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)

A 67-year-old, right-handed woman became unable to recognize familiar faces following a period of nonconvulsive status epilepticus. Neuropsychological assessment revealed a relatively selective impairment of familiar face recognition in the absence of low-level visual deficits or widespread cognitive impairment. MRI scanning demonstrated an isolated lesion, probably a venous angioma, involving the left fusiform gyrus, mirror-symmetrical to the site typically linked to prosopagnosia. Potential explanations for the patient's prosopagnosia include seizure-related damage to a left fusiform region required for fully competent face recognition and damage to the contralateral fusiform gyrus via interhemispheric connections. Focal neuropsychological deficits in patients with refractory partial epilepsy who develop nonconvulsive status epilepticus may be underdiagnosed.
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PMID:Prosopagnosia following nonconvulsive status epilepticus associated with a left fusiform gyrus malformation. 1677 88

We recently experienced a case with asymmetrical cortical abnormality on MRI with focal status epilepticus following severe hypoglycemia. The cerebral blood flow and metabolisms for oxygen and glucose were determined using positron emission tomography (PET) during focal status epilepticus following severe hypoglycemia and at the follow-up period. Prolonged seizure activity produced profound glucose hypermetabolism and mild hyperemia in the region of the presumed cortical focus of epilepsy and in structures anatomically remote from the focus, corresponding to the areas of abnormal signal intensity on the MRI. The patient remained comatose and exhibited a diffuse hypoperfusion/hypometabolism and symmetrical brain atrophy on the follow-up PET and MRI, respectively. Cytotoxic brain edema due to profound glucose metabolism without compensatory increase of the blood flow during status epilepticus may account for the brain abnormality observed on the early MRI. Simultaneous examination of the cerebral blood flow and metabolism using PET can provide useful information about the pathology in patients with status epilepticus.
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PMID:Magnetic resonance imaging and positron emission tomography findings in status epilepticus following severe hypoglycemia. 1687 11

We report the first case of an autoimmune thyroid encephalopathy presenting with multifocal motor status epilepticus. A 37-year-old female patient was admitted with multifocal motor seizures intractable to intravenous status epilepticus treatments, asymmetrical quadriparesis, truncal ataxia and continuous semi-rhythmical jerks. Pathological signal alterations were detected in both precentral cortices in MRI examination. Autoimmune thyroiditis was diagnosed after radiological examinations of the thyroid gland and thyroid function tests. Seizures promptly ceased following intravenous steroid treatment. Immunohistochemistry studies showed mild to moderate neuronal staining with the plasma and CSF samples. Remarkably, autoimmune thyroiditis may present with migrating focal motor status epilepticus. We recommend anti-thyroid antibody screening for multifocal motor status epilepticus cases of unspecified cause.
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PMID:Autoimmune thyroid encephalopathy presenting with epilepsia partialis continua. 1692 5

Lithium-pilocarpine induces status epilepticus (SE), leading to extensive damage and spontaneous recurrent seizures (SRS). Neuroprotective and antiepileptogenic effects of topiramate (TPM) associated with diazepam (DZP) were investigated in this model. SE was induced by LiCl and pilocarpine. TPM (10, 30 or 60 mg/kg) was injected at the onset of SE and 10h later and DZP (2.5 and 1.25mg/kg) at 2 and 10h after SE. TPM treatment was continued twice daily for 6 days. Other rats received two injections of DZP on the day of SE. Cell counting was performed on thionine-stained sections 14 days after SE and after 2 months of epilepsy. Occurrence and frequency of SRS were video-recorded. The MRI T2-weighted signal was quantified in hippocampus and ventral cortices. DZP-TPM treatment induced partial neuroprotection in CA1 and hilus, and tended to increase the percentage of rats with protected neurons in layer III/IV of the ventral entorhinal cortex. The latency to and frequency of SRS were not modified by DZP-TPM. T2-weighted signal was decreased in hippocampus 3 days after SE at all TPM doses and in ventral hippocampus after epilepsy onset. In conclusion, although DZP-TPM treatment was able to partially protect two areas critical for epileptogenesis, the hippocampus and ventral entorhinal cortex, it was not sufficient to prevent epileptogenesis.
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PMID:The combination of topiramate and diazepam is