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)

A 12-year-old Bedouin boy presented with sudden asymmetrical status epilepticus. The presence of a scalp wound, rubbery cervical lymphadenopathy and a history of contact with cats suggested the diagnosis of cat-scratch disease complicated by encephalopathy. The diagnosis was supported by histological examination of a cervical lymph node and a positive skin test with cat-scratch antigen. The rapid establishment of the diagnosis avoided the necessity of elaborate and complicated neuroradiological investigations.
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PMID:Cat-scratch disease presenting as status epilepticus. A case report. 46 26

Two cases of discontinuous status epilepticus (SE) characterized by repetitive asymmetrical atonic episodes associated with diffuse but asymmetrical spike waves are reported. Both patients also had partial seizures and interictal rolandic discharges. Dynamic EEG topography was performed to investigate the location or propagation of each ictal discharge overlying the scalp during status and showed immediate bilateral spread of discharges originating from a primary epileptogenic focus from a rolandic area.
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PMID:Status epilepticus characterized by repetitive asymmetrical atonia: two cases accompanied by partial seizures. 211

Next to tonic-clonic seizures psychomotor (complex focal) seizures are the most common form of all epileptic seizures, except in infancy where they are seen rarely. Differently from generalised non convulsive seizures (like petit mal absences), their first appearance has no typical age limit, however, their proportion to other forms of seizures increases in adolescence and adults especially between the third and fifth decade of life. The main symptom is the disorder of consciousness which lasts at least more than half a minute, normally several minutes in completely distinct seizures, which doesn't begin abruptly and which often ends ill defined. This twilight attack is proceeded by an aura of sensory, psychic or vegetative character. The aura is followed either by a transitory state of immobility and later by motor phenomena or at once by motor phenomena in the form of diverse automatisms of variable intensity, reaching from mild movements in the oral region over verbal expressions to highly dramatic scenes, often accompanied by vegetative symptoms. Tonic versive and tonic symmetrical or tonic asymmetrical seizure symptoms are quite often motor variants which also can lead to sudden drops. Psychomotor attacks can be reduced to "pseudo-absences", however, they also can develop into tonic-clonic seizures (Grand mal). Generally, the succession of seizure symptoms is constant in the same patient, the expression can differ from seizure to seizure. Psychomotor attacks can be spread over the whole day or can show a strict connection to sleep, in the course they can likely occur in clusters and can accumulate to a continuous or discontinuous form of psychomotor status epilepticus. Predominantly, but not exclusively psychomotor attacks start from the temporal lobe, whereas neocortical temporal attacks (especially of lateral posterior origin) can be distinguished from those coming from the limbic system, especially from hippocampal or mesio-basal temporal structures and from the nucleus amygdalae. About 20% of the psychomotor attacks are of frontal origin coming from the mesial frontal region or from the gyrus cinguli anterior. Also seizures of occipital or parietal origin can spread so quickly that the seizure itself is impressing as a "temporal lobe attack". On account of that, epilepsies with psychomotor attacks cannot be compared to temporal lobe epilepsies. The etiology of psychomotor epilepsies is closely connected to the topographic site of the temporal lobe, who is especially vulnerable for traumatic lesions, cerebral edema and hypoxemia. Also small dysgeneses, heterotopies or small abnormalities of vessels are relatively often found in surgical specimens.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Psychomotor epilepsy: phenomenology, localization, pathogenesis and therapy]. 219 20

The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.
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PMID:[Sebaceous linear nevus syndrome with hemimegalencephaly. Report of a case]. 276 20

Based on the clinicoelectrographic data of 28 patients (14 children and 14 adults) with absence status epilepticus thoroughly documented by CCTV/EEG, it was found that there were significant differences between the children and adults. In childhood, absence status tended to occur in those who had experienced individual short-lived atypical absence seizures and also other types of generalized seizure. In contrast, there was a general tendency for absence status in adulthood to occur in females without individual absence seizure. With respect to the clinicoelectrographical manifestations, absence status with a decreased postural tone was prone to be associated with a more profound clouding of consciousness, whereas in those with myoclonic components there was a less profound clouding. The former was found solely in children while the latter was both in children and in adults. During absence status, the focal motor features with or without secondarily generalized convulsions were observed in 8 adult patients (57%). On the other hand, no focal motor manifestations were observed during absence status in children. Absence status is composed of two modalities: either a prolongation or repetition of absence seizures. It was demonstrated that, in children, either the prolongation or repetition of individual absence seizures developed into absence status. The short-lived absence was of an atypical nature, whereas in adults, absence status consisted of the prolongation of an absence seizure which occurred in patients with no experience of individual absence seizures. With respect to the drug treatment, antiabsence drugs had some effect in children, while none of the adult patients responded either to the antiabsence drugs or other antiepileptic drugs. The long-term seizure prognosis was not necessarily poor in children but invariably poor in adults. Four adult patients showed diffuse, but unilateral frontally accentuated asymmetrical paroxysmal activity during the status. Three of them showed initial localized spike-wave discharges in the unilateral frontal region followed by a generalized spike-wave rhythm. Furthermore, all of these 4 patients with focally accentuated ictal EEGs have shown partial motor seizures intermingled with absence status.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:A comparative study of absence status epilepticus between children and adults. 324 75

The prognostic value of early neonatal continuous electroencephalographic recordings in hypoxic ischaemic encephalopathy was evaluated. Thirty seven full term infants with hypoxic ischaemic encephalopathy were studied. The electroencephalogram (EEG) was recorded using four or eight channel Oxford Medilog recorders and was started as soon as possible after birth. The recordings were initially visually analysed and divided into four categories: three in relation to the grade of discontinuity of the background activity (continuous, discontinuous, and maximum depression) and an additional fourth category to include status epilepticus. The EEGs with discontinuous activity were then analysed by computer to obtain a more objective assessment of discontinuity. The results were related to neurological outcome. Continuous background activity was associated with a normal outcome in all but the three infants who had continuous, but asymmetrical EEGs and who developed contralateral hemiplegia. In the eight infants with discontinuous activity, the outcome appeared to be related to the grade of continuity and the presence of clear convulsions on the EEG. The 10 infants with maximum depression and status epilepticus had severe impairment. These preliminary results suggest that continuous recording of EEGs could be used routinely in term infants with hypoxic ischaemic encephalopathy. Computer analysis can improve the value of this technique, allowing the identification of infants who might benefit from early therapeutic intervention.
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PMID:Prognostic value of continuous electroencephalographic recording in full term infants with hypoxic ischaemic encephalopathy. 797 86

Status epilepticus (SE) is defined as recurrent epileptic seizures without full recovery of consciousness before the next seizure begins, or more-or-less continuous clinical and/or electrical seizure activity lasting for more than 30 min whether or not consciousness is impaired. Three presentations of SE are now recognized: recurrent generalized tonic and/or clonic seizures without full recovery of consciousness between attacks, nonconvulsive status where the patient appears to be in a prolonged "epileptic twilight state," and continuous/repetitive focal seizure activity without alteration of consciousness. Generalized convulsive status epilepticus (GCSE) encompasses a broad spectrum of clinical presentations from repeated overt generalized tonic-clonic seizures to subtle convulsive movements in a profoundly comatose patient. Thus, GCSE is a dynamic state that is characterized by paroxysmal or continuous tonic and/or clonic motor activity, which may be symmetrical or asymmetrical and overt or subtle but which is associated with a marked impairment of consciousness and with bilateral (although frequently asymmetrical) ictal discharges on the EEG. Just as there is a progression from overt to increasingly subtle clinical manifestations of GCSE, there is also a predictable sequence of progressive EEG changes during untreated GCSE. A sequence of five patterns of ictal discharges has been observed: discrete electrographic seizures, waxing and waning, continuous, continuous with flat periods, and periodic epileptiform discharges on a relatively flat background. A patient actively having seizures or comatose who exhibits any of these patterns on EEG should be considered to be in GCSE and should be treated aggressively to stop all clinical and electrical seizure activity to prevent further neurological morbidity and mortality.
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PMID:Generalized convulsive status epilepticus in the adult. 846 89

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

The "dormant basket cell" hypothesis suggests that postinjury hippocampal network hyperexcitability results from the loss of vulnerable neurons that normally excite insult-resistant inhibitory basket cells. We have reexamined the experimental basis of this hypothesis in light of reports that excitatory hilar mossy cells are not consistently vulnerable and inhibitory basket cells are not consistently seizure resistant. Prolonged afferent stimulation that reliably evoked granule cell discharges always produced extensive hilar neuron degeneration and immediate granule cell disinhibition. Conversely, kainic acid-induced status epilepticus in chronically implanted animals produced similarly extensive hilar cell loss and immediate granule cell disinhibition, but only when granule cells discharged continuously during status epilepticus. In both preparations, electron microscopy revealed degeneration of presynaptic terminals forming asymmetrical synapses in the mossy cell target zone, including some terminating on gamma-aminobutyric acid-immunoreactive elements, but no evidence of axosomatic or axoaxonic degeneration in the adjacent granule cell layer. Although parvalbumin immunocytochemistry and in situ hybridization revealed decreased staining, this apparently was due to altered parvalbumin expression rather than basket cell death, because substance P receptor-positive interneurons, some of which contained residual parvalbumin immunoreactivity, survived. These results confirm the inherent vulnerability of dendritically projecting hilar mossy cells and interneurons and the relative resistance of dentate inhibitory basket and chandelier cells that target granule cell somata. The variability of hippocampal cell loss after status epilepticus suggests that altered hippocampal structure and function cannot be assumed to cause the spontaneous seizures that develop in these animals and highlights the importance of confirming hippocampal pathology and pathophysiology in vivo in each case.
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PMID:"Dormant basket cell" hypothesis revisited: relative vulnerabilities of dentate gyrus mossy cells and inhibitory interneurons after hippocampal status epilepticus in the rat. 1262 66

Although there are many types of epilepsy of both genetic and acquired forms, temporal lobe epilepsy (TLE) with hippocampal sclerosis is probably the single most common human epilepsy, and the one most intensely studied. Despite a wealth of descriptive data obtained from patient histories, imaging techniques, electroencephalographic recording, and histological studies, the epileptogenic process remains poorly understood. Progress toward understanding the etiology of an acquired neurological disorder is largely dependent on the degree to which experimental animal models reflect the human condition. Recent observations suggest that significant disparities exist between the features of human TLE with hippocampal sclerosis and those of animal models that involve prolonged status epilepticus to initiate the epileptogenic process. TLE most commonly involves patients with focal seizures who exhibit limited and often asymmetrical brain damage, did not experience status epilepticus prior to the onset of epilepsy, and who appear relatively normal on neurological examination. Conversely, animals subjected to prolonged status epilepticus exhibit severe brain damage, behavioral abnormalities, and frequent generalized seizures. In addition, although many TLE patients exhibit an atrophic hippocampus that may, or may not, be a source of spontaneous seizures, hippocampal damage in animals subjected to status epilepticus is an inconsistent and often minor part of a much greater constellation of damage to other brain structures. Furthermore, many patients exhibit developmental structural abnormalities that presumably play a role in the clinical etiology, whereas most animal models involve severe insults in initially normal laboratory rats. Although much has been learned using the current animal models, the available data suggest the need for a critical reappraisal of the assumptions underlying their use, and the need to develop experimental preparations that may more closely model the human epileptic state.
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PMID:The neurobiology of temporal lobe epilepsy: too much information, not enough knowledge. 1577 Oct


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