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)

The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
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PMID:A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis? 1546 68

Electroencephalography (EEG) remains central to the investigation of epilepsy. This review discusses two clinical problems at the temporal extremes of neurophysiologic recording: evaluation of the clinical significance of individual spike discharges in benign epilepsy of childhood with centrotemporal spikes (BECTS), and prolonged (several days) continuous EEG monitoring in the ICU. BECTS is misdiagnosed often, and probably mis-treated often as well. Though the long-term outcome is usually excellent, it remains unclear whether the individual epileptiform discharges have a clinical effect. Answering this question is difficult, in part because of the natural evolution of the epilepsy and its different appearance depending on wakefulness or sleep state, and also due to substantial methodologic problems in measuring short and long-term cognitive effects. Continuous EEG (CEEG) recording has grown remarkably over the last 10 years. It has proved crucial in the diagnosis of nonconvulsive status epilepticus (NCSE), especially in the ICU, given the usual lack of obvious clinical signs of seizures in most of these patients, many of whom are critically ill. Much progress has been made in agreeing on terminology for the EEG findings, but diagnosis is still complicated. More efficient and reliable technology is being developed to help process the massive amount of data captured by CEEG and make it more useful (and in a timely fashion) clinically. Still, it is not completely clear which patients should be monitored, for how long, and what is the best role for CEEG in assessing and adjusting treatment once the diagnosis has been made. Investigators are using CEEG to study "seizure burden," to help determine what are the long-term effects of nonconvulsive seizures and NCSE, and to help guide treatment and improve outcome.
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PMID:Electroencephalography in clinical epilepsy research. 2182 21

Epilepsy is a neurological disorder with significant prevalence and the individuals affected by this disease have a great probability of occurrence of a lethal phenomenon known as Sudden Unexpected Death in Epilepsy (SUDEP). SUDEP occurs mainly during the night and probably during sleep. The pathophysiological mechanisms involved in this lethal phenomenon are still obscure and new evidences that could corroborate in this area are warranted. Thus, the aim of the present study was to evaluate the effect of sleep deprivation in the expression of microRNA (miRNA) in the frontal cortex and heart tissues of adult male rats after 50days of saline (SAL) or pilocarpine-induced status epilepticus (PILO). Initially 389 miRNA expressions were evaluated between SAL and PILO groups by microarray. Subsequently, 3 differentially expressed miRNAs of each tissue were investigated after total sleep deprivation (TSD 6h) and paradoxical sleep deprivation (PSD 24h). Still, it was analyzed that the effects of sleep rebound with equivalent duration of PSD and TSD. There was a significant increase of miR-146a expression, an important inflammatory modulator in the frontal cortex of PILO rats when compared to SAL animals. Animals treated with pilocarpine were affected by TSD (through overexpression of miRNAs related to inflammatory process) and these changes were maintained even after a sleep window of 6h. In contrast, miRNAs associated with heart diseases were down-regulated in PSD rebound, suggesting a possible restoration of homeostasis in cardiovascular system of SAL and PILO groups.
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PMID:The effects of sleep deprivation on microRNA expression in rats submitted to pilocarpine-induced status epilepticus. 2453 Aug 30

Most patients with tuberous sclerosis complex (TSC) suffer from epilepsy, and many have cognitive and behavioral problems like severe intellectual disability, autism, and hyperactivity. Only rare patients with TSC and autism have a normal intelligence quotient. We report a 13-year-old girl with definite TSC who had early-onset severe epilepsy, autistic behavior, and moderate developmental delay. By school age, however, she had normal intelligence; her intelligence quotient was at least 70 based on a Stanford-Binet test that she refused to complete. She showed good reading, writing, and language comprehension skills, and the special abilities of hyperlexia, hypermnesia, and hypercalculia. However, she did not speak. Criteria of the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, and her Childhood Autism Rating Scale score of 36 indicated mild to moderate autism. She had severe electroencephalographic abnormalities: hypsarrhythmia, multifocal or generalized epileptiform discharges, and electrical status epilepticus during sleep, with a continuous left temporal focus. Magnetic resonance imaging showed many cortical tubers in all brain lobes, and subependymal nodules. We discuss possible explanations for her lack of speech. Considered as speech apraxia, her mutism could be either a symptom of her TSC or a component of her autism. Another possibility is that long-lasting electrical status epilepticus during sleep led to her autistic behavior and language arrest. Still another possibility is that a disinhibited mammalian target of rapamycin (mTOR) pathway was at the root of all of her neuropsychiatric symptoms.
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PMID:A girl with tuberous sclerosis complex presenting with severe epilepsy and electrical status epilepticus during sleep, and with high-functioning autism and mutism. 2496 9

Status epilepticus care and treatment are already being touched by the revolution in data science. New approaches designed to leverage the tremendous potential of "big data" in the clinical sphere are enabling researchers and clinicians to extract information from sources such as administrative claims data, the electronic medical health record, and continuous physiologic monitoring data streams. Algorithmic methods of data extraction also offer potential to fuse multimodal data (including text-based documentation, imaging data, and time-series data) to improve patient assessment and stratification beyond the manual capabilities of individual physicians. Still, the potential of data science to impact the diagnosis, treatment, and minute-to-minute care of patients with status epilepticus is only starting to be appreciated. In this brief review, we discuss how data science is impacting the field and draw examples from the following three main areas: (1) analysis of insurance claims from large administrative datasets to evaluate the impact of continuous electroencephalogram (EEG) monitoring on clinical outcomes; (2) natural language processing of the electronic health record to find, classify, and stratify patients for prognostication and treatment; and (3) real-time systems for data analysis, data reduction, and multimodal data fusion to guide therapy in real time. While early, it is our hope that these examples will stimulate investigators to leverage data science, computer science, and engineering methods to improve the care and outcome of patients with status epilepticus and other neurological disorders. This article is part of the Special Issue "Proceedings of the 7th London-Innsbruck Colloquium on Status Epilepticus and Acute Seizures".
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PMID:Big data in status epilepticus. 3144 29