Cases reported "Epilepsy, Frontal Lobe"

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1/78. A case of startle epilepsy and SSMA seizures documented with subdural recordings.

    PURPOSE: To study the mechanisms of startle-induced supplementary sensorimotor area (SSMA) seizures. methods: We present a patient investigated with indwelling subdural grid electrodes covering both the SSMA and the dorsolateral frontal lobe. RESULTS: We found a simultaneous seizure onset in the right dorsolateral premotor cortex and the right SSMA. High-resolution magnetic resonance imaging (MRI) showed a small subcortical lesion adjacent to the right SSMA. The patient became seizure free after resection of the lesion and the ictal-onset zone. CONCLUSIONS: We conclude that an extended region of abnormally excitable tissue within the frontal lobe could facilitate the generation of startle-induced seizures. We speculate that a widespread epileptogenic zone could help to explain why some patients with SSMA or dorsolateral frontal lobe seizures are more likely to have startle-induced seizures.
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2/78. Motiveless firesetting: implicating partial limbic seizure kindling by revived memories of fires in "Limbic Psychotic Trigger Reaction".

    23 unselected juvenile firesetters (M age 12.0 yr.) consisted of seven with schizophrenia, three with organic mental disorder, six with posttraumatic stress disorder, two with severe mental retardation, and two with conduct disorders. Three previously nondestructive boys (M age 11.0 yr.), all of them loners, did not fit such traditional diagnoses. Their fleeting (c. 20 min.) symptoms included flat affect, autonomic arousal, and delusions or hallucinations. It appeared that their motiveless, unplanned acts were each preceded by a chance encounter with an individualized stimulus which revived the three boys' repeatedly ruminated memories of intermittently experienced merely moderate stresses associated with fire, smoke, or matches. Such a sequence of events is characteristic of seizure kindling. One boy's abnormal EEG was congruent with seizures in the temporal lobe area, which includes the amygdala, i.e., that part of the limbic system particularly susceptible to seizure kindling. The three boys' consistent symptomatology was very similar to that reported for 17 men with bizarre homicidal acts implicating a kindled partial seizure called "Limbic Psychotic Trigger Reaction." In primates, too, similar partial nonconvulsive "behavioral seizures" with psychosis-like symptoms can be elicited through experiential kindling.
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3/78. Circling seizures in a case with Wilson's disease.

    We report a case of Wilson's disease with circling seizures. Because of the existence of other types of frontal automatism and the EEG focus on the frontal regions, circling seizures of the patient were thought to originate from the frontal lobe. magnetic resonance imaging demonstrated large cavitary lesions on bilateral frontal lobes. The mechanisms of circling behavior are discussed in association with Wilson's disease.
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4/78. Electroclinical picture of autosomal dominant nocturnal frontal lobe epilepsy in a Japanese family.

    PURPOSE: Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is the first described partial epilepsy syndrome known to be due to a single gene mutation. We found a first Japanese ADNFLE family with a novel mutation of the neuronal nicotinic acetylcholine receptor (nAChR) alpha4 subunit (CHRNA4) gene. The aim of this report is precisely to describe the electroclinical manifestations of ADNFLE in this family and to compare these findings with those of other families reported previously in the literature. methods: Three affected family members were investigated electroclinically by close clinical observation, interictal EEG, video-EEG monitoring, magnetic resonance imaging, and single-photon-emission tomography. Information about other affected family members was obtained from either the spouse or the parents. Mutations within the CHRNA4 gene were examined in seven family members. RESULTS: The clinical manifestations and diagnostic findings in the members of this family were consistent with ADNFLE. However, there were intrafamilial and interfamilial variations in clinical features. The seizures of the patients were brief tonic seizures, with hyperventilation in children and secondarily generalized tonic-clonic convulsions in adults. The onset of the children's seizures began in infancy and early childhood. The children's seizures were sometimes provoked by movement and sound stimulation, and did not respond to antiepileptic drugs. On the other hand, the adults' seizures disappeared spontaneously or were easily controlled with carbamazepine. Three children showed hyperactivity, and two children had mild mental retardation. All patients had impaired consciousness during their seizures and no auras. A novel missense mutation (c755C>T) in exon 5 of the CHRNA4 gene was found in four affected family members. CONCLUSIONS: The electroclinical pictures of a Japanese family with ADNFLE were basically the same as those of other families reported, but with slight differences. ADNFLE is probably not uncommon, and it is very likely that there are unidentified patients with this inherited disorder in japan.
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5/78. Systematic approach to dipole localization of interictal EEG spikes in children with extratemporal lobe epilepsies.

