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)

Children with adrenocortical insufficiency are commonly instructed to increase their baseline glucocorticoid replacement doses by three to five times during periods of stress such as surgery or febrille illness. We conducted this to determine whether these recommendations reflect the actual change in urinary free cortisol (UFC) output during stress. The 24-hour UFC excretion was determined in 78 children who were admitted to a general pediatric department or intensive care unit with temperature > 38.7 degrees C, after major surgery, or during status epilepticus; we reevaluated 43 of the patients 2 weeks after recovery. In addition, the 24-hour UFC levels were determined in 127 healthy children aged 1.8 to 17 years. The UFC level positively correlated with age (r = 0.254; p < 0.001). The amount of UFC per gram of creatinine was inversely correlated with age (r = 0.255; p < 0.001). The amount of UFC per surface area was independent of age. The mean change in the level of UFC per square meter surface area was highest among children who had cardiothoracic surgery and those with multiple trauma. The increase in UFC level during bacterial infection was significantly greater than that during viral infection. The current recommendation to increase the dose to three to five times the baseline glucocorticoid dose during times of stress may underestimate the changes in UFC found in some patients with major surgery, trauma, or certain serious bacterial infections. Production rate studies are needed to prove this point.
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PMID:Urinary free cortisol values in children under stress. 763 74

Eleven cases (5 F + 6 M; mean age 48.0 years) of acute noninflammatory renal failure (ANRF) in the course of rhabdomyolysis (RBM) were treated with hemodialysis in years 1995-1999. The causes of RBM were the following: ischemia of lower limbs after vascular operations (4 cases), exhausting exercise with rapid body cooling (3 cases), multiorgan failure after traffic accident, acute myositis (1 case), status epilepticus (1 case), rapid clinical course of viral infection (1 case). It was necessary to perform from 1 to 13 hemodialyses in every patient. In nine cases, complete normalization of renal function during 5 to 30 days of therapy was achieved. Two patients died due to multiorgan complications after vascular operations despite effective dialysis therapy. The following correlation were found: positive between initial values of creatine phosphokinase (CPK) activity and creatinine and uric acid concentrations in the blood and negative correlation between CPK and serum calcium concentrations. The higher initial values of CPK activity were observed the more hemodialysis procedures were necessary and the longer time was needed to normalize renal function. On the base of initial, limited up to now, own results it seems that hemodialysis in ANRF in the course of RBM should be started immediately in cases with high activity of CPK in the blood (above 10,000 U/L).
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PMID:[Acute kidney failure in the course of rhabdomyolysis with hemodialysis in personal material from 1995-1999]. 1125 48

A previously healthy, 29-year-old man developed generalized convulsion with disturbed consciousness 3 days after the flu-like symptoms. On the next day, his convulsion evolved into generalized status epilepticus (GSE) that was unresponsive to administration of various anticonvulsants. Then, intravenous anesthetic agents, including midazolam, propofol and vecuronium sodium, were continuously infused. During the following 9 days, despite of high-dose infusion of these anesthetic agents, his GSE could not be suppressed, and complex partial seizure with secondary generalization frequently occurred. The patient was comatose and mechanically ventilated, whereas the brainstem reflexes were preserved. Electroencephalogram revealed a diffuse slowing of the background activity and occasional focal spike and wave complexes with secondary generalization. Cerebrospinal fluid analysis showed pleocytosis and elevated protein concentration. Cranial CT scans obtained on day 8 demonstrated brain edema. On day 10, his GSE ceased with abolition of the brainstem reflexes, and he lapsed into brain death resulting from the progressive brain edema. Over the following six days, his systemic circulation gradually worsened, and he died on day 16. On postmortem examination, the brain was markedly edematous and showed the findings consistent with acute anoxic encephalopathy with extensive circulatory stasis. Mild gliosis was observed in the insular cortex, yet no evidence of inflammatory disease was found throughout the brain. This patient was characterized by acutely progressive severe brain edema without inflammatory changes, suggesting that his primary disease was acute toxic encephalopathy presumably induced by viral infection. His GSE was refractory and unresponsive to the intensive treatment with the intravenous anesthetic agents. This case epitomizes the difficulties in controlling refractory seizures in some patients with encephalopathy or encephalitis.
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PMID:[Acute edematous encephalopathy presenting with refractory generalized status epilepticus: an autopsy case]. 1176 16

