Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014547 (focal epilepsy)
 1,627 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ictal vomiting in patients with focal epilepsy has mostly been associated with an epileptogenic zone in the non-language-dominant hemisphere. Here we present the case of a left hemisphere language-dominant patient suffering from typical mesial temporal lobe epilepsy with histologically proven hippocampal sclerosis and ictal vomiting during complex partial seizures. He became seizure-free after selective left-sided amygdalohippocampectomy. This case implies that ictal vomiting may not necessitate invasive electrophysiological exploration of left hemisphere language-dominant patients with temporal lobe epilepsy if surface EEG and MRI indicate a left-sided epileptogenic zone. It thus corroborates that with concordant imaging and neurophysiological data, clinical signs become less valuable.
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PMID:Ictal vomiting in a left hemisphere language-dominant patient with left-sided temporal lobe epilepsy. 1633 34

The authors studied clinical characteristics in 11 patients with intractable focal epilepsy and preictal headache (PIHA) using a standardized interview. Headache was frontotemporal, ipsilateral to the focus, in nine patients with temporal lobe epilepsy (TLE) and contralateral in one with TLE and in one with frontal seizures. Migrainous features were found in four. After surgery, all seven seizure-free patients and two with rare seizures were free of PIHA. It may be a useful lateralizing sign in patients with TLE.
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PMID:Preictal headache in partial epilepsy. 1638 Jun 25

We report on two patients with reflex periodic spasms (PS) triggered by eating. Both patients also had significant cognitive and motor deficits. In both patients, reflex eating PS started during meal and occurred repeatedly at intervals of about 5-30 s, and the whole episode lasted about 10-15 min with 15-20 consecutive PS. Clinically, each PS was characterized by the trunk and head flexion, eyeball elevation, abduction of the upper limbs, and loss of consciousness. Ictal EEG recordings of PS revealed high-voltage sharp slow waves followed by a brief 1-2s voltage attenuation. Simultaneous EMG recording of right and left deltoid muscles revealed an abrupt increase of tone lasting 0.5-1.5s. In two patients, the origins of reflex PS were perirolandic suprasylvian and temporolimbic, respectively, because the first patient had focal epilepsy associated with cortical malformation including bilateral opercular dysplasia and the second one had cryptogenic temporal lobe epilepsy.
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PMID:Reflex periodic spasms induced by eating. 1641 21

Pharmacoresistant epilepsy arising from the dominant temporal region in patients with intact memory and normal anatomical imaging, presents major challenges in the preoperative definition of the epileptogenic zone, and the planning of the extent of the surgical resection. We report on the case of a 42-year-old, right-handed male who presented with recurrent daily seizures that were resistant to antiepileptic drugs. Multiple, non-invasive (scalp) video-EEG evaluations revealed focal epilepsy arising from the left fronto-temporal region. Multiple high resolution MRIs that were performed at multiple Epilepsy Centers failed to show any abnormality. Fluoro-deoxyglucose PET scan showed extensive, left antero-mesial temporal hypometabolism, and ictal SPECT showed increased perfusion in the left insula in addition to the left mesial and anterior temporal pole. Neuropsychological testing and intracarotid methohexital testing revealed excellent memory to the left, dominant side. A two-stage invasive evaluation with subdural grid electrodes followed by depth electrode recordings allowed the localization of the epileptogenic region to the temporal pole. A selective resection of the left temporal pole (that spared the hippocampal formation) resulted in a seizure-free outcome (one year follow-up) with no significant consequences on memory function. We conclude that targeted, invasive recording techniques should be used for the accurate localization and delineation of the extent of the epileptogenic zone in cases of suspected, non-lesional, dominant hemisphere, temporal lobe epilepsy with preserved memory function. The use of the staged invasive approach may increase the chances for memory (function) sparing through tailored, temporal resection.
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PMID:Definition of the epileptogenic zone in a patient with non-lesional temporal lobe epilepsy arising from the dominant hemisphere. 1701 70