    OBJECTIVES: To assess the reliability of dipole localization based on residual variances (RV), using equivalent current dipole analysis of interictal EEG spikes in children with extratemporal lobe epilepsy. methods: Four pediatric patients with extratemporal lobe epilepsy were studied. Digital EEG was recorded from 19 scalp electrodes. Computer programs for spike detection and clustering analysis were used to select spikes. Dipoles were calculated 5 times for each spike using different initial guesses by the moving dipole model. Standard deviation (SD) of the dipole positions was calculated at each time point in the 5 trials. RESULTS: We analyzed the dipoles at 1097 time points from 4 patients. Among 106 time points with RV < 2%, the SD was < 1 mm in 78 (74%), while in those with SD > 1 mm the dipole positions varied between 2.8 and 52.6 mm. Of dipoles with RV < 1%, 26 of 27 (96%) had an SD < 1 mm; the one dipole with SD > 1 mm varied within 2.5 mm. The dipole localizations with RV < 2% corresponded to the epileptogenic zones identified on intracranial invasive video EEG and intraoperative ECoG. CONCLUSIONS: The systematic approach of equivalent current dipole analysis using spike detection, clustering analysis, and an RV < 2% as a standard is useful for identifying extratemporal epileptic regions.
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6/78. Secondary bilateral synchrony due to fronto-mesial lesions. An invasive recording study.

    frontal lobe epilepsies may present difficulties in focus localization in the pre-operative work-up for epilepsy surgery. This is specially true in patients with normal MRIs. We report on a 16 years-old girl that started with seizures by the age of 8 years. They were brief nocturnal episodes with automatisms such as bicycling and boxing. Seizure frequency ranged from 4-10 per night. scalp EEG showed few right frontal convexity spiking and intense secondary bilateral synchrony (SBS). High resolution MRI directed to the frontal lobes was normal. Ictal SPECT suggested a right fronto-lateral focus. Ictal video-EEG showed no focal onset. She was submitted to invasive recordings after subdural plates implantation. electrodes covered all the frontal convexity and mesial surface bilaterally. Ictal recordings disclosed stereotyped seizures starting from the right mesial frontal. Using a high-resolution tool to measure intra and interhemispheric latencies, the timing and direction of seizure spread from the right fronto-mesial region were studied. Motor strip mapping was performed by means of electrical stimulation. She was submitted to a right frontal lobe resection, 1.5 cm ahead of the motor strip and has been seizure free since surgery (8 months). Pathological examination found a 4 mm area of cortical dysplasia. Invasive studies are needed to allow adequate localization in patients with non-localizatory non-invasive work-up and may lead to excellent results in relation to seizures after surgery.
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7/78. Chronological progression of a language deficit appearing to be postictally reversible in a patient with symptomatic localization-related epilepsy.