West Nile virus (WNV) has emerged as an important cause of several outbreaks of febrile illness and encephalitis in North America over the past few years. The most common manifestation in symptomatic patients is a transient febrile illness. Neuroinvasive disease, that can be fatal, occurs most often in elderly and immunocompromised hosts. The role of this virus as a cause of meninoencephalitis in organ transplant recipients is becoming better recognized. We describe herein the clinical course of two renal allograft recipients who developed WNV encephalitis. One patient developed status epilepticus and eventually died, while the other had a full recovery. In both cases, the diagnosis was confirmed by detection of WNV-specific IgM in CSF or serum, with a delayed antibody response in one patient. This viral infection should be considered in all renal transplant recipients who present with a febrile illness associated with neurological symptoms.
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PMID:West Nile virus encephalitis: an emerging disease in renal transplant recipients. 1551 57

Neurological disorders of different etiology may cause identical clinical symptoms requiring additional diagnostic procedures for a precise differential diagnosis. Focal epileptic seizures have been shown to cause increased signal intensities in T2 and diffusion-weighted magnetic resonance images (MRI), mimicking other neurological disorders or diseases such as viral encephalitis. In some cases even the combination of neuroimaging and cerebrospinal fluid (CSF) analysis is not sufficient to obtain the final diagnosis, since epileptic seizures may cause pleocytosis as well. Some epilepsy centers presented cases of focal status epilepticus with severe but reversible MRI changes. These cases indicate that MRI-changes following focal seizures are reversible over a different time window compared to MRI changes associated with other etiologies, such as viral infection. This data further suggest that in cases where focal seizures can not be ruled out, a follow-up MRI scan within a few days following the onset of symptoms significantly improves the precision of the differential diagnosis. Recently new scientific data were reported in this review.
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PMID:MRI changes associated with partial status epilepticus. 1603 13

Acute infantile encephalopathy predominantly affecting the frontal lobes (AIEF) is proposed as a novel form of acute encephalopathy in infancy. To establish the diagnostic criteria for AIEF, we reviewed the clinical data of 10 patients who were seen by us and diagnosed as having AIEF, and those of 7 patients in the literature compatible with the diagnosis of AIEF. The mean age of onset was 1 year and 7 months. Boys and girls were equally affected. There is always an association with hyperpyrexia due to viral illness. Manifestations at the onset were convulsive status epilepticus and prolonged coma followed by signs of frontal lobe dysfunction such as a lack of spontaneity and regression of verbal functions. Imaging studies demonstrated edematous changes of the bilateral frontal lobes, which showed increased cerebral perfusion initially but attenuated perfusion several weeks later. The recovery of intellectual deficit was generally slower than that of motor disability. Based on these findings, we propose tentative diagnostic criteria of AIEF.
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PMID:Acute infantile encephalopathy predominantly affecting the frontal lobes (AIEF): a novel clinical category and its tentative diagnostic criteria. 1682 46

Rubella is an important childhood disease that was historically widespread but is now very infrequent. It is an acute viral infection ordinarily characterized by mild constitutional symptoms. Complications are relatively uncommon in childhood. Encephalitis similar to that seen with measles occurs in about 1 in 6,000 cases. The severity is highly variable, and there is an overall mortality rate of 20%. Symptoms in survivors usually resolve within 1-3 week without neurologic sequelae. An 8.5-year-old boy presented with rubella encephalitis and status epilepticus. Five days before admission the patient had erythematous maculopapular rash on the face, spreading to the trunk and extremities. On the admission day, he had a generalized tonic-clonic seizure with loss of consciousness. Microscopic and cytologic examinations of cerebrospinal fluid showed nonspecific. Electro-encephalography (EEG) showed diffuse slowing. An enzyme linked immunosorbent assay (ELISA) revealed that rubella IgM antibody titer was positive in serum and in cerebrospinal fluid. One day later, the patient became conscious with normal physical condition. As a conclusion, it is possible to prevent the complications of rubella infection, especially the congenital rubella syndrome and encephalitis with a rapid and efficient vaccination program.
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PMID:A patient with rubella encephalitis and status epilepticus. 1827 73

