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)

Three types of clinical features at the onset are well known to be characteristic of acute hemiplegia syndrome (AHS). Type 1 comprises status epilepticus of hemiconvulsions with fever. Representative diseases of this type are the infectious diseases of the central nervous system, acute encephalopathy and cerebral vascular diseases. Type 2 comprises status epilepticus of hemiconvulsions without fever. Cerebral vascular diseases and epilepsy are the major ones of this type. Type 3 comprises hemiplegia or hemiparesis of sudden onset without fever or convulsions. Most patients with this type had cerebral vascular diseases, about half of which were moyamoya disease in Japan. Recent progress in neuroimaging studies has allowed considerable elucidation of the etiology of AHS. Gadolinium-enhanced MRI showed minimal lesions such as capsular infarction more clearly than plain MRI. Acetazolamide test 99mTc-HMPAO SPECT imaging is one of the useful assisted diagnostic techniques for moyamoya disease, because it reveals the reserve capacity of the collaterals. [123I]IMP SPECT is useful for the diagnosis and follow-up of acute disseminated encephalomyelitis (ADEM), as the images of the lesions coincide well with the MRI ones. 99mTc-HMPAO SPECT in a case with alternating hemiplegia revealed normoperfusion in the ictal periods. Four cases of AHS are reported here.
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PMID:Acute hemiplegia syndrome in childhood. 805 23

Status epilepticus (SE) is a condition requiring emergency care, which is often poorly managed in developing countries due to the lack of personnel, drugs, and insufficient technical and medical means. This study aims at determining the epidemiologic and etiologic characteristics and the difficulty in treating SE under the existing medical practice conditions in a developing country such as Senegal. A retrospective study was therefore carried out based on SE medical files at the University Hospital of Dakar over the period January 1988 to December 1998, and included several hospital departments, i.e., paediatrics, infectious diseases and neurology. Over an 11-year period 697 cases were recorded; of these, 48.2% of patients were under 5 years of age. The seizures were generalized in 58.2% of cases, partial in 21.2%, partial secondarily generalized, or with an association of both clinical presentations in 20.6% of cases. The etiology was as follows: mainly infectious (67%), followed by resistant and/or unbalanced epilepsy (9.9%), epilepsy of vascular origin (8%), and various other causes. The overall mortality rate was 24.8%. A long period between the onset of clinical symptoms and hospital treatment was noted, with an average time lapse of 16.6 h before treatment. The drugs utilized were diazepam and phenobarbitol, administered by injection. The overall outcome could be improved by better management, i.e., better prevention and an efficient treatment of infectious diseases, a reduction in the time before treatment, and improved means of intensive care.
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PMID:[Treatment of status epilepticus in a developing country]. 1091 24

Japanese encephalitis (JE) causes at least 10 000 deaths each year. Death is presumed to result from infection, dysfunction and destruction of neurons. There is no antiviral treatment. Seizures and raised intracranial pressure (ICP) are potentially treatable complications, but their importance in the pathophysiology of JE is unknown. Between 1994 and 1997 we prospectively studied patients with suspected CNS infections referred to an infectious disease referral hospital in Ho Chi Minh City, Vietnam. We diagnosed Japanese encephalitis virus (JEV), using antibody detection, culture of serum and CSF, and immunohistochemistry of autopsy material. We observed patients for seizures and clinical signs of brainstem herniation, measured CSF opening pressures (OP) and, on a subset of patients, performed EEGs. Of 555 patients with suspected CNS infections, 144 (26%) were infected with JEV (134 children and 10 adults). Seventeen (12%) patients died and 33 (23%) had severe sequelae. Of the 40 patients with witnessed seizures, 24 (62%) died or had severe sequelae, compared with 26 (14%) of 104 with no witnessed seizures [odds ratio (OR) 4.50, 95% confidence interval (CI) 1.94-10.52, P < 0.0001]. Patients in status epilepticus (n = 25), including 15 with subtle motor seizures, were more likely to die than those with other seizures (P = 0.003). Patients with seizures were more likely to have an elevated CSF OP (P = 0.033) and to develop brainstem signs compatible with herniation syndromes (P < 0.0001). Of 11 patients with CSF OP > or =25 cm, five (46%) died, compared with seven (9%) of 80 patients with lower pressures [OR 8.69, 95% CI 1.73-45.39, P = 0.005). Of the 50 patients with a poor outcome, 35 (70%) had signs compatible with herniation syndromes (including 19 with signs of rostro-caudal progression), compared with nine (10%) of those with better outcomes (P < 0.0001). Of 11 patients with CSF OP > or =25 cm, five (46%) died, compared with seven (9%) of 80 patients with lower pressures (OR 8.69, 95% CI 1.73-45.39, P = 0.005). The combination of coma, multiple seizures, brainstem signs and illness for 7 or more days was an accurate predictor of outcome, correctly identifying 42 (84%) of 50 patients with a poor outcome and 82 (87%) of 94 with a better outcome. These findings suggest that in JE, seizures and raised ICP may be important causes of death. The outcome may be improved by measures aimed at controlling these secondary complications.
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PMID:Seizures and raised intracranial pressure in Vietnamese patients with Japanese encephalitis. 1196 Aug 97

