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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Continuous myoclonus in a localised area of the body was observed in three patients. In two cases the myocloni sometimes developed into motor Jacksonian convulsions. All three patients had neurological signs on the same side as their continuous twitching indicating a lesion of the contralateral hemisphere. The surface EEG did not show changes which could be directly correlated with continuous convulsions in any of the cases. The cause was found to be a malformation of the hemisphere in one case, a recent encephalomalacia in the second and a severe hyperosomolar diabetic electrolyte imbalance in the third. Epilepsia partialis continua Kozevnikov differs from motor Jacksonian epilepsy in the continuous non-attack character and the absence of a "march of convulsions". Pathophysiologically they are both forms of focal cortical status epilepticus.
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PMID:[Epilepsia partialis continua of Kozevnikov (author's transl)]. 40 31

The correlations between sleep and prolonged epileptic activity are discussed on the basis of the status classification of Gastaut (1983). Little information is available on the interrelation of sleep and the status of tonic-clonic seizures (grand mal status). Most important is the therapeutical management of these cases. Tonic seizures have been reported to occur in large numbers during NREM sleep in patients with Lennox-Gastaut syndrome. A status-like increase is possible. Tonic seizures occur almost exclusively during sleep. Myoclonic status epilepticus arising (a) in the course of primary generalized epilepsy and (b) in the course of encephalopathies, are usually markedly attenuated during sleep. In absence status (petit mal status) synchronized sleep generally fragments the continuous discharge which is replaced by isolated bursts of polyspikes, or polyspike and wave complexes. The absence status can recur upon awaking during the night or in the morning. The abnormal EEG activity of a petit mal status can, however, occasionally persist during the whole night. Improvement as well as activation during sleep have been observed in elementary (= simple) partial status epilepticus; improvement seems to be more frequent. Epilepsia partialis continua may persist or decrease during sleep. An increase as well as decrease of motor phenomena has been observed during the REM stages. 'Epileptic aphasia' of childhood is associated with subclinical bioelectric status epilepticus during sleep. The electrical status epilepticus must be delineated as a separate group. The term encephalopathy related to electrical status epilepticus during slow sleep (ESES) has been proposed on the basis of associated psychic syndromes. This form of status epilepticus disappears during the waking state and during REM sleep. Cases with hypsarrhythmia without clinical signs may also be classified under the group of electrical or bioelectrical status. In some cases, a continuous hypsarrhythmia is observed only during sleep. In this context, one must also mention those patients who demonstrate continuous activation of spikes, or spike and wave potentials (without clinical seizures) during eye closure.
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PMID:Sleep and prolonged epileptic activity (status epilepticus). 176 86

Status epilepticus (SE) is one of the most common neurologic emergencies in children, adolescents, and young adults. SE may be due to acute neurologic conditions such as meningitis, encephalitis, or stroke, complicated febrile seizures, intractable epilepsy, degenerative diseases, intoxication, or may be the first manifestation of epilepsy. Initial treatment of convulsive SE is usually with an intravenous benzodiazepine (BZD) [lorazepam (LZP) or diazepam (DZP)], phenobarbital (PB), or phenytoin (PHT). LZP is less likely to cause respiratory depression than DZP and is therefore preferred. Sequelae and risk for recurrence of SE are primarily related to the underlying cause. Refractory SE (RSE) is most often symptomatic of an acute neurologic condition or neurodegenerative disease. Treatment for RSE is difficult, usually requiring intensive support of vital functions. Reported treatments for RSE include very high dose PB, continuous infusions of pentobarbital or BZDs (DZP, midazolam), lidocaine, inhalation anesthesia, and propofol. Outcome is related to underlying cause. Nonconvulsive SE may present as confusion or may mimic psychiatric illness. Response to BZDs is usually rapid but may not be sustained. Rapid initiation of oral or rectal valproate may be useful. Epilepsia partialis continua (EPC) is almost always due to an acute or chronic destructive lesion. Surgical treatment may be the only effective modality in some children with EPC. Acute treatment of breakthrough seizures and clusters of seizures at home with rectal BZDs (usually DZP, 0.2-0.5 mg/kg) may prevent progression to SE in some children and adolescents and reduce the need for visits to emergency facilities.
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PMID:Status epilepticus and acute repetitive seizures in children, adolescents, and young adults: etiology, outcome, and treatment. 864 55

