1/5. 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.- - - - - - - - - - ranking = 1keywords = consciousness (Clic here for more details about this article) |
2/5. Cardiac asystole in epilepsy: clinical and neurophysiologic features.PURPOSE: Cardiac asystole provoked by epileptic seizures is a rare but important complication in epilepsy and is supposed to be relevant to the pathogenesis of sudden unexplained death in epilepsy (SUDEP). We sought to determine the frequency of this complication in a population of patients with medically intractable epilepsy and to analyze the correlation between EEG, electrocardiogram (ECG), and clinical features obtained from long-term video-EEG monitoring. methods: Retrospective analysis of the clinical records of hospitalized patients from May 1992 to June 2001 who underwent long-term video-/EEG monitoring. RESULTS: Of a total of 1,244 patients, five patients had cardiac asystole in the course of ictal events. In these patients, 11 asystolic events, between 4 and 60 s long in a total of 19 seizures, were registered. All seizures had a focal origin with simple partial seizures (n = 13), complex partial seizures (n = 4), and secondarily generalized seizures (n = 2). One patient showed the longest asystole ever reported (60 s) because of a seizure. Cardiac asystole occurred in two patients with left-sided temporal lobe epilepsy (TLE) and in three patients with frontal lobe epilepsy (FLE; two left-sided, one bifrontal). Two patients reported previous cardiac disease, but only one had a pathologic ECG by the time of admission. Two patients had a simultaneous central ictal apnea during the asystole. None of the patients had ongoing deficits due to the asystole. CONCLUSIONS: These findings confirm that seizure-induced asystole is a rare complication. The event appeared only in focal epilepsies (frontal and temporal) with a lateralization to the left side. A newly diagnosed or known cardiac disorder could be a risk factor for ictal asystole. Abnormally long postictal periods with altered consciousness might point to reduced cerebral perfusion during the event because of ictal asystole. Central ictal apnea could be a frequent associated phenomenon.- - - - - - - - - - ranking = 1keywords = consciousness (Clic here for more details about this article) |
3/5. Prefrontal disturbances as the sole manifestation of simple partial nonconvulsive status epilepticus.We describe a case of frontal lobe epilepsy with rare nocturnal generalized tonic-clonic seizures and repeated prolonged episodes of altered behavior lasting 1 to 2 days. The changes consisted of poor organizational strategies, impaired set shifting, emotional indifference, reduced motivation, and impairment of emotional decision making. memory and consciousness were undisturbed, as she was able to follow her profession as a teacher. During the episode, the EEG was marked by right frontal rhythmical spikes and waves with spread to the homologous left region. Behavioral abnormalities and EEG changes were successfully treated with intravenous diazepam. The clinical representation can be ascribed to alterations of prefrontal-subcortical circuits, especially the anterior cingulate and orbitofrontal circuits. This unique case is classified as simple partial nonconvulsive status epilepticus with prefrontal disturbances as the sole manifestation.- - - - - - - - - - ranking = 1keywords = consciousness (Clic here for more details about this article) |
4/5. frontal lobe epilepsy with secondarily generalized 3 Hz spike-waves: a case report.We report a case of frontal lobe epilepsy with bursts of 3 Hz spike-wave, often dominant in the left frontal lobe, that sometimes developed into a secondary bilateral generalization. This patient was a 69-year-old male with a history of epileptic seizures that began 3 months following a head injury at the age of 17. His seizures occurred two or three times a day, but after medication the frequency was halved. The seizures began with a feeling of heaviness of the head, accompanied by myoclonus at both angles of the mouth. consciousness was clear for most of the seizures, which ranged from several seconds to many minutes. When seizures lasted for longer than 10 minutes, a temporary generalization accompanied by a loss of consciousness was sometimes observed. Neurological tests and cranial MRI yielded no abnormal local findings. The secondary generalized seizure seen in this case is the so called "secondary bilateral synchrony (SBS)." We succeeded in making an EEG recording of the bilateral generalization of a seizure discharge originating in the left frontal lobe, which until now has not been done. This case is direct proof that a primary frontal focus can give rise to SBS.- - - - - - - - - - ranking = 1keywords = consciousness (Clic here for more details about this article) |
5/5. Case report of a patient with complex partial frontal lobe seizures as a complication of bifrontal lobotomy.This case report describes an 85 year-old woman who underwent a bilateral frontal lobotomy for presumed schizophrenia 50 years ago. The patient presented with sudden alterations of consciousness accompanied by motionless staring and speech and movement arrest. After 5 to 10 seconds, contact recovered briskly. As there was electrophysiological evidence for a frontal epileptic focus, these fits were classified as complex partial frontal lobe seizures with impairment of consciousness at onset. An aetiological link with the bifrontal lobotomy is probable. At the occasion of this case report, complications of frontal lobotomy are reviewed.- - - - - - - - - - ranking = 2keywords = consciousness (Clic here for more details about this article) |