Acute encephalitis is a common CNS infectious disease in children. However, there are limited studies concerning about the correlation between the clinical evaluations and neurological outcome. To investigate the value of neurological evaluations, and the correlation between these evaluations and neurological outcomes of acute encephalitis, in the present study we retrospectively evaluated the neurological outcome of 0- to 16-year-old children with encephalitis or meningoencephalitis between 1999 and 2000. Of 101 children enrolled, 4 died and 25 had other neurological sequelae, including epilepsy, headache, developmental delay, and emotional or behavioral changes during the 5 years of follow-up. The causative organisms in patients with neurological sequelae were herpes virus (HSV) 2/2 (100%), influenza 2/3 (67%), mycoplasma 5/12 (42%), and enterovirus 71 2/7 (29%). The important predictors for adverse outcomes were focal neurological signs, multiple seizures or status epilepticus on admission, leukopenia, focal slow waves or continuous generalized delta waves in electroencephalography (EEG), and focal cortical parenchymal hyperintensity in the magnetic resonance imaging (MRI) (p<0.05). Patients with initial presentations of focal neurological signs, papilledema, myoclonic jerks, and status epilepticus tended to have higher incidence of abnormal findings in brain MRI, although not achieving statistic significances. In addition, children with focal spikes or continuous generalized delta waves in EEG also had higher incidence of MRI abnormalities. We conclude that brain MRI studies may be indicated in patients with focal neurological signs, intractable seizure, and focal spikes, focal delta waves, or continuous generalized delta waves in EEG. For those with MRI examinations, focal cortical hyperintensity suggests poorer neurological outcomes.
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PMID:The correlation between neurological evaluations and neurological outcome in acute encephalitis: a hospital-based study. 1724 Jan 77

We present a case of an inherited disorder of copper metabolism, Menkes disease in which MRI studies revealed the coexistence of T2 hypersignal in the temporal white matter with an increase of apparent diffusion coefficient indicative of vasogenic oedema combined with T2 hypersignal of the putamen and head of the caudate and decreased apparent diffusion coefficient indicative of cytotoxic oedema. These unusual MRI features emphasize the interest of newly developed techniques in early diagnosis in Menkes disease. The acute cerebral damage might result from the combined effects of acute metabolic stress due to infectious disease and prolonged status epilepticus, acting on a highly susceptible developing brain. Vasogenic oedema in the temporal white matter could be related to prolonged status epilepticus and vascular abnormalities. Cytotoxic oedema of the putamen and head caudate could result from energetic failure.
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PMID:Unusual magnetic resonance imaging features in Menkes disease. 1824 19

We evaluated the usefulness of intravenous lidocaine therapy for managing of status epilepticus (SE) during childhood in a retrospective multi-institutional study. Questionnaires were sent to 28 hospitals concerning patients admitted for SE who were managed with lidocaine, assessing patient characteristics, treatment protocols and efficacy. In 279 treated patients, 261 SE occurrences at ages between 1 month and 15 years were analyzed. SE was classified as showing continuous, clustered, or frequently repeated seizures. Considering efficacy and side effects in combination, the usefulness of lidocaine was classified into six categories: extremely useful, useful, slightly useful, not useful, associated with deterioration, or unevaluated. In 148 SE cases (56.7%), lidocaine was rated as useful or extremely useful. Multivariate analysis indicated lidocaine was to be useful in SE with clustered and frequently repeated seizures, and SE attributable to certain acute illnesses, such as convulsions with mild gastroenteritis. Efficacy was poor when SE caused by central nervous system (CNS) infectious disease. Standard doses (approximately 2mg/kg as a bolus, 2mg/kg/h as maintenance) produced better outcomes than lower or higher doses. Poor responders to the initial bolus injection of lidocaine were less likely to respond to subsequent continuous infusion than good initial responders. We recommend lidocaine for use in SE with clustered or frequently repeated seizures, and in SE associated with benign infantile convulsion and convulsions with mild gastroenteritis. Lidocaine should be initiated with a bolus of 2mg/kg. If SE is arrested by the bolus, continuous maintenance infusion should follow; treatment should proceed to different measures when SE shows a poor response to the initial bolus of lidocaine.
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PMID:Effectiveness of lidocaine infusion for status epilepticus in childhood: a retrospective multi-institutional study in Japan. 1828 Jun 80