Focal status epilepticus (FSE) and Epilepsia partialis continua (EPC) are relatively uncommon disorders. Antiepileptic drugs do not usually alter the FSE-EPC. An 11 year old female patient with progressive neurologic deficits and FSE showed a remarkable response to clonazepam, both clinically and electrophysiologically.
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PMID:Oral clonazepam sensitive focal status epilepticus (FSE). 1077 67

Epilepsia Partialis Continua (EPC), a subtype of status epilepticus has varied etiology and the outcome depends on the cause. The aim of this study was to analyze the demographic, semiology, etiology, radiological findings, therapeutic response and outcome of EPC. This is a retrospective analysis of 76 patients (M:F: 46:30; mean age: 30.2+/-23.4 years; median age: 26 years) evaluated at our center over last 14 years. Twenty-three subjects (30.3%) had epilepsy for a mean of 25.8+/-52.3 months (range: 1-81 years; median: 14) before developing EPC and in half of them, seizures were controlled with anti-epileptic drugs (AEDs). Rest 53 (69.3%) manifested as de novo. The mean duration of EPC was 47.02+/-188.2 days (range: 1h to 48 months; median: 3 days). One patient of generalized convulsive SE (GCSE) evolved into EPC while five patients of EPC evolved into GCSE. CT scan of brain (n-76) was abnormal in 53 (69.7%) while all the 11 MRI scans which were available were abnormal. EEG (n-21) was abnormal in all but one, however it was non-specific in 7. The diagnoses were-idiopathic: 17, ischemic stroke: 15, meningo-encephalitis: 8, Rasmussen's encephalitis (RE): 7, granuloma: 6, diabetic-non-ketotic-hyperosmolar-coma (DNKHC): 6, CNS malignancies (primary/secondary): 4, birth injury: 4, cerebral venous thrombosis: 3, CNS tuberculosis: 2, and cerebritis, HIV-related, toxemia of pregnancy, and MERRF one each. Patients of >40 years (n=21) had stroke (10), idiopathic (6), DNKHC (4) and metastasis (1) as common causes. Only 12 of them received single AED, while others required 2 or more AEDs to control the seizures. The outcome (n=72) was-controlled: 43 (59.7%); uncontrolled: 26 (36.1%) (RE: 7, idiopathic: 5, birth injury: 4, encephalitis: 3, malignancy: 2, granuloma and MERRF: 1 each) and three patients succumbed (encephalitis: 2, idiopathic: 1). Causes of EPC are varied and it depends on age. Underlying cause determined the outcome and could be refractory in RE, idiopathic, and when associated with birth injury, malignancy and encephalitis. Treatment of underlying cause is essential in addition to AEDs.
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PMID:Epilepsia Partialis Continua over last 14 years: experience from a tertiary care center from south India. 1729 88

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. It may have vascular, immune-mediated, neoplastic or metabolic-toxic causes. The origin of EPC has been linked with the motor cortex. This has been solidly supported by sophisticated electrophysiological studies. Here, a series of video sequences from patients with EPC (due to Rasmussen encephalitis, early-stage multiple sclerosis, and steroid responsive encephalopathy with autoimmune thyroiditis), and other cases with repetitive myoclonic jerks or movement disorders (myoclonic epilepsy associated with ragged-red fibers, Jacksonian march, myoclonic seizures in other types of frontal lobe or idiopathic generalized epilepsies, and different types of tremor) is presented. [Published with video sequences].
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PMID:Epilepsia partialis continua: semiology and differential diagnoses. 1836 24

Epilepsia partialis continua (EPC) is a rare form of focal motor status epilepticus. There is typically a predilection for facial and distal limb involvement, but rarely trunk or abdomen muscles may be affected. Rarely, EPC may also present in association with cortical dysplasia. In this report, we describe the clinical, neuroimaging and ictal electroencephalographic findings of a young woman presenting with persistent myoclonic twitches of the abdominal muscles that were considered to represent a rare manifestation of EPC due to cortical dysplasia. To the best of our knowledge, this is the first report of malformation of cortical development causing abdominal myoclonus.
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PMID:A patient with two episodes of epilepsia partialis continua of the abdominal muscles caused by cortical dysplasia. 1901 73

The C10orf2 gene encodes the mitochondrial DNA helicase Twinkle, which is one of the proteins important for mitochondrial DNA maintenance. Dominant mutations cause multiple mitochondrial DNA deletions and progressive external ophthalmoplegia, but recent findings associate recessive mutations with mitochondrial DNA depletion and encephalopathy or hepatoencephalopathy. The latter clinical phenotypes resemble those associated with recessive POLG1 mutations. We have previously described patients with infantile onset spinocerebellar ataxia (MIM271245) caused either by homozygous (Y508C) or compound heterozygous (Y508C and A318T) Twinkle mutations. Our earlier reports focused on the spinocerebellar degeneration, but the 20-year follow-up of 23 patients has shown that refractory status epilepticus, migraine-like headaches and severe psychiatric symptoms are also pathognomonic for the disease. All adolescent patients have experienced phases of severe migraine, and seven patients had antipsychotic medication. Epilepsia partialis continua occurred in 15 patients leading to generalized epileptic statuses in 13 of them. Eight of these patients have died. Valproate treatment was initiated on two patients, but had to be discontinued because of a severe elevation of liver enzymes. The patients recovered, and we have not used valproate in infantile onset spinocerebellar ataxia since. The first status epilepticus manifested between 15 and 34 years of age in the homozygotes, and at 2 and 4 years in the compound heterozygotes. The epileptic statuses lasted from several days to weeks. Focal, stroke-like lesions were seen in magnetic resonance imaging, but in infantile onset spinocerebellar ataxia these lesions showed no predilection. They varied from resolving small cortical to large hemispheric oedematous lesions, which reached from cerebral cortex to basal ganglia and thalamus and caused permanent necrotic damage and brain atrophy. Brain atrophy with focal laminar cortical necrosis and hippocampal damage was confirmed on neuropathological examination. The objective of our study was to describe the development and progression of encephalopathy in infantile onset spinocerebellar ataxia syndrome, and compare the pathognomonic features with those in other mitochondrial encephalopathies.
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PMID:Recessive twinkle mutations cause severe epileptic encephalopathy. 1930 94

Dengue fever is becoming a great public health problem leading to significant morbidity and mortality, particularly in developing countries. In the past few years, various neurological complications have been reported globally. Dengue encephalitis as a clinical presentation of dengue fever is now gaining recognition in the scientific community. It is characterized by fever, headache, reduced consciousness, and generalized seizures. Epilepsia partialis continua, a type of focal status epilepticus, is caused by multiple clinical disorders, including viral encephalitis. We describe a young woman who manifested epilepsia partialis continua caused by dengue encephalitis.
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PMID:Epilepsia partialis continua as a manifestation of dengue encephalitis. 2121 5

Epilepsia partialis continua (EPC) is clinically defined as a syndrome of continuous focal jerking of a body part, usually a distal limb, occurring over hours, days, or even years. It is considered the status epilepticus equivalent of simple partial motor seizures. A 48-year-old right-handed man with a history of traumatic intracranial hemorrhage was admitted for right-sided hemiplegia and drowsiness after complex partial status epilepticus. An EEG showed periodic lateralized epileptiform discharges over the left hemisphere. Brain MRI revealed extensive multifocal encephalomalaciac changes in the left temporo-parieto-occpital lobe and both frontal lobes with some hemorrhagic residual change. After administration of a loading dose of intravenous phenytoin, his mental status returned to normal. However, his weakness only partially improved. [(18)F]Fluorodeoxyglucose PET (FDG-PET) demonstrated severe hypometabolism in the left cerebral hemisphere, including the basal ganglia and thalamus, with cerebellar diaschisis. At the 3-month follow-up, he complained of symptoms of alien hand phenomenon. Follow-up MRI revealed more extensive encephalomalaciac changes in previously noted regions with thinning of the posterior end of the body of the corpus callosum. Moreover, FDG-PET demonstrated persistent severe hypometabolism over the left cerebral hemisphere. We suggest that the alien hand phenomenon was a result of thinning of the corpus callosum related to EPC.
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PMID:Alien hand syndrome after epilepsia partialis continua: FDG PET and MRI studies. 2210 67


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