Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sudden loss of consciousness in childhood presents itself usually as a syncope and occurs in approximately 15% of all children. Although syncope in the pediatric age group appears to be an isolated phenomenon with good prognosis, it could also be a manifestation of a life-threatening disease. Thus, even the first syncope should be evaluated in children. A thorough history and a careful physical examination with special attention to cardiovascular and neurologic abnormalities usually point to the etiology of the syncope and must result in the appropriate laboratory tests. About 50% of pediatric syncopes are non-cardiovascular, 20 to 30% are cardiovascular, and 20 to 30% are of unknown etiology. The most frequent syncope in childhood is neurocardiogenic. It is important to distinguish neurocardiogenic syncope from cardiac syncope and epileptic seizures. Neurocardiogenic and cardiac syncopes can be discriminated from epileptic seizures by their usually shorter duration of unconsciousness and rare postsyncopal disorientation. Cardiac syncope is rare in children with a structurally normal heart and may be associated with Wolff-Parkinson-White syndrome, long QT syndrome or congenital AV block. Children with congenital heart defects and cardiomyopathies who present with syncope must raise a high degree of suspicion for a cardiac syncope. Cardiac syncopes often yield a poor prognosis with substantial percentages of sudden death; therefore, a vigorous attempt has to be made to diagnose and adequately treat cardiac syncope in children.
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PMID:[Sudden loss of consciousness in childhood]. 933 81

Myocarditis with complete atrioventricular block is a very unusual complication of the herpex simplex infection. We report a 10-year-old boy infected very likely by the herpes simplex virus and who presented with high fever, erythema multiforme, complete atrioventricular block, and Adams-Stokes seizures. Emergent temporary pacemaker was performed for bradycardia. A sixteen-fold rise in herpes simplex antibody titer by a complement fixation method occurred within two weeks. Normal cardiac rhythm recovered 11 days later with a sequela of complete right bundle branch block after 2 years follow-up.
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PMID:Myocarditis with complete atrioventricular block associated with herpes simplex virus infection: report of one case. 940 Nov 83

Trifascicular block, which consists of impaired conduction in the three main fascicles of the ventricular conduction system, may progress to high-grade or complete atrioventricular block. It rarely occurs in children with a structurally normal heart. We report a case of trifascicular block in a previously healthy 9-year-old girl. The patient presented with repetitive seizures. The electrocardiogram showed a complete right bundle branch block, left axis deviation, PR interval prolongation, and intermittent high-degree and complete atrioventricular block. She was successfully treated with a temporary pacemaker and her electrocardiogram returned to a normal sinus rhythm in 3 days. She has remained well over a 4-year follow-up. Although her cardiac enzyme levels were normal, the clinical course and electrocardiography findings suggested myocarditis. We emphasize the diagnosis of trifascicular block using electrocardiography; clinical outcome is good, if the patient is managed properly.
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PMID:Trifascicular block with intermittent complete atrioventricular block in a child. 940 28

An infant presented with neonatal syncope and seizures. An ECG showed a preexcitation pattern, most compatible with Wolff-Parkinson-White (WPW) syndrome. Rhythm monitoring during an event demonstrated prolonged periods of complete AV block with no ventricular escape mechanism. We postulated that ventricular asystole was initiated by mechanical or autonomic influences on the accessory pathway and sustained by electrophysiologic interactions between the accessory pathway and the junctional escape focus. This is the first case report of a newborn having coexisting congenital AV block and WPW syndrome.
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PMID:Coexisting preexcitation syndrome and intermittent heart block presenting as neonatal seizures. 1035 29

Ephedrine is widely used to elevate blood pressure, however, one should be cautious to use it concomitantly with phenytoin infusion in neurosurgical procedures. A 59-year-old female was admitted for craniotomy with removal of metastatic brain tumor. During operation phenytoin infusion was given to forestall postoperative seizure. Hypotension, bradycardia and complete atrioventricular block followed by ventricular asystole suddenly occurred when the patient was given ephedrine to elevate the blood pressure to see the hemostatic effect close to the end of operation. We discontinued the phenytoin infusion and immediately injected 1.5 mg epinephrine. She was successfully resuscitated. We conclude that when phenytoin is used intraoperatively it should be administered by an infusion pump at a rate of less than 25 mg/min and under continuous monitoring of cardiac rhythm, heart rate, and blood pressure. When pressure support is required, the use of a pure alpha-agonist may minimize the risk of adverse reactions in the presence of phenytoin infusion.
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PMID:Ephedrine-induced complete atrioventricular block with ventricular asystole during rapid concomitant phenytoin infusion: a case report. 1040 28

Progressive myoclonic epilepsies are rare, genetically transmitted diseases characterized by epileptic seizures, myoclonus, and progressive neurologic deterioration. Unverricht-Lundborg disease, Lafora's disease, neuronal ceroid lipofuscinosis, mitochondrial disorders, and sialidosis are included in this group. Lafora's disease is a progressive disorder of the central nervous system with onset in the late first or second decade of life and is inherited in an autosomal-recessive pattern. The first clinical manifestation is generalized tonic-clonic seizures, myoclonus, or both, usually seen between the ages of 11 and 18 years. The other clinical manifestations are progressive dementia and limb ataxia. Diagnosis is based on showing the typical inclusions in the brain, liver, skin, or muscle tissue specimens. The case of a 6-year-old male patient, who was admitted with the clinical findings of third-degree atrioventricular block and dementia and eventually diagnosed with Lafora's disease, is presented.
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PMID:A case of Lafora's disease associated with cardiac arrhythmia. 1059 53