Impaired transmission in GABAergic circuits is thought to contribute to the pathogenesis of epilepsy. Although it is well established that major reorganization of GABA(A) receptor subtypes occurs in the hippocampus of patients with medically refractory temporal lobe epilepsy (TLE), it is unclear whether this disorder is also associated with alterations in GABA(A) receptor subtypes in the neocortex. Here we have investigated immunohistochemically the subunit composition and neocortical distribution of three major GABA(A) receptor subtypes using antibodies specifically recognizing the subunits alpha1, alpha2, alpha3, beta2/3 and gamma2. Cortical tissue was obtained at surgery from patients with TLE and hippocampal sclerosis (HS; n = 9), TLE associated with neocortical lesions (non-HS; n = 12) and frontal lobe epilepsy (FLE; n = 5), with post-mortem samples serving as controls (n = 4). A distinct laminar and neuronal expression pattern of the alpha-subunit variants was found across the neocortical regions examined in the temporal and frontal lobes in both control and patient tissue samples. In the five patients with FLE, GABA(A) receptor subunit staining was unchanged as compared to controls. In patients with TLE we observed a marked decrease in alpha3-subunit staining in the superficial neocortical layers (I-III), but no change in the deep layers (V and VI) or in the expression pattern of the alpha1 and alpha2-subunits. Reduced expression in alpha3-containing GABA(A) receptors was detected in six out of nine patients of the HS group and four out of twelve patients of the non-HS group. Histopathological changes were present in eight out of the ten patients with decreased alpha3-subunit staining. The selective reduction in alpha3-containing GABA(A) receptors was confirmed using semiquantitative measurements of optical density (OD). The specific changes unique to alpha3-subunit expression in the superficial neocortical layers of patients with TLE suggest that this subtype is of particular significance in the reorganization of cortical GABAergic systems in focal epilepsy.
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PMID:Altered expression of alpha3-containing GABAA receptors in the neocortex of patients with focal epilepsy. 1704 56

A cerebral network comprising precuneus, medial frontal, and temporoparietal cortices is less active both during goal-directed behavior and states of reduced consciousness than during conscious rest. We tested the hypothesis that the interictal epileptic discharges affect activity in these brain regions in patients with temporal lobe epilepsy who have complex partial seizures. At the group level, using electroencephalography-correlated functional magnetic resonance imaging in 19 consecutive patients with focal epilepsy, we found common decreases of resting state activity in 9 patients with temporal lobe epilepsy (TLE) but not in 10 patients with extra-TLE. We infer that the functional consequences of TLE interictal epileptic discharges are different from those in extra-TLE and affect ongoing brain function. Activity increases were detected in the ipsilateral hippocampus in patients with TLE, and in subthalamic, bilateral superior temporal and medial frontal brain regions in patients with extra-TLE, possibly indicating effects of different interictal epileptic discharge propagation.
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PMID:Temporal lobe interictal epileptic discharges affect cerebral activity in "default mode" brain regions. 1713 85

The differential diagnosis between panic disorder and focal epilepsy may sometimes pose a serious challenge. We report the case of a 32-year-old woman who complained of paroxysmal episodes of acute anxiety that evaded diagnosis for 8 years. Standard EEGs and brain CT scan showed no clear pathologic findings. Antidepressants, support psychotherapy and several courses of antiepileptic drugs were not beneficial. She was referred to our centre for a comprehensive diagnostic assessment. Clinical and standardized personality assessment did not reveal the personality organization typically associated with proneness to develop phobic anxiety disorders. Also, agoraphobic avoidance was absent, and the patient's main worries during the episodes involved negative social judgments rather than health. A brain MRI revealed a slightly increased signal at FLAIR images in the right amygdala. Video-EEG monitoring was decisive in establishing the diagnosis of drug-resistant right mesial temporal lobe epilepsy. Anteromesial temporal lobectomy was offered and successfully performed. Pathological examination of removed brain tissue revealed amygdalar sclerosis and mild hippocampal neuronal loss. At a 6-month follow-up visit, the paroxysmal episodes had completely disappeared. Depression, anxiety and quality of life were markedly improved. This case suggests that focal epilepsy should be considered in patients with paroxysmal episodes of anxiety, especially if dissociative symptoms and atypical clinical features for panic disorder are present, and if there is no satisfactory response to adequate trials of medication and psychotherapy within one year. A careful psychopathological analysis rather than a descriptive enumeration of symptoms is needed to bring these features to light. In such cases, even if routine EEGs or MRI are inconclusive and there is no response to antiepileptic drugs, it would be advisable to perform video-EEG monitoring to rule out partial seizures.
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PMID:Partial seizures due to sclerosis of the right amygdala presenting as panic disorder. On the importance of psychopathological assessment in differential diagnosis. 1733 38

