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)

The transistorized implanted pacemaker is proving to be an effective and reliable method for long-term pacing of the heart. All patients suffering from Stokes-Adams seizures were first given a trial period of conservative therapy, including isoproterenol (Isuprel), ephedrine, atropine and steroids. Twenty-four pacemaker implants were performed on 23 patients over a 21-month period. The preoperative insertion of a pacemaker cardiac catheter was a very valuable safety precaution. In this way the heart could be safely and reliably paced during the period of preoperative assessment and during the critical periods of anesthetic induction and thoracotomy. Infection did not occur, probably because of careful gas sterilization of the units. Various models of pacemakers are compared, and the reasons for two pacemaker failures are presented. There were two early deaths and one late death in the series. The relationship of progressive coronary disease to recent infarction is stressed. Patients having intermittent heart block frequently showed the picture of "competing pacemakers" postoperatively, but without deleterious effect. Twenty patients, between 54 and 88 years of age, are alive and well at the time of reporting, with excellent pacemaker response and no further Stokes-Adams attacks.
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PMID:THE IMPLANTABLE CARDIAC PACEMAKER. 1411 81

Infantile spasms is a catastrophic form of epilepsy found only in infants and young toddlers, with the peak incidence between 4 - 7 months of age. Estimated prevalence is 1 in 2000 - 6000 live births. There are many causes of infantile spasms, including tuberous sclerosis, hypoxic-ischaemic injury, congenital infectious diseases, inborn errors of metabolism, malformations of cortical development, genetic syndromes such as Aicardi's syndrome and chromosomal abnormalities. A small percentage of patients have idiopathic infantile spasms, with normal growth and development prior to the onset of infantile spasms and no known aetiology. Because of the poor prognosis of infantile spasms, treatment is usually aggressive and immediate, with the hopes of altering the natural history of the disease. The majority of patients with infantile spasms have a poor prognosis with intractable epilepsy, severe developmental delays and/or significant cognitive impairments. Of all patients with infantile spasms, 70 - 90% have mental retardation. Furthermore, 20 - 50% of patients with infantile spasms develop Lennox-Gastaut syndrome with multiple seizure types, cognitive impairments and a markedly abnormal electroencephalogram, arguably one of the most difficult epilepsy syndromes to treat. Infantile spasms are resistant to most of the standard antiepileptic drugs. Adrenocorticotropin hormone (ACTH) or oral steroids result in a significant reduction of seizures, as well as an improvement in the electroencephalogram. Some studies have indicated that infants treated with ACTH within the first month of onset have a more favourable prognosis. Vigabatrin has also been shown to be effective in the treatment, although it is not yet FDA-approved in the US. Valproate has also been used in the treatment of infantile spasms, with an efficacy of approximately 25 - 40%. However, in the very young infant, it does carry a high risk of fatal hepatotoxicity. Surgical resection may be the treatment of choice for those infants with focal cortical dysplasia and intractable infantile spasms. Emerging therapeutic possibilities include topiramate, felbamate, lamotrigine, zonisamide and perhaps levetiracetam. With the advancements in molecular biology, genetics and neuroimaging, there is the hope of novel therapies in the future.
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PMID:Infantile spasms. 1459 57

OBJECTIVE: To describe a case of movement disorder associated with cisapride use. METHOD: Case report.RESULTS: This is the case of a male eight months old child who began to use cisapride, 0,2 mg/kg tid to treat gastroesophageal reflux disease. One month after beginning with the drug, he started to present repetitive movements of the hands characterized by opening and closing hands with flexion and extension of the wrists. According to the mother, these movements became more evident as the dose of the medication was increased, and, thereafter, started to happen also on the feet. When the child was six months old, time of the first neurological evaluation, he presented normal neurological development, except for the Parachute reflex, which was absent. After excluding metabolic, toxic and infectious diseases, the drug was withdrew. The child evolution was benign, with gradual disappearance of the movements, and he was completely normal 30 days after.CONCLUSIONS: The authors stress the need suspending the use of cisapride in any case of neurologic symptoms as seizures, somnolence, malaise or involuntary movements in previously normal patients.
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PMID:[Cisapride related movement disorders] 1468 4