From 1983 to 1997, we have studied ten children with complete atrioventricular block likely due to myocarditis in order to assess its prognosis and to define a therapeutic strategy. Their age ranged from 6 days to 16 years (median: 4.1 years). All were admitted for sudden complete block, with symptoms in seven: syncope or fainting, seizures, collapse. Three had an asymptomatic bradycardia which was detected on routine auscultation in children with fever or already hospitalized; fever was present in 5. The disease was related to infection on biological data in 4 cases (1 listeriosis and 3 seroconversions for Epstein Barr or cytomegalic or Coxsackie B viruses), on a myocardial biposy in 1 case and on scintigraphic data in 1 case. In the remaining 4, indirect arguments were considered such as infectious context, normal recent ECG, favourable outcome. Five children were given intravenous isoprenalin with ventricular tachycardia in 3. Five were treated with steroids and 3 with specific antiviral agents. Seven patients were paced temporarily. One child died, 6 recovered totally and 3 have a permanent block with a definitive pacemaker implanted in 2. In conclusion, sudden acquired complete atrioventricular blocks are often ill-tolerated in children and have to be treated with transient pacing. Recovery occurs as a rule but some of these blocks may be definitive. Infective myocarditis is likely to be the cause of the disease even if the pathogen agent cannot always be identified.
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PMID:[Therapy and prognosis of infectious complete atrioventricular block in children]. 1085 52

Heterozygous mutations in genes encoding cardiac ionic channel subunits KCNQ1, HERG, SCN5A, KCNE1, and KCNE2 are causally involved in the dominant form of long-QT syndrome (LQTS) while homozygous mutations in KCNQ1 and KCNE1 cause LQTS with or without congenital deafness. In addition, two homozygous HERG mutations have been associated with severe LQTS with functional atrioventricular conduction anomalies in young children. A 2:1 atrioventricular block (AVB) with a major QTc prolongation (526 ms) was evidenced in a 5-year-old boy referred for syncope and seizure. LQTS was diagnosed and beta-blocking therapy initiated leading to normal atrioventricular conduction. Electrophysiological study provided support that location of the AVB was infra-Hisian. DNA analysis was performed in the proband and in asymptomatic family members. A novel missense mutation, V1777M, in the early C-terminal domain of SCN5A was identified. The proband was homozygous while the parents and two siblings were heterozygous carriers. Homozygote and heterozygote expression of the mutant channels in tsA201 mammalian cells resulted in a persistent inward sodium current of 3.96+/-0.83% and 1.49+/-0.47% at -30 mV, respectively, which was dramatically reduced in the presence of tetrodotoxin. This study provides the first evidence for a homozygous missense mutation in SCN5A and suggests that LQTS with functional 2:1 AVB in young children, a severe phenotype associated with bad prognosis, may be caused by homozygous or heterozygous compound mutations not only in HERG but also in SCN5A. The full text of this article is available at http://www.circresaha.org.
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PMID:Homozygous SCN5A mutation in long-QT syndrome with functional two-to-one atrioventricular block. 1146 28

Symptomatic second- or third-degree atrioventricular (AV) block at any anatomical level is a class I indication for permanent pacemaker implantation. We describe a 44-year-old male with acute viral myocarditis who suffered from syncope followed by a seizure attack associated with AV conduction disturbance. His initial electrocardiogram in our emergency room revealed sinus tachycardia with 2:1 AV conduction and a right bundle branch block QRS morphology with a ventricular rate of 60 beats/min. Because of episodic slowing of the heart rate below 40 beats/min, he received 1 mg atropine intravenously which increased the atrial rate but further worsened the AV conduction, resulting in ventricular asystole for more than 30 seconds associated with loss of consciousness and seizure-like activity. He was immediately paced transcutaneously and transferred to the catheterization laboratory to receive temporary transvenous cardiac pacing. The cardiac catheterization study showed a normal coronary angiogram and very mild diffuse hypokinesis. Electrophysiological studies revealed advanced infra-Hisian AV block. The infra-Hisian AV block, however, resolved rapidly in 2 days. The patient did not receive a permanent pacemaker and remained asymptomatic with normal electrocardiogram over 1 year of follow-up. We suggest that symptomatic infra-Hisian AV block due to viral myocarditis can be reversible, and implantation of a permanent pacemaker may not be necessary.
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PMID:Reversible infra-Hisian atrioventricular block in acute myocarditis. 1177 Nov 89

Complete atrioventricular block (CAVB) can be either congenital or acquired in children. Acquired CAVB is occasionally seen in myocarditis patients. To determine the etiology, natural history, and outcome of children with acquired nonsurgical CAVB, we retrospectively reviewed nine children who had suffered CAVB caused by suspected infectious myocarditis. All of them had CAVB with a wide QRS escape ventricular rhythm on admission. Three of them had ventricular tachycardia in addition to CAVB. Seven of them had a preceding upper respiratory tract infection. All of them had congestive heart failure. Five of them had Stokes-Adams seizures. Three etiologies were identified in four of the children. All patients received inotropic agents and emergency temporary pacing. In all except one case, the cardiac rhythm returned to sinus rhythm within 10 days. During a follow-up period of 9 to 96 months, all were asymptomatic and drug-free. Electrocardiograms showed that four patients were completely normal, there was complete RBBB in four and left anterior fascicular block in one patient. We conclude that although CAVB associated with myocarditis can be life-threatening, the long-term prognosis is good if patients are diagnosed early and proper management is employed.
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PMID:Complete atrioventricular block following myocarditis in children. 1218 8


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