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1/425. Serial electroencephalographic findings in patients with MELAS.

    To clarify the electroencephalographic characteristics of mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), the medical records and electroencephalograms of six patients with MELAS and two of their relatives with MELA (mitochondrial myopathy, encephalopathy, and lactic acidosis, without strokelike episodes) were retrospectively reviewed. All have a point mutation in the mitochondrial dna at nucleotide position 3243. The electroencephalograms (n = 79) were divided into four groups according to the time relation to the strokelike episode: (1) before the first strokelike episode, (2) within 5 days after the strokelike episode (acute stage), (3) between 6 days and 1 month after the strokelike episode (subacute stage), and (4) more than 1 month after the strokelike episode (chronic stage). In the acute stage, 10 of the 11 electroencephalograms (9 strokelike episodes in four patients) revealed focal high-voltage delta waves with polyspikes (FHDPS), which were recognized as ictal electroencephalogram. Ictal events during FHDPS included focal clonic or myoclonic seizure and migrainous headache. In the subacute and chronic stages, focal spikes or sharp waves and 14- and 6-Hz positive bursts were frequently recorded. The authors' results suggest that FHDPSs present a reliable and accurate indicator of a strokelike episode in patients with MELAS.
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ranking = 1
keywords = spike
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2/425. seizures induced by frustration and despair due to unresolved moral and political issues: a rare case of reflex epilepsy.

    We present a case of reflex-induced simple partial seizures, triggered by feelings of frustration, anger and despair. Such emotions were provoked by pondering over complex national and international, political and moral issues. The present case may suggest that activation of right temporal networks may mediate negative and adverse emotions induced by preoccupation with agitating, controversial issues.
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ranking = 46.752874760111
keywords = epilepsy
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3/425. Stabilizing seizure disorders.

    Scientific understanding of seizures and epilepsy is rapidly evolving. Recent advances in diagnosis, classification, and medical and surgical treatment have significantly altered our approach to patients with these conditions.
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ranking = 11.688218690028
keywords = epilepsy
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4/425. Interictal change in cardiac autonomic function associated with EEG abnormalities and clinical symptoms: a longitudinal study following acute deterioration in two patients with temporal lobe epilepsy.

    The purpose of the present study was to investigate the ictal and interictal changes in cardiac autonomic function (CAF), and the relationship between the interictal change in CAF to the electroencephalogram (EEG) and clinical findings. In two patients with temporal lobe epilepsy (TLE) showing acute deterioration, a quantitative evaluation of their interictal CAF based on heart rate variability and their EEG using spectral analysis was conducted, and the findings compared with repeated clinical evaluations during the recovery period. The ictal heart rate changes and their temporal relationship to ictal discharge were investigated using simultaneous EEG/electrocardiogram (ECG) monitoring in one of the patients. Interictal parasympathetic function was decreased during the period of acute deterioration, but was increased in association with improvements in the EEG and clinical findings. In contrast, the sympathetic function showed no specific changes. The ictal discharges were preceded by a brief bradycardia, with a long delay of up to 40s. The results demonstrated that this decrease in parasympathetic function was closely related to the interictal changes in central nervous system function. On the other hand, the ictal discharges in one of the patients were thought to have caused a transient elevation of parasympathetic function. It is strongly suggested that patients with TLE have interictal as well as ictal changes in CAF that are mediated mainly through the parasympathetic nervous system.
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ranking = 58.441093450139
keywords = epilepsy
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5/425. A case of epileptic negative myoclonus: therapeutic considerations.

    This study presents a patient with epileptic negative myoclonus who showed interictal focal epileptic discharges in the centrotemporal region. The patient's seizures were exacerbated by carbamazepine, zonisamide, and valproate, but completely controlled by ethosuximide, and were suggested to have some relation with thalamocortical oscillation mechanisms. ethosuximide is supposed to be a drug of worth to try to use in epileptic negative myoclonus patients with centrotemporal spike foci.
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ranking = 0.5
keywords = spike
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6/425. Behavioral psychophysiological intervention in a mentally retarded epileptic patient with brain lesion.

    Behavioral psychophysiological treatment entailing Slow Cortical Potential (SCP) biofeedback training and behavioral self-control training was conducted with a 27-year-old male epileptic patient (seizures for 23 years) with Wechsler IQ 64 who underwent callosotomy. The patient had 12/week secondary generalized tonic-clonic seizures. The treatment, consisting of 43 SCP training sessions and 22 behavioral control sessions, yielded a highly significant reduction of seizure frequency to about 7.5/week; such a decrease had never been observed after administration of new anticonvulsant drugs, nor after the callosotomy. During SCP feedback training, the patient was able to produce highly-significant cortical differentiation of SCPs of about 4 microV. In addition, he developed several new behaviors indicating growing ability of self-perception and self-regulation. These findings suggest that a combination of SCP biofeedback with behavioral treatment of epilepsy can be used even in mentally retarded patients with organic brain disorders.
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ranking = 11.688218690028
keywords = epilepsy
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7/425. amitriptyline-associated seizures in a toddler with Munchausen-by-proxy.

