摘要:

Summary:The kynurenine pathway metabolites, quinolinic acid (QUIN) and L‐kynurenine are convulsants, whereas kynurenic acid (KYNA) is an antagonist of excitatory amino acid receptors. Imbalances in the concentrations of these metabolites have been implicated in the etiology of human seizure disorders. In the present study, L‐kynurenine and QUIN concentrations in both cerebrospinal fluid (CSF) and serum were reduced in patients with intractable complex partial seizures (CPS) in both the postictal period (15–75 min after a seizure) and the interictal period (absence of seizure for>24 h) as compared with neurologically normal control subjects. Linear regression analyses and analysis of covariance showed that the reductions in serum QUIN and L‐kynurenine were correlated to blood antiepileptic medication. L‐Tryptophan (L‐TRP) levels also tended to be lower in both CSF and serum of the seizure patients. CSF KYNA and serum 3‐hydroxykynurenine concentrations were not affected in seizure patients, whereas serum levels of KYNA were reduced. 3‐Hydroxykynurenine was not detected in the CSF of either control or seizure patients. The results do not support a role for a generalized reduction in KYNA concentrations or an increased ratio of QUIN:KYNA, or increases in CSF L‐kynurenine in initiation and maintenance of intra

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02428.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:The concept of the mirror focus (MF) implies that an actively discharging epileptiform region may induce similar paroxysmal behavior in a homologous site. In a group of patients with complex partial seizures (CPS) we investigated whether occurrence of a MF was influenced by certain clinical factors and whether surgical out‐come was influenced by the presence of an MF. Factors studied included age at onset, duration, and total number of seizures. Patients had had CPS for>3 years and had pathologically proven temporal lobe neoplasms. Seizure frequency was estimated by the history‐taking physician. We estimated total seizure number by multiplying frequency by duration. Seven patients had MF, and 15 did not. Mean age at onset of seizures, duration of seizure disorder, and total seizure number did not vary statistically between the two groups of patients. All patients with an MF except 1 were seizure‐free at follow‐up. Ten of the 15 patients without MF were seizure‐free. Three patients who were not seizure‐free had had subtotal resection owing to tumor overlap with eloquent cortex. We conclude that an MF is not a contraindication to operation even when the preponderance of interictal spike activity is contralateral to the tumor or when seizures appear to arise from the MF o

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02429.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:To determine the localizing value and electrophysiology of bimanual‐bipedal automatisms (BBAs), we studied these behaviors in 54 seizures of 8 patients with temporal or frontal lobe seizure onset. BBAs occurred with a frequency of 27% in frontal lobe epilepsy (FLE) and of 7% in temporal lobe epilepsy (TLE). The distribution of electrode sites showing ictal activity during these automatisms was significantly different in the two patient groups (0.0001 Chi‐square). Mesioand/or laterotemporal plus orbital frontal areas were involved areas when the behaviors appeared in patients with TLE; dorsolateral and mesiofrontal regions were the most commonly involved when the behaviors occurred during the course of frontal lobe seizures. We concluded that BBAs represent activation of frontal lobe circuitry but are not unique to seizures of frontal lobe origin. Eyelid flutter and repetitive body movements in either the axial or sagittal plane were significantly associated with the frontal lobe group whereas oral‐alimentary automatisms were associated with the temporal lobe group. Thus, these associated behaviors may help indicate whether a frontal or temporal lobe seizure onset has occurred when BBAs are observed. A new concept of ictal expression is proposed to conform with the results as well as with other apparently disparate ictal behaviors that may have localizing

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02430.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:Magnetoencephalographic (MEG) discharges were recorded with multichannel superconducting quantum interference device (SQUID) gradiometers in 13 young candidates for epilepsy surgery. The sources of epileptic activity were related to generators of somatosensory and auditory evoked cortical responses and projected on magnetic resonance imaging (MRI) scans. Seven subjects had restricted or regional MEG foci, located in the frontoopercular (1), sensorimotor (3), perisylvian (1), mesiotemporal (1), or temporooccipital cortex (1). The MEG foci in the 3 patients who underwent operation agreed with the intracranial findings. Findings in the other patients emphasize the need to collect further data to define the ultimate role of MEG in preoperative evaluation of epilepsy.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02431.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:We made a long term prospective study of 66 patients with juvenile myoclonic epilepsy (JME). Prevalence was 10.2% among 672 patients with epilepsies. Sex distribution was equal. Sixty‐three were not diagnosed on referral; JME was not initially recognized in the epilepsy clinic in 22. Clinical typical absence seizures were reported in 33.3%, myoclonic jerks in 97% and generalized tonic‐clonic seizures (GTC) in 78.8% of the patients. Mean age (±SD) at onset was 10.5 ± 3.4 years (range 5–16 years) for absence seizures, 15 ± 3.5 years (range 8–26 years) for myoclonic jerks, and 16 ± 3.5 years (9–28) for GTC. Absence predated myoclonic jerks by 3.9 ± 2.3 years (range‐1–9 years) and GTC by 4.4 ± 2.7 years (range 1–8 years) in 14 (21.2%) patients who manifested all three types of seizure. Absence were never antedated by myoclonic jerks or GTC. Myoclonic jerks occurred on awakening in 87.5% of the patients. GTC occurred mainly on awakening, but other patients had nocturnal or diurnal GTC with no circadian distribution. Neurologic examination was normal for all patients except for tremor of the hands similar to essential tremor, noted in 35% of patients. Computed tomography (CT) brain scans were normal: 93% of patients had precipitating factors: sleep deprivation (89.5%), fatigue (73.7%), photosensitivity (36.8%; television and video games 8.8%), menstruation (24.1% of women), mental concentration (22.8%), and stress (12.3%). Incidence of JME among siblings (13 of 41 examined families) implies an autosomal recessive mode of inheritance for this Arab population. EEGs were frequently normal in treated patients. At least one abnormal EEG was recorded in 56 (84.9%) patients. Abnormalities consisted mainly of generalized discharges of spike/double spike and/or polyspike and slow wave. Frequent multiple spikes and discharge fragmentations varied from 0.5to 20‐s duration (mean 6.8 s). Twenty (30.3%) had focal abnormalities, and 18 (27.3%) had photoconvulsive discharges. Eighty‐eight percent of patients remained seizure‐free for 3 years of follow‐up. Effective treatment was achieved with valproate (VPA); control of myoclonic jerks was improved with clonazepam (CZP). CZP monotherapy did not consistently prevent GTC. Adding small doses of CZP with simultaneous reduction of VPA was the most effective and better tolerated form of medication, particularly in patients demonstrating an adverse reaction or requiring a large VPA dosage. VPA dosage was successfully reduced in 15 patients who were seizure‐free for>2 years and had infrequent seizures before treatment, but 9 of 11 patients rel