Balamuthia mandrillaris meningoencephalitis is a rare but often fatal infection; only 2 survivors have been reported to date worldwide. We report the case of an apparently immunocompetent patient (72-year-old woman) who developed several episodes of seizures without prior history of respiratory or skin infections. Magnetic resonance imaging with contrast revealed 2 ring-enhancing lesions, one in the right precentral region and the other in the left posterotemporal region. Open biopsy revealed Balamuthia encephalitis. The patient was treated with combination antibiotics (pentamidine, 300 mg intravenously once a day; sulfadiazine, 1.5 g 4 times a day; fluconazole, 400 mg once a day; and clarithromycin, 500 mg 3 times a day) and was discharged home. There have been no significant neurological sequelae at this writing (6 months after biopsy). We present this case with unusual clinical course to raise awareness of this infectious disease, which may have a more favorable outcome if diagnosed and treated in its early states.
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PMID:Balamuthia mandrillaris meningoencephalitis in an immunocompetent patient: an unusual clinical course and a favorable outcome. 1504 86

Brain abscess is a potentially life-threatening complication that is only rarely associated with pregnancy. Although predispositions such as a preexisting infection, foreign body, or immunosuppression are often present, up to 30% of individuals could have no risk factors. Presenting symptoms are often nonspecific but suggestive of a central nervous system process and include headache, seizures, mental status changes, and focal neurologic deficits. Cranial imaging by computed tomography (CT) or magnetic resonance imaging (MRI) can suggest the diagnosis of abscess. Diagnosis is confirmed by aspiration of purulent material. Treatment involves antibiotics, often with surgical drainage. Vaginal delivery at term appears to be safe. Care of the affected gravida, including time and route of delivery, should be approached by a team representing perinatology, neurosurgery, infectious disease, and anesthesiology.
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PMID:Brain abscess complicating pregnancy. 1510 10

Susac syndrome is a rare microangiopathy of cochlea, retina, and brain. We report a case of a 30-year-old man with Susac syndrome. The patient initially suffered from unilateral hearing loss associated with peripheral vestibular syndrome, and followed with recurrent arterial retinal occlusions and encephalopathy. The patient underwent clinical, laboratory, and neuroradiological examination. Laboratory tests were negative for systemic inflammatory or infectious disease. Signs of encephalopathy and vestibular syndrome regressed after 6 weeks, retinal obstructions were partially improved, and deafness remained unchanged. Two unexplained epileptic seizures had been documented 7 years before the development of typical clinical course. The etiology is still unknown and diagnosis was suggested by the clinical triad of bilateral sensorineural hearing loss on low frequency on audiology, recurrent bilateral retinal branch artery occlusions, and small multiple areas of signal hyperintensity in the white and gray matter on brain magnetic resonance T2-weighted images. The clinical course is self-limited and treatment options are not codified. Epileptic seizures, as those in our patient, may extend the clinical spectrum of Susac syndrome. This case also documents the possibility of multiphasic disease course.
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PMID:Susac syndrome: retinocochleocerebral vasculopathy. 1518 30

Vagal nerve stimulator pocket infections are uncommon but can cause considerable morbidity. We describe 3 children from our institution and 8 others previously reported with infection after vagal nerve stimulator implantation for seizure control. Infection was suppressed but recurred despite appropriate antimicrobial therapy when the device remained in situ. Device removal was required in all patients to achieve cure.
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PMID:Vagal nerve stimulator pocket infections. 1524 13