The objective of this study is to evaluate the clinical and radiological characteristics and the outcome of status epilepticus (SE). 117 consecutive patients with SE were evaluated including their demographics, history of epilepsy, antiepileptic drug (AED) default, comorbidities, SE type and duration. The study included 22 children, 77 adults and 18 elderly patients with SE. Blood counts, serum chemistry, ECG, cranial MRI, cerebrospinal fluid and EEG were done. Patients were treated with IV phenytoin, valproate, lorazepam or diazepam as per a fixed protocol and responses to first and second drugs were noted. Death during hospital was recorded. The etiology of SE was infection in 53.8%, drug default in 7.9%, metabolic in 14.5%, stroke in 12.8% and miscellaneous in 11% of patients. 92.3% of patients had convulsive and 7.7% nonconvulsive SE. Cranial MRI was abnormal in 62%. Infection as an etiology was more common in children, drug default and metabolic causes in adults and stroke in adults and elderly. Following first AED, SE was controlled in 50%. 30% of patients remained refractory to second AED which was related to duration of SE and mortality. 29% patients died and death was higher in elderly (44%) compared to children (14%). Acute symptomatic SE is more common in developing countries. Refractory SE is associated with SE duration and mortality.
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PMID:A clinical, radiological and outcome study of status epilepticus from India. 1973 Sep 28

This paper reports the first systematic review of uncommon causes of status epilepticus reported in the literature between 1990 and 2008. Uncommon causes are defined as those not listed in the main epidemiological studies of status epilepticus. 181 causes were identified. These were easily categorised into 5 specific aetiological categories: immunological disorders, mitochondrial disorders, infectious diseases, genetic disorders and drugs/toxins. A sixth category of 'other causes' has also been included. Knowledge of these causes is important for clinical management and treatment, and also for a better understanding of the pathophysiology of status epilepticus.
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PMID:The uncommon causes of status epilepticus: a systematic review. 2070

To clarify the incidence of first-ever episodes of status epilepticus (SE), their etiologies and outcomes among Japanese children, we performed an epidemiological study in Okayama City. One hundred and twenty patients (69 males, 51 females) experienced first-ever SE episodes between 2003 and 2005. Overall, the annual incidence of SE was 42.0 per 100,000 population (95% CI: 34.5-49.5). The highest incidence was seen in patients aged <2years, especially in the second year of life. Febrile SE accounted for 59 (49.2%) cases, and acute-symptomatic etiologies accounted for 21. Ten were considered to have remote-symptomatic etiologies, and eight to have acute-on-remote-symptomatic etiologies. Ten were classified as cryptogenic/idiopathic epilepsy-related, and 12 were unclassified. Nineteen (15.8%) patients were diagnosed with exanthema subitum, including three with encephalitis/encephalopathy, and 17 (14.2%) patients with influenza, including four with encephalitis/encephalopathy. After SE, eight (6.7%) patients suffered from motor disturbance with or without mental disturbance. One of these died during the follow-up period. Ultimately 34 (28.3%) of the 120 patients had been diagnosed with epilepsy by the end of the follow-up. We conclude that the incidence of SE among Japanese children is higher than the reported incidence among Caucasian children. Febrile SE accounted for approximately half of the cases. Among the etiologies, exanthema subitum was the most important infectious disease, followed by influenza. Both types of infection caused encephalitis/encephalopathy associated with SE as well as febrile SE.
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PMID:An epidemiological study of children with status epilepticus in Okayama, Japan: incidence, etiologies, and outcomes. 2174 Dec 14

Scrub typhus is a rickettsial disease, caused by Orientia tsutsugamushi, which is transmitted via the bite of a chigger. This disease is one of the most important infectious diseases in the Asia-Pacific area; however, a severe infant case has not yet been reported. Here, we present the case of an 8-month-old boy with scrub typhus accompanied by hemophagocytic lymphohistiocytosis (HLH). His rapid course was complicated by acute respiratory distress syndrome (ARDS), status epilepticus and disseminated intravascular coagulation (DIC). He recovered after clarithromycin therapy and intensive supportive care. Although being extremely rare, scrub typhus can be life-threatening in an infant; therefore, physicians in endemic countries should be aware of the necessity for early recognition and prompt treatment of suspected cases.
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PMID:Life-threatening scrub typhus with hemophagocytosis and acute respiratory distress syndrome in an infant. 2273 91


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