    We describe an unusual case of a toddler diagnosed with an idiopathic seizure disorder that later was proved to be caused by deliberate administration of amitriptyline by his custodian. In spite of seizures associated with widened electrocardiographic wave (QRS) and right axis deviation on the electrocardiogram (EKG), the correct diagnosis eluded clinicians through a series of hospital admissions. Unfortunately, clinicians are quite accustomed to the fact that patients previously diagnosed with epilepsy have seizures and may not investigate other causes of seizure. This allowed classic signs of cyclic antidepressant poisoning to go unrecognized.
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ranking = 11.688218690028
keywords = epilepsy
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8/425. X-linked mental retardation syndrome with seizures, hypogammaglobulinemia, and progressive gait disturbance is regionally mapped between xq21.33 and Xq23.

    We identified a family with three males in two generations with moderate mental retardation. The two oldest were first cousins whose mothers were sisters. The third affected was a grandson through a daughter of one of the sisters, strongly suggesting X- linked inheritance. The affected males had prominent glabella, synophrys, prognathism, generalized hirsutism, and bilateral single palmar creases. All developed seizures in childhood. The two oldest have had a slow deterioration in neurological status with poor gait and balance and progressive weakness. No deterioration in their mental status has been observed. The oldest had cerebellar atrophy confirmed on computed tomography and magnetic resonance imaging scans of the brain and prolonged nerve conduction velocity. Two of the males had hypogammaglobulinemia (IgA deficient). Two-point linkage analysis using 27 microsatellite markers on the x chromosome resulted in a maximum lod score of 2.23 at straight theta = 0 for locus DSX101. Recombination was observed at locus DSX1170 in Xq21.33 and locus DXS8067 in Xq23. We conclude that this family represents an X-linked disorder associated with a recognizable phenotype, progressive neurological deterioration, and variable hypogammaglobulinemia. The gene appears to lie between Xq21.33 and Xq23.
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ranking = 0.004347506084971
keywords = childhood
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9/425. Nonconvulsive status epilepticus in childhood localization-related epilepsy.

    PURPOSE: To report on three children with localization-related epilepsy who exhibited minor seizures (atypical absences, brief atonic, and myoclonic) and nonconvulsive status epilepticus (NCSE) consisting of these minor seizures, and to elucidate their significance. methods: We studied the electroclinical characteristics of these children. Ictal electroencephalograms (EEGs) of NCSE were evaluated by using simultaneous video-EEG-electromyogram (EMG) polygraphic recordings. RESULTS: All patients began to have partial seizures between the ages of 6 months and 2 years 7 months, with minor seizures appearing later, between the ages of 1 year 11 months and 6 years 6 months. These minor seizures evolved into NCSE. Complex partial seizures remained after suppression of the minor seizures. Interictal EEGs taken when the minor seizures appeared showed excessive diffuse epileptic discharges in addition to multifocal spike-waves. Before and after suppression of the minor seizures, focal epileptic discharges predominated on the EEGs. On ictal EEGs of brief atonic and myoclonic seizures, diffuse spike-wave and polyspike-wave bursts were detected. Ictal EEGs of the atypical absences revealed diffuse spike-wave bursts mixed with irregular high-voltage slow waves, often interspersed with brief atonic and myoclonic seizures. When atypical absences lasted for a long time, patients manifested NCSE. Polytherapy might be related to the occurrence of minor seizures and NCSE, because all patients were treated with polytherapy at their appearance, and simplification of antiepileptic drug (AED) therapy seemed to be effective. CONCLUSIONS: We concluded that this NCSE is a type of atypical absence status which is an age-dependent, transient, electroclinical condition. The mechanism of occurrence of these minor seizures might be related to secondary bilateral synchrony.
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ranking = 60.458483474479
keywords = epilepsy, spike, childhood
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10/425. Post-ictal psychosis: an unusual clinical entity.

    OBJECTIVE: The aim of this paper is to describe a case of post-ictal psychosis (PIP) and to discuss the literature surrounding this interesting disorder. CLINICAL PICTURE: A 32-year-old woman with epilepsy presented on three occasions with psychosis following tonic-clonic seizures. TREATMENT AND OUTCOME: The psychosis resolved within 14 days on haloperidol 7 mg daily. CONCLUSIONS: Post-ictal psychosis is an unusual clinical entity that is frequently recurrent and may be misdiagnosed and treated inappropriately. Prophylaxis of PIP is an area for important future research.
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ranking = 11.688218690028
keywords = epilepsy
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