems with increasing age, suggesting that the behavioural phenotype in rett syndrome varies with age. epilepsy occurred in over half the children, but there was no clear association with behavioural and emotional problems. A number of families had established strategies for coping with their child's anxiety and mood changes.
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ranking = 9
keywords = behaviour
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4/5. Transient psychosis in a girl with epilepsy and continuous spikes and waves during slow sleep (CSWS).

    A normally developed and healthy 6-year-old girl suffered the onset of epilepsy with generalized tonic-clonic seizures and atypical absences. Initially the EEG showed epileptiform activity over the temporal and parietal regions, later there were episodes of bilateral synchronous spike-wave activity with a frequency of 1.5-2.5 Hz. After a few months, deterioration of cognitive and behavioural functions appeared and gradually increased with the development of a full-blown disintegrative psychosis that went on for several months. sleep EEG recordings showed the characteristic abnormality described as continuous spikes and waves during slow sleep. Later there was a remarkable improvement of neuropsychiatric functions but a second outbreak of psychosis seems to have left the girl, who is now 9 years of age, with severe mental impairment.
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ranking = 1
keywords = behaviour
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5/5. Nonconvulsive status epilepticus with generalized 'fast activity'.

    We report the case of a 55-year-old patient with infrequent generalized tonic-clonic seizures since the age of 40 years and additional frequent episodes of disturbed behaviour and impaired cognition. The latter last from several hours up to one day with sudden onset and end of electroencephalographic (EEG) changes and clinical manifestations. They occurred about once a week before they were successfully treated with valproate and lamotrigine. From clinical, therapeutic and EEG findings we conclude that the patient suffers from nonconvulsive status epileptici, although the ictal EEG showed an untypical pattern of monomorphic generalized alpha rhythm. Many EEG characteristics point towards a primary generalized seizure disorder but a focal origin with secondary bilateral synchrony has to be considered as well. However, this is obviously a rare type which has not been described previously.
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ranking = 1
keywords = behaviour
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