Proton magnetic resonance spectroscopy ((1)H MRS) is beneficial in the lateralization of the epileptogenic zone in temporal lobe epilepsy; however, its role in extratemporal and, especially, MRI-negative epilepsy has not been established. This study seeks to verify how (1)H MRS could help in localizing the epileptogenic zone in patients with MRI-negative extratemporal epilepsy. Seven patients (8-23 years) with MRI-negative refractory focal epilepsy were studied using (1)H MRS on a 1.5T MR system. Chemical shift imaging sequence in the transversal plane was directed towards the suspected epileptogenic zone localized by seizure semiology, scalp video/EEG, ictal SPECT and (18)FDG-PET. Spectra were evaluated using the program CULICH, and the coefficient of asymmetry was used for quantitative lateralization. MRS detected lateralization in all patients and was able to localize pathology in five. The most frequent findings were decreased ratios of N-acetylaspartate to choline compounds characterized by increasing choline concentration. The localization of the (1)H MRS abnormality correlated well with ictal SPECT and subdural mapping. In all cases, histopathological analysis revealed MRI-undetected focal cortical dysplasias. (1)H MRS could be more sensitive for the detection of discrete malformations of cortical development than conventional MRI. It is valuable in the presurgical evaluation of patients without MRI-apparent lesions.
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PMID:(1)H MR spectroscopic imaging in patients with MRI-negative extratemporal epilepsy: correlation with ictal onset zone and histopathology. 1734 Jan 2

Newer MRI methods can detect cerebral abnormalities not identified on routine imaging in patients with focal epilepsy. Correlation of MRI with histopathology is necessary to understand the basis of MRI abnormalities and subsequently predict histopathology from in vivo MRI. The aim of this study was to determine if particular quantitative MR parameters were associated with particular histological features. Nine patients with temporal lobe epilepsy were imaged at 1.5 T using standard presurgical volumetric and quantifiable sequences: magnetization transfer and FFT2. The resected temporal lobe was registered with the volumetric MRI data according to our previously described method to permit correlation of the modalities. Stereologically measured neuronal densities and field fraction of GFAP, MAP2, synaptophysin and NeuN immunohistochemistry were obtained. Analyses were performed in the middle temporal gyrus and compared with quantitative MRI data from the equivalent regions. There was a significant Spearman Rho negative correlation between NeuN field fraction and the T2 value in gray matter (correlation coefficient -0.72, p=0.028). There were no significant correlations between any neuropathological and MR measures in white matter. These preliminary findings suggest that T2 in gray matter is sensitive to the proportion of neuronal tissue. Novel quantitative MRI measures acquired with higher field strength magnets, and so with superior signal to noise ratios, may generate data that correlate with histopathological measures. This will enable better identification and delineation of the structural causes of refractory focal epilepsy, and will be of particular benefit in patients in whom current optimal MRI does not identify a relevant abnormality.
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PMID:Correlation of quantitative MRI and neuropathology in epilepsy surgical resection specimens--T2 correlates with neuronal tissue in gray matter. 1755 88

Epilepsy is a common neurological disorder with an incidence of approximately 0.5%. In order to develop better strategies for treatment of epilepsy, more insight on the etiology and pathogenesis of epilepsy is required. In 2001, based on the diagnostic scheme of the International League Against Epilepsy, three new forms of familial epilepsy were identified. These include familial temporal lobe epilepsy, familial focal epilepsy with variable foci, and generalized epilepsy with febrile seizure plus. Mutation of a distinct set of genes has been reported in several forms of epilepsy. Mutation of LGI1 gene has been identified in familial lateral temporal lobe epilepsy while mutations of genes which encode sodium channels and GABAA receptors have been reported in generalized epilepsy with febrile seizure plus. However, no disease-causing gene has yet been found in families with familial mesial temporal lobe epilepsy or those with familial focal epilepsy with variable foci. Here, we review the genetic background of these three familial epilepsy syndromes, and provide a better insight on their genetic etiology.
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PMID:Genetic etiology of new forms of familial epilepsy. 1798 85


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