Orbital cellulitis is rare. However the high risk of severe ocular and neurological complications make early diagnosis and adequate therapy essential. The purpose of this retrospective study is to describe 33 cases observed in the pediatric infectious disease department of the Casablanca Children's Hospital in Morocco from 1994 to 2000. Orbital cellulitis was preseptal in 24 patients and retroseptal in 9. Infection occurred in relation with sinusitis in 10 cases, polydermitis in 8, wound infection in 6, ocular infection in 2, and dental abscess in 2. Ages ranged from 40 days to 15 years with a mean age of 5 years. Infants accounted for 25% of cases and always presented preseptal cellulitis. Fever and local edema were noted in all patients. Exophthalmia occurred in six patients and seizures in 2. The 9 cases of retroseptal cellulitis were complicated by empyema in 2 cases, meningitis in 1 case and thrombophlebitis of cavernous sinus with cerebromalacia in 1 case. Bacteriological testing identified micro-organisms in 10 cases, i.e., Staphylococcus aureus in 6 cases, Streptococcus B in 1, Streptococcus pyogenes in 1, Enterobacter Cloacae in land Acinitobacter jejuni in 1 case. Therapy was based on broad-spectrum antibiotics in association with surgery in the patient presenting in intracranial abscess. Ophthalmoplegia-like sequels including blindness, aphasia, and motor deficit occurred in 2 patients. Orbital cellulitis in children are usually preseptal and have a favorable prognosis. However prompt and adequate antibiotherapy is essential due to the risk of retroseptal involvement with inflammatory palpberal edema and possible cerebral extension.
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PMID:[Orbital cellulitis in children: a retrospective study of 33]. 1561 87

Human herpesvirus-6 (HHV-6) is the causative agent of the common childhood infectious disease, exanthem subitum. Soon after the virus was isolated from humans it was found to be closely related to human cytomegalovirus, and thus was classified within the beta subgroup of human herpesviruses. HHV-6 possesses neuro-tropism in-vitro, and it has been suggested that primary infection can cause complications of the central nervous system (CNS), including febrile seizures and encephalitis/encephalopathy. In addition, this virus has recently been associated with limbic encephalitis, which occurred in mainly immunocompromised adult patients. It is proposed that direct invasion of the virus into the CNS may play an important role in causing these neurological complications. Although pathogenesis of HHV-6 encephalitis/encephalopathy remains unclear, it is thought that two different mechanisms such as direct invasion (primary encephalitis) and immune mediated impairment (secondary encephalitis) might play important role in causing CNS manifestations. It is possible that anti-viral drugs (ganciclovir and foscarnet) might be effective to patients with primary encephalitis due to HHV-6. Therefore, rapid diagnosis of active HHV-6 infection is crucial for patient management.
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PMID:[Newly discovered human herpesviruses: pathogenesis and treatments for central nervous system infection due to HHV-6]. 1565 11

Infection with influenza viruses produces a spectrum of clinical responses, ranging from upper respiratory illness to central nervous system (CNS) involvement. Recently, the number of reports of influenza-associated encephalopathy in Japan has increased. During the winters of 1997-1998 and 1998-1999, when epidemics of type A influenza (H3N2) occurred, many pediatricians reported cases of influenza-associated encephalitis or encephalopathy in children. The prominent indicators of influenza-associated encephalopathy are the abrupt onset of seizures and coma within a few days of developing a high-grade fever. These patients often develop multi-organ failure and have high morbidity and mortality. The pathogenesis of influenza-associated encephalopathy remains unclear. Because not all patients with influenza develop encephalopathy, infection with the influenza vieus is necessary, but not sufficient, for the development of influenza-associated encephalopathy. Viral RNA is rarely detected in the cerebrospinal fluid (CSF), and the presence of viral antigen in the brain has not been proven. Pathological findings, including the lack of detectable viral antigen and inflammatory cells in brain tissues, suggest that direct viral invasion and inflammation are unlikely to be causes of this disease. In influenza-associated encephalopathy, serum and CSF concentrations of several proinflammatory cytokines and cytokine receptors--such as interleukin (IL)-6, IL-1beta, and soluble tumor necrosis factor (TNF) receptor-1 (sTNF-R1)--are elevated and are related to the clinical severity of the disease. Moreover, the damage of vascular endothelial cells has been shown. Using SNPs, molecular analysis of whole genome of the patients are now on going.
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PMID:[Influenza-associated encephalopathy]. 1565 45


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