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02432.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:A detailed study of EEGs of patients with an unequivocal diagnosis of juvenile myoclonic epilepsy (JME) showed a high prevalence of focal EEG abnormalities. Focal slow waves, spikes, and sharp waves and focal onset of the generalized discharge were present in 36.7% of EEGs in our patients with JME. In more than half of the patients, at least one EEG showed focal abnormalities. These features should not be misconstrued as indicative of partial epilepsy.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02433.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:We reviewed records of 85 patients with juvenile myoclonic epilepsy (JME) for significant asymmetries in clinical seizures or the EEG. We noted asymmetries in 26 of 85 patients (30.6%). Only 2 patients had both clinical and EEG asymmetries; 12 had clinical asymmetries and 12 had EEG asymmetries exclusively. Analysis of patients with and without asymmetries showed no statistically significant differences in comparisons of sex, age at seizure onset, family history of epilepsy, seizure type, or response to treatment. The delay in diagnosis was greater in JME patients with asymmetries (9.5 years) than in JME patients with no asymmetries (7.5 years), but this difference was not statistically significant. Fourteen of the 26 patients with asymmetries (53.8%) were initially misdiagnosed as having partial seizures. Asymmetries in JME patients are not only common, but are also a frequent cause of misdiagnosis.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02434.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:We studied myoclonic seizures (MS) in 5 patients with juvenile myoclonic epilepsy (JME) using video polygraphic recordings to investigate the clinical characteristics of MS in this epileptic syndrome. The total number of MS analyzed was 302 (range 27–125, mean 60) seizures per patient. MS occurred either singly or repetitively (37 vs. 63%) and corresponded to generalized bilaterally synchronous single or multispike‐and‐wave complexes at 3–5 Hz. Video analysis of the myoclonic jerks demonstrated that either distal or proximal muscle involvement predominated. In the former, there was mild bilateral flexion and some external rotation of the forearms. In the latter, flexion of both arms at the elbow, flexion and abduction of the thighs, and extension of the back was observed. Asymmetry of MS was noted in 4 of 5 patients. Facial involvement of MS occurred infrequently in 2 patients. When the patients kept both arms outstretched, the arms dropped or there was sudden interruption of ongoing electromyographic (EMG) potentials immediately after myoclonic jerks (postmyoclonic inhibition) in all patients. One should inquire about these clinical characteristics of MS in JME when taking a thorough history in patients with primary generalized tonic‐clonic seizu

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02435.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:Circling seizures (CS) have been described in association with focal lesions as well as with generalized EEG discharges. We report 1 patient with juvenile myoclonic epilepsy (JME) who developed CS. There were no focal findings on clinical examination, EEG, or imaging studies. We propose that CS in this patient may represent a profound asymmetry in expression of an idiopathic generalized epilepsy rather than a partial condition.

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02436.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Summary:In a study of 32 unrelated Arab patients with juvenile myoclonic epilepsy (JME), we compared the frequencies of human leukocyte antigen (HLA) class I and II alleles with those of unrelated healthy controls. A significant difference between the phenotypic frequencies in JME patients and controls was observed for DRW13, the split of DRW6 (37.5 vs 11% of controls). The strength of association as measured by the relative risk was 4.85 for this antigen (p = 0.002). The possible association of JME with HLA‐DRW6 recently reported in Caucasians was confirmed in this study. This finding speaks for the homogeneity of the disease among Arabic and Caucasian JME patients. The existence of this association is evidence of a locus in the HLA region that influences expression of JM

ISSN:0013-9580    

DOI:10.1111/j.1528-1157.1994.tb02437.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY