Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Viral infection-associated acute encephalopathy in children is a clinical syndrome with high mortality and neurological sequelae. Its main symptoms of acute phase are impaired consciousness and convulsive status epilepticus with hyperpyrexia. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized clinically by biphasic seizures and late MRI abnormalities such as reduced subcortical diffusion. Despite the intensive care, patients with AESD often have severe neurological impairment and it is very difficult to distinguish AESD from febrile seizures in the early phase. Although there is currently no specific biomarker for early diagnosis of acute encephalopathy syndrome, we believe tau protein and 8-hydroxy-2'-deoxyguanosine (8-OHdG) are potential biomarkers which could be useful in following the clinical course and monitoring the efficacy of therapies.
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PMID:[CSF biomarkers in children with acute encephalopathy syndrome]. 2262 18

Objective. Several tests are available in the diagnostics of sporadic Creutzfeldt-Jakob disease (sCJD); however, none of these is conclusive. We review the values of these tests, from an intensive care unit (ICU) perspective. Methods. Case report and review of the literature. Results. A 53-year-old woman initially presenting with psychiatric symptoms developed myoclonus and was admitted 1 month later to the ICU with a suspected nonconvulsive status epilepticus and respiratory insufficiency, probably due to extensive antiepileptic drug therapy. Typical MRI and EEG findings and a positive 14-3-3 protein led to the diagnosis of sCJD. All treatments were terminated, and autopsy confirmed sCJD. Conclusions. Clinical signs combined with MRI, EEG, and 14-3-3 and/or tau protein determination might be sufficient to diagnose or exclude sCJD and may therefore prevent the application of unnecessary diagnostic tests.
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PMID:Diagnosing sporadic creutzfeldt-jakob disease in a patient with a suspected status epilepticus in the intensive care unit. 2365 71

Epilepsy is worldwide one of the most common neurologic diseases (prevalence 0.5-1%). The diagnostic procedure following a first epileptic seizure includes cerebral imaging, blood examinations, electroencephalography, and an investigation of the cerebrospinal fluid (CSF) in patients seen soon after a seizure in order to exclude dangerous causes which require immediate treatment. Basic CSF investigation comprises the determination of cell counts, glucose, and lactate levels in serum and CSF and of albumin, immunoglobulins, and their quotients. Taken together recent studies show that otherwise unexplained liquor pleocytosis following an epileptic seizure is a rare and transient phenomenon, mainly observed in the first 72 hours. Blood-CSF barrier disruption, however, is observed in a considerable percentage of patients with epileptic seizures and plays a critical role in epileptogenesis and also is observed as a sequel of epileptic activity, in particular in status epilepticus. Similarly, elevated tau protein and lactate levels are commonly seen after epileptic seizures and depend on seizure duration. Whereas elevated lactate levels are transient and observed only up to 72 hours after a seizure, tau levels and albumin quotients remain increased for 9-14 days. Intrathecal immunoglobulin synthesis is increasingly observed in patients with focal cryptogenic epilepsy. Systematic prospective clinical and experimental trials are required to identify antigenic targets and to select patients for whom immunotherapy might be a therapeutic option.
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PMID:Epilepsy. 2911 Jul 74

Hyperphosphorylation of the microtubule-associated protein tau and its resultant aggregation into neurofibrillary tangles (NFT) is a pathological characteristic of neurodegenerative disorders known as tauopathies. Tau is a neuronal protein involved in the stabilization of microtubule structures of the axon and the aberrant phosphorylation of tau is associated with several neurotoxic effects. The discovery of tau pathology and aggregates in the cortex of Temporal lobe epilepsy (TLE) patients has focused interest on hyperphosphorylation of tau as a potential mechanism contributing to increased states of hyperexcitability and cognitive decline. Previous studies using animal models of status epilepticus and tissue from patients with TLE have shown increased tau phosphorylation in the brain following acute seizures and during epilepsy, with tau phosphorylation correlating with cognitive deficits in patients. Suggesting a functional role of tau during epilepsy, studies in tau-deficient and tau-overexpressing mice have demonstrated a causal role of tau during seizure generation. Previous studies, analyzing the impact of seizures on tau hyperphosphorylation, have mainly used animal models of acute seizures. These models, however, do not replicate all aspects of chronic epilepsy. In this study, we investigated the effects of acute seizures (status epilepticus) and chronic epilepsy upon the expression and phosphorylation of tau using the intra-amygdala kainic acid (KA)-induced status epilepticus mouse model. Status epilepticus resulted in an immediate increase in total tau levels in the hippocampus, in particular, the dentate gyrus, and phosphorylation of the AT8 epitope (Ser202, Thr205), with phosphorylated tau mainly localizing to the mossy fibers of the dentate gyrus. During epilepsy, abnormal phosphorylation of tau was detected again at the AT8 epitope with lower total tau levels in the CA3 and CA1 subfields of the hippocampus. Chronic epilepsy in mice also resulted in a strong localization of AT8 phospho-tau to microglia, indicating a distinct pattern of tau hyperphosphorylation during chronic epilepsy compared to status epilepticus. Our results reaffirm previous observations of tau phosphorylation post-status epilepticus, but also elaborate on tau alterations in epileptic mice which more faithfully mimic TLE. Our results confirm seizures affect tau hyperphosphorylation, however, suggest epitope-specific phosphorylation of tau and differences in cell-specific localization according to disease progression.
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PMID:Tau Phosphorylation in a Mouse Model of Temporal Lobe Epilepsy. 3178 Sep 21