Human herpes virus 6 (HHV6) infection is nearly ubiquitous in childhood and may include central nervous system invasion. There are two variants, HHV6A and HHV6B. Usually asymptomatic, it is associated with the common, self-limited childhood illness roseola infantum and rarely with more severe syndromes. In patients with immune compromise, subsequent reactivation of viral activity may lead to severe limbic encephalitis. HHV6 has been identified as a possible etiologic agent in multiple sclerosis, myocarditis, and encephalitis. A preponderance of evidence supports an association between HHV6 and febrile seizures. An ongoing multicenter study is investigating possible links between HHV6 infection, febrile status epilepticus, and development of mesial temporal sclerosis (MTS). Investigation of temporal lobectomy specimens showed evidence of active HHV6B but not HHV6A replication in hippocampal astrocytes in about two-thirds of patients with MTS but not other causes of epilepsy. It has been suggested that HHV6B may cause "excitotoxicity" by interfering with astrocyte excitatory amino acid transport. Although conventional inflammatory changes are not found in most MTS specimens, inflammatory modulators may play a role in neuronal injury leading to MTS as well. If the link between early viral infection, complex or prolonged febrile seizures, and later development of intractable temporal lobe epilepsy is confirmed, new therapeutic approaches to a common intractable epilepsy syndrome may be possible.
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PMID:Human herpes virus 6B: a possible role in epilepsy? 1862 18

In acute encephalopathy with febrile convulsive status epilepticus (AEFCSE), subcortical white matter lesions on diffusion-weighted images are sometimes encountered on magnetic resonance imaging (MRI), such as in acute encephalopathy with biphasic seizures and late reduced diffusion (AESD). We report here a severe case of AEFCSE following respiratory syncytial virus infection, with emphasis on the cranial MRI findings. MRI in this patient showed widespread T2-hyperintensity along the cerebral cortical gray matter from day 3 to day 22. Lesions with reduced diffusion were noted on day 3 in the deep zone of gray matter of the left occipito-temporo-parietal cortex, but on day 7 they shifted to the subcortical white matter of both the cerebral hemispheres. These MRI findings provide radiologic evidence for damage to the cortical gray matter in AEFCSE. The serial change of diffusion-weighted images suggests that the cortical gray matter may be injured prior to the involvement of the subcortical white matter.
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PMID:Cortical gray matter lesions in acute encephalopathy with febrile convulsive status epilepticus. 1884 52

Encephalitis with anti-NMDA receptor antibodies is a recently-recognised form of paraneoplastic encephalitis characterized by a prodromal phase of unspecific illness with fever resembling viral disease, followed by memory loss, psychiatric features, seizures, disturbed consciousness, prominent abnormal movements and autonomic imbalance. Association with ovarian teratoma is common. Neurological outcome can be good, especially when surgery is performed at an early stage. Here, we report a case of anti-NMDA receptor encephalitis associated with ovarian teratoma presenting with inaugural complex partial status epilepticus. The nature of abnormal movements at early stages was unclear and abnormal movements were misinterpreted as the recurrence of partial epileptic seizures. Despite its rarity, all clinicians treating epilepsy and movement disorders should be familiar with anti-NMDA receptor encephalitis, that appears to be a very severe but curable disease.
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PMID:Complex partial status epilepticus revealing anti-NMDA receptor encephalitis. 1973 68


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