Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Generalised 3 Hz spike wave (SW) discharges with or without absences have been described in children with benign epilepsy with centrotemporal spikes (BECTS), leading to speculations about a continuum between childhood absence epilepsy (CAE) and BECTS. We thus decided to evaluate the prevalence of absence seizures (AS) and generalised 3 Hz SW in patients with BECTS. All patients with BECTS first referred since 1986 have been identified prospectively. Their medical and electroencephalograph (EEG) records were analysed retrospectively, in the search for AS and generalised SW discharges. Over a period of 11 years, we found typical rolandic spikes in 66 newly referred patients; 64 had seizures typical for the condition (18 female, 46 male), two were asymptomatic and were not further analysed. All had routine waking EEG recordings, and 49 children (76%) had at least one sleep EEG. AS or classical generalised 3 Hz SW were never recorded from history or EEG data, respectively. However, 17 patients had some diffuse SW discharges, lasting 1-5 s, which appeared as grossly symmetrical in only seven children, with a clearly asymmetrical aspect in the others. Among these seven patients, the discharges were only seen on awakening in one, both during waking and nREM sleep stage I or II in one and only during nREM sleep stage I or II in five. They were apparently subclinical in all. We thus found neither AS nor classical 3 Hz SW discharges among 64 consecutive patients with BECTS. Brief bursts of bilateral abnormalities occur in about 25% of the cases, mostly with sleepiness. Such findings do not substantiate the existence of a continuum between CAE and BECTS.
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PMID:Are there generalised spike waves and typical absences in benign rolandic epilepsy? 1048 73

A 43-year-old, right-handed woman experienced right hand paresthesias and a visual field abnormality. We attributed her symptoms to psychiatric abnormalities, due to the presence of delusions and auditory hallucinations. Upon photostimulation, she experienced left visual field hallucinations and demonstrated slow waves on the right parieto-occipital regions. The clinical and electro-encephalographic findings suggested that these episodes were epileptic seizures originating from the right occipital region. Ictal fear appeared at the end of the seizure, reflecting the spread of seizure activity to the mesial temporal region. Ictal SPECT images showed hyper-perfusion in the right occipital region and left cerebellar cortex. rCBF in the occipital lobe was significantly asymmetrical. When we encounter an epileptic patient with psychosis who has a visual hallucination, we should consider the possibility of epileptic seizure originating from the occipital lobe.
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PMID:[Ictal visual hallucination intermittent photic stimulation: using evaluation of the clinical findings, ictal EEG, ictal SPECT, and rCBF]. 1051 57

This report describes the observations of two patients with a several years' history of multiple sclerosis who presented sudden neurologic impairment. The symptomatology was suggestive of a non-convulsive partial status epilepsy. The clinical presentation was a paroxysmal dysphasic phenomenon in the first case without any consciousness impairment, associated with slight right hemiparesis. Electroencephalographic investigations revealed asymmetrical patterns, left-sided slow waves and periodic lateralized epileptiform discharges (PLEDs). Antiepileptic treatments were partially effective and intravenous steroids were needed for complete recovery. For the second patient, clinical presentation was acute psychiatric symptoms with disorientation, alternating manic symptomatology and mutism. Electroencephalography showed left fronto-central rhythmic continuous slow wave and spike wave activity. Intravenous antiepileptic treatment quickly improved the symptomatology. These observations draw attention to the fact that an epileptic cause should not be ruled out when a patient with multiple sclerosis presents sudden neurologic or psychiatric impairment. An early diagnosis allows immediate antiepileptic treatment. Intravenous steroids can be added to stop seizures.
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PMID:[Partial non-convulsive status epilepsy in multiple sclerosis]. 1067 21

The interhemispheric difference of the motor-cortical threshold (IDMT) was studied with focal magnetic transcranial stimulation (TCS) in ten patients with idiopathic generalized epilepsy (IGE) who also displayed versive or circling seizures (IGEvc). The data were compared with those obtained from two control groups; 13 patients with IGE without asymmetrical motor seizures, and 25 normal volunteer subjects. The IDMT, referred to as the percentage of maximum stimulator output, was assessed by focal TCS applied to the hand areas. Seven patients with IGEvc and only one patient with IGE had an interhemispheric motor threshold beyond the normal range. The IDMT in IGEvc patients was significantly higher compared to that of IGE patients and normal individuals. An interhemispheric imbalance of cortical excitability may explain lateralized ictal motor manifestations in patients with IGEvc.
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PMID:Interhemispheric threshold differences in idiopathic generalized epilepsies with versive or circling seizures determined with focal magnetic transcranial stimulation. 1077 Dec 52

In the kainic acid (KA) model of temporal lobe epilepsy, mossy fibers (MFs) are thought to establish recurrent excitatory synaptic contacts onto granule cells. This hypothesis was tested by intracellular labeling of granule cells with biocytin and identifying their synaptic contacts in the dentate molecular layer with electron microscopic (EM) techniques. Twenty-three granule cells from KA-treated animals and 14 granule cells from control rats were examined 2 to 4 months following KA at the light microscopic (LM) level; four cells showing MF sprouting were further characterized at the EM level. Timm staining revealed a time-dependent growth of aberrant MFs into the dentate inner molecular layer. The degree of sprouting was generally (but not invariably) correlated with the severity and frequency of seizures. LM examination of individual biocytin-labeled MF axon collaterals revealed enhanced collateralization and significantly increased numbers of synaptic MF boutons in the hilus compared to controls, as well as aberrant MF growth into the granule cell and molecular layers. EM examination of serially reconstructed, biocytin-labeled MF collaterals in the molecular layer revealed MF boutons that form asymmetrical synapses with dendritic shafts and spines of granule cells, including likely autaptic contacts on parent dendrites of the biocytin-labeled granule cell. These results constitute ultrastructural evidence for newly formed excitatory recurrent circuits, which might provide a structural basis for enhanced excitation and epileptogenesis in the hippocampus of KA-treated rats.
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PMID:Kainic acid-induced mossy fiber sprouting and synapse formation in the dentate gyrus of rats. 1090 94

We considered the evolution of Ca2+ oscillation dynamics in recurrent seizure-like events. Dynamic system behaviour was characterized in the state space reconstructed from intra- and extracellular [Ca2+] fluctuations simultaneously measured in cultured rat hippocampal slices under low-[Mg2+] conditions. When associated in the seizure-like event, these fluctuations occurred on a restricted set, the attractor, embedded in the full state space with less than five degrees of freedom. Instantaneous relative phase differences indicated field potential-driven phase jumps locked onto seizure-like events. To account for recurrent dynamics, calculations were performed on different extensions of a model for Ca2+ oscillation. These identified bidirectional, asymmetrical coupling of extracellular with intracellular (cytosolic, Ca2+ store, mitochondrial) Ca2+ dynamics as critical in its development.
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PMID:Coupled intra- and extracellular Ca2+ dynamics in recurrent seizure-like events. 1106 84

Mosaic trisomy 7 is a rare condition that can be seen in individuals with Blaschkolinear skin pigmentary variation, somatic asymmetry, and variable other clinical anomalies. In any patient presenting with Blaschkolinear skin pigmentary variation, varying degrees of asymmetrical growth disturbance, developmental delay, and a normal lymphocytic karyotype, chromosomal mosaicism may be present. To rule out tissue-specific or occult chromosomal mosaicism, it is recommended to culture and karyotype skin fibroblasts, since lymphocyte cell lines may not demonstrate the abnormal cell line. Early diagnosis is of paramount importance, since early physical, occupational, and speech/language therapy can greatly improve the developmental outcome of these patients. We report on a fourth patient with trisomy 7 mosaicism in whom early diagnosis and developmental therapy contributed to an improved developmental outcome when compared with patients in previous reports. Early intervention can greatly benefit patients with this diagnosis, especially in minimizing the aggressive behavior associated with communication difficulties. Our patient has milder manifestations than the previously reported patients with no seizure activity or asymmetry and fewer cells with trisomy 7.
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PMID:Blaschkolinear skin pigmentary variation due to trisomy 7 mosaicism. 1110 36

The combination of axial spasms in clusters, hypsarrhythmia, and psychomotor delay beginning in the first year of life defines West syndrome. Variants of this classical triad comprise variations of age of onset ranging from the first month to 4 years, spasms that may be asymmetrical or combined with focal seizures, asymmetrical, synchronous or fragmented hypsarrhythmia, and psychomotor function which may be delayed, deteriorated or normal. These variations mainly seem to depend on etiology, and specific patterns have been identified for the various causes. Most causes relate to non-progressive uni- or multifocal cortical lesions, although some are due to inborn errors of metabolism. Ten to 20% exhibit no evidence of brain lesion and are considered idiopathic. This condition is intermediary between epilepsy in which the disorder is limited to paroxysmal events during which time the patient returns to his prior condition, and status epilepticus in which the paroxysmal activity is not interrupted. Here, there are both paroxysmal events and a continuous non-convulsive paroxysmal activity that contributes to the deterioration. In the present understanding of pathophysiology, spasms seem to involve subcortical structures, whereas hypsarrhythmia affects cortical areas, also causing psychomotor deterioration. Deafferentation of subcortical structures by the continuous spiking and slow wave activity could account for release of autonomic activity in the basal ganglia. Cortical paroxysmal activity could be caused by age-related hyperexcitability linked to the development of cortical neuronal networks throughout infancy. The mode of action of steroid and vigabatrin therapies, the two therapies with demonstrated efficacy, can be explained on this basis.
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PMID:What is West syndrome? 1170 Dec 38

To describe the poorly known characteristics of epilepsy during infancy in focal cortical dysplasia (FCD), one of the most frequent cause of infantile epilepsy. All 28 patients with FCD referred to two specialized centres were retrospectively studied regarding seizure characteristics, psychomotor evaluation, and response to medical and surgical treatment. All patients presented with early partial seizures. Semiology, but not the age of onset, depended on the topography of the dysplasia, with abnormal eye movements in all cases of posterior FCD. Eleven patients also developed infantile spasms (IS), mainly asymmetrical. IS were easily controlled with Vigabatrin or ACTH, but no partial seizures could be medically controlled except in one patient. All patients except one had abnormal neuropsychological findings. Fifteen patients had surgery, eight became seizure free, and seven were significantly improved regarding psychomotor development. Very early and refractory partial seizures, but easily controlled IS are the main characteristics of FCD in infancy. Only the focal ictal semiology may help differentiate the localization of FCD. Its intrinsic epileptogenicity could sustain this clinical pattern. Since the chances for medical control and normal neurodevelopment are poor, surgical treatment should be considered early in infants with FCD.
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PMID:Characteristics of epilepsy in focal cortical dysplasia in infancy. 1235 Mar 89

We describe two young children who presented with frequent falls and myoclonic jerks affecting the trunk and legs associated with a sharp and slow wave epileptic focus at the vertex. The initial neurological examination and brain magnetic resonance imaging were normal. Both patients had a persistent gait dysfunction, sometimes asymmetrical, fluctuating with the intensity of the epilepsy and the electroencephalogram abnormalities. The localization of the epileptic focus at the vertex, corresponding to the motor control of the legs and trunk, can explain this peculiar semiology. The seizures were difficult to treat, but one patient is currently in remission. Although epileptic falls are most often a feature of severe epilepsies of childhood, we think that these two patients present a variant of benign partial epilepsy of childhood.
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PMID:Epileptic falls and gait disturbance in two young children with a sharp wave focus at the vertex: a variant of benign partial epilepsy of childhood? 1236 5


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