Cases reported "Epilepsy, Reflex"

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1/71. 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. ( info)

2/71. Psychological management of intractable seizures in an adolescent with a learning disability.

    Psychological interventions aimed at seizure management are described with a 14-year-old boy with a learning disability and intractable epilepsy. Baseline records suggested that a majority of tonic seizures and 'drop attacks' were associated with going off to sleep and by environmental 'startles'. Psychological formulation implicated sudden changes in arousal levels as an underlying mechanism of action. Cognitive-behavioural countermeasures were employed to alter arousal levels and processes in different ways in different 'at-risk' situations. A multiple baseline design was used to control for non-specific effects of interventions on non-targeted seizures. Results suggested significant declines in the number of sleep onset and startle-response seizures were attained by these methods. Gains were maintained at 2-month follow-up. ( info)

3/71. Lamotrigine for startle-induced seizures.

    Startle-induced seizures are reflex seizures precipitated by a sudden, surprising stimulus, usually auditory. Aetiologies, electroencephalographic correlates, and brain structural abnormalities are variable. Because of the frequent tonic component at onset, falling is a major clinical problem. There is no established drug of choice, and therapy is often unsatisfactory. Adjunctive lamotrigine therapy was used in four consecutive patients with this syndrome seen in a referral epilepsy practice. All four had been refractory to virtually every other drug, but responded dramatically to lamotrigine with elimination of falls from seizures. This observation may serve as pilot data for trials of lamotrigine for startle-induced or other varieties of reflex epilepsies, as adjunctive or monotherapy. ( info)

4/71. Hot water epilepsy: a benign and unrecognized form.

    Hot water epilepsy is a reflex epilepsy. seizures are provoked by hot water, and result from the association of both cutaneous and heat stimuli. Described mainly in india and japan, the condition seems to be rare in europe, where it occurs in young children. We report five infants aged from 6 months to 2 years. They had brief seizures during bathing with activity arrest, hypotonia, and vasoactive modification; clonic movements were observed. A simple treatment-decreasing the bath temperature-can be sufficient. Sometimes an antiepileptic drug is required. Seizure course and psychomotor development are favorable. Hot water epilepsy is a benign form of epilepsy. Its incidence could be underestimated because of confusion with febrile convulsions, vagal fits, or aquagenic urticaria. ( info)

5/71. Postoperative seizure outcome after corpus callosotomy in reflex epilepsy.

    Flickering light and color patterns, reading, language, movement, decision making, eating, tapping and touching, hot water immersion and auditory stimulation can induce seizures in some epileptic patients. These are known as the "reflex epilepsies". The mechanism of reflex epilepsy is not clear. Recently, we performed anterior two-thirds corpus callosotomies in two reflex epilepsy patients (ages 12 and 14 years), with follow-up for more than three years. Patient 1 had Lennox-Gastaut syndrome with auditory-induced generalized atonic or tonic seizures (startle epilepsy), which decreased by 60% after callosotomy. Patient 2 had Lennox-Gastaut syndrome with somatosensory-induced generalized tonic seizures (tap epilepsy). He was seizure-free for one year immediately after callosotomy, but his seizures recurred with the same degree and frequency as before surgery. The nonsignificant postoperative seizure outcome suggests that the corpus callosum only plays a partial role in seizure generation. Our report also discusses the possible mechanisms of generation of reflex seizures. ( info)

6/71. An established case of dentatorubral pallidoluysian atrophy (DRPLA) with unusual features on muscle biopsy.

    Dentatorubral pallidoluysian atrophy (DRPLA) belongs to the group of autosomal dominant ataxias. central nervous system pathology and inheritance are both well characterized, although the illness is rare. The presentation of a European child affected by this illness is described. He presented at 9 years of age with intractable progressive myoclonus epilepsy against a background of learning difficulties and developed progressive hypertonicity and dementia before his death at 15 years of age. Significant histological changes in a muscle biopsy were found. There was an absence of type IIB fibres and a predominance of type I fibres. Mean fibre diameter of all the fibre types was markedly reduced. All type I fibres showed an increase in lipid droplets. No previous descriptions exist of muscle histology in DRPLA. Although at least five adult family members have symptoms consistent with a diagnosis of DRPLA, their condition had not been recognized. We therefore describe the clinical picture and histological findings. ( info)

7/71. The relationship between photosensitive temporal lobe epilepsy and eye closure activity.

    We describe a 16-year-old female patient affected by photo-induced temporal lobe epilepsy. During intermittent photic stimulation she showed a photoparoxysmal response in the EEG. This case was diagnosed from clinical symptoms, single photon emission computer tomography, and EEG data. The clinical symptoms were relieved by the administration of carbamazepine. As these photoparoxysmal responses were observed not only during photic stimulation, but also when patient was closing her eyes during an eye-opening test in complete darkness, we propose the existence of an alternative pathway such as from the extraocular muscles or orbicularis oculi, or activation of cortical activity due to the change of consciousness by closing eyes in inducing photosensitive epilepsy. We describe an additional case and discuss a novel aspect of photo-induced temporal lobe epilepsy. ( info)

8/71. 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. ( info)

9/71. eating epilepsy. Heterogeneity of ictal semiology: the role of video-EEG monitoring.

    In this paper, three more cases of eating-induced seizures are reported. We have obtained ictal video-electroencephalogram (EEG) recordings for two patients, which confirm the heterogeneity of ictal semiology of these seizures. However, in all the cases the inclusion of this kind of reflex epilepsy (RE) among the localization-related epilepsies (LRE) is confirmed. The usefulness of video EEG monitoring in studying these seizures and reflex epilepsy in general (RE) is stressed. ( info)

10/71. Eyelid fluttering accompanying diffuse epileptic EEG induced by eye closure.

    A 13-year-old male exhibited the following clinical and electroencephalographic (EEG) manifestations. He exhibited marked photosensitivity on EEG from the first visit at 9 years of age until 11 years of age and had an episode of television epilepsy at 9 years of age. Generalized epileptic EEG findings followed by marked eyelid fluttering, induced by eye closure in a bright room that lasted until the eyes were opened, but not by eye closure in a dark room, were first noticed at 11 years of age. A generalized epileptiform EEG was not induced when the light was turned off if the eyes were kept open. His photosensitivity disappeared by 12 years of age, but the eyelid fluttering with epileptic EEG induced by eye closure was still present at 13 years of age. The epileptic EEG was thought to be induced by a modification of the retinal input on eye closure, not by mechanical or sensory stimulation on eye closure or fixation-off sensitivity. In addition, in this patient a change from photosensitive to eye closure-sensitive epilepsy seemed to occur, which suggests that the correlation between the epileptic EEG and retinal input are complex. ( info)
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