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

Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disease characterized by sensorineural hearing loss and multiple cranial nerve palsies, usually involving the VIIth and IXth to XIIth cranial nerves. We describe the clinical and pathological features of a 33-year-old woman with BVVLS. The patient developed progressive exertional dyspnea, with clinical and laboratory findings of right-sided heart failure and pulmonary hypertension. She developed status epilepticus in the setting of cardiac deterioration and respiratory infection, and died of cardiogenic and septic shock. Autopsy disclosed bilateral neuronal loss and gliosis in the inferior colliculi, locus coeruleus and facial and vestibular nuclei. Cor pulmonale is a complication of hypoventilation-induced hypoxia and hypercapnia and had not yet been reported in BVVLS.
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PMID:Cor pulmonale in a patient with Brown-Vialetto-Van Laere syndrome: a case report. 2105 69

The aim of this study was to determine the clinical characteristics of children demonstrating neurological complications with pandemic influenza (H1N1). We reviewed the medical and laboratory records of all children who were hospitalized with neurological symptoms and who had proven influenza virus infection by reverse transcriptase-polymerase chain reaction on nasal and throat swabs. Eight children aged between 10 months and 7 years had neurological complications due to pandemic influenza (H1N1) and five of them were female. Four of them were previously healthy; there was chronic renal failure (CRF) in one and neurologic disease in three patients. Seven of them had seizure and altered consciousness. Seven of them were followed in pediatric intensive care units. We performed lumbar puncture in four patients and their cerebrospinal fluid examinations showed pleocytosis in one and no cell in three specimens. Neuroimaging was performed in four patients and three of them had abnormalities. We diagnosed aseptic meningitis in one, acute disseminated encephalomyelitis (ADEM) in one, acute necrotizing encephalopathy (ANE) in one, meningoencephalitis in one, and status epilepticus in four patients. All patients were treated with oseltamivir and antiepileptic drugs. One patient with CRF died; four previously healthy patients recovered fully, and three patients who had neurologic disorder returned to their previous neurological status. In conclusion, during pandemic influenza (H1N1) infection, neurological complications may be seen in addition to the respiratory infection. The type of neurological involvement may be variable such as triggering seizure, aseptic meningitis, encephalitis, ADEM, and ANE. Neurological complications frequently recover fully especially in previously healthy children, but sometimes a severe clinical course occurs.
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PMID:Neurological complications of pandemic influenza (H1N1) in children. 2111 Feb 4

Phenobarbital is an old antiepileptic drug used in severe epilepsy. Despite this, little is written about the need for dose adjustments in renal replacement therapy. Most sources recommend a moderately increased dose guided by therapeutic drug monitoring.A 14 year old boy with nonketotic hyperglycinemia, a rare inborn error of metabolism, characterized by high levels of glycine, epilepsy, spasticity, and cognitive impairment, was admitted to the emergency department with respiratory failure after a few days of fever and cough. The boy was unconscious at admittance and had acute renal and hepatic failure.Due to the acute respiratory infection, hypoxic hepatic and renal failure occurred and the patient had a status epilepticus.The patient was intubated and mechanically ventilated. Continuous renal replacement therapy was initiated. Despite increased phenobarbital doses, therapeutic levels were not reached until the dose was increased to 500 mg twice daily. Therapeutic drug monitoring was performed in plasma and dialysate. Calculations revealed that phenobarbital was almost freely dialyzed.Correct dosing of drugs in patients on renal replacement therapy may need a multidisciplinary approach and guidance by therapeutic drug monitoring.
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PMID:High phenobarbital clearance during continuous renal replacement therapy: a case report and pharmacokinetic analysis. 2510 86

Paroxysmal sympathetic hyperactivity (PSH) is a dysautonomic condition that is associated with various types of acquired brain injuries. Traumatic brain lesions have been documented as the leading cause of PSH. However, detailed clinical features of pediatric PSH caused by intrinsic brain lesions remain to be elusive. We present a 3-year-old boy, who had been diagnosed as having cerebral palsy, developmental delay and epilepsy after perinatal hypoxia-induced brain injury. He developed status epilepticus with fever on the third day of respiratory infection. Whereas the seizure was terminated by systemic infusion of midazolam, consciousness remained disturbed for the next 48h. Serial magnetic resonance imaging studies revealed that acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) evolved on 3days after the seizure. Therapeutic hypothermia was immediately introduced, however, the brain lesion extended to the whole subcortical white matters on day 8. The intermittent bilateral dilation of pupils with increased blood pressure and tachycardia were observed until day 12. Real-time monitoring of electroencephalograms ruled out the recurrent attacks of seizures. The abnormal signs of autonomic nervous system gradually ceased and never relapsed after recovery from the hypothermia. PSH or a transient condition of dysautonomia may emerge and persist during the acute phase of AESD.
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PMID:Transient dysautonomia in an acute phase of encephalopathy with biphasic seizures and late reduced diffusion. 2841 25

Dravet Syndrome (DS) is burdened by high epilepsy-related premature mortality due to status epilepticus (SE). We surveyed centres within Europe through the Dravet Italia Onlus and EpiCARE network (European Reference Network for Rare and Complex Epilepsies). We collated responses on seven DS SCN1A+ patients who died following refractory SE (mean age 6.9 year, range 1.3-23.4 year); six were on valproate, clobazam, and stiripentol. All patients had previous SE. Fatal SE was always triggered by fever: either respiratory infection or one case of hexavalent vaccination. SE lasted between 80 min and 9 h and all patients received IV benzodiazepines. Four patients died during or within hours of SE; in three patients, SE was followed by coma with death occurring after 13-60 days. Our survey supports the hypothesis that unresponsive fever is a core characteristic feature of acute encephalopathy. We highlight the need for management protocols for prolonged seizures and SE in DS.
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PMID:Fatal Status Epilepticus in Dravet Syndrome. 3323 77