    A language deficit occurring interictally, with chronological progression, and postictally in a patient with symptomatic localization-related epilepsy, which began at 1.6 years of age, is reported. The patient was a 30-year-old right-handed man whose seizures seemed to originate from the left frontal lobe and to involve the left temporal lobe. The deficit in oral language consisted mainly of features of motor aphasia, including delayed initiation of speech with great effort, echolalic and palilalic tendencies, and word-finding difficulty, but he also showed features of sensory aphasia. Written language had agraphia observed in sensory aphasia, including well-formed letters, paraphasias, neologisms, and paragrammatism. Postictally, the language deficit appeared to be superficially reversible, and evolved from mutism through non-fluent jargon to the interictal level of language. Analysis of the patient's diaries from 10 to 26 years of age disclosed chronologically progressive deterioration of language with paragrammatism, showing an increase of grammatical errors, neologismus, literal and verbal paraphasias and misconstruction of sentences. The results suggest that localization-related epilepsy of presumably left frontal lobe origin causes not only a postictal language deficit but also a slowly progressive deficit of language function.
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8/78. Cryptogenic gelastic epilepsy of frontal lobe origin: a paediatric case report.

    Gelastic (laughing) seizures are an uncommon seizure type which in most cases has an organic cerebral pathology and specifically a hypothalamic hamartoma. The interictal EEG frequently shows focal activity. This report describes a 3 1/2-year-old boy who presented with episodes of unmotivated laughter associated with other epileptic symptomatology before the age of 3 years. Prolonged ambulatory EEG monitoring recorded electroclinical seizures starting in the right frontal area and spreading to the adjacent frontotemporal region. Neurological examination and brain magnetic resonance imaging were normal. vigabatrin resulted in immediate remission of the seizures and normalization of the EEG.
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9/78. Reduction of benzodiazepine receptor binding is related to the seizure onset zone in extratemporal focal cortical dysplasia.

    PURPOSE: Comparison of regional reduction of GABA receptor binding and seizure onset zone in patients with extratemporal epilepsy due to focal cortical dysplasia. methods: Two patients with frontal lobe epilepsy who remained seizure free after partial frontal lobe resection were investigated with magnetic resonance imaging, positron emission tomography (PET) with 18F-fluoro-deoxy-glucose (FDG) and 11C-flumazenil, subdural EEG-video recordings, and postoperative benzodiazepine (BDZ)-receptor autoradiography. RESULTS: The area of reduced BDZ-receptor binding as documented by preoperative flumazenil-PET and postoperative BDZ-receptor autoradiography corresponded to the seizure onset zone and was smaller than the interictal hypometabolism documented by FDG-PET. CONCLUSION: flumazenil-PET is a useful tool for localization of the epileptogenic zone in patients with extratemporal epilepsy caused by focal cortical dysplasia. Neuronal distribution of BDZ-receptor density confirms in vivo flumazenil-PET findings. The regional reduction of BDZ-receptor binding in focal cortical dysplasia seems to be confined to the seizure onset zone and not to the extent of dysplastic cortex.
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10/78. Gelastic seizures: video-EEG and scintigraphic analysis of a case with a frontal focus; review of the literature and pathophysiological hypotheses.

    We report scalp EEG and SPECT findings in a young patient who experienced gelastic seizures; clinical, EEG and scintigraphic data strongly suggested a frontal focus in a context of cryptogenic epilepsy. Few cases of gelastic seizures originating in the frontal lobe have been reported in the literature, most of them involving a diencephalic hamartoma or a temporal focus although, no clinical pattern has been found to be specific for each of these three anatomical regions. The ictal laughter is of variable nature, unmotivated or associated with feelings of mirth, forced or natural, except in the case of a frontal focus where the laughter seems consistently described as forced and unmotivated. However, mirth and laughter are two dissociable clinical elements; their genesis probably involves distinct mechanisms. Anatomical considerations lead to several hypotheses concerning laughter generation: it could be a simple reactional behavior in response to a modified cognitive process, an automatic behavior or a forced action. In a few cases with a temporal focus, laughter seems directly related to a disorganization of the associative temporal cortex and may be considered as a reactional behavior. In cases with frontal focus, anterior cingulate and orbital structures would be particularly implicated in laughter genesis, although with possible different pathophysiological routes: in the first case by disconnection within the premotor mesial system or by an imbalance between premotor mesial and premotor lateral systems, and in the second case by activation of a previously conditioned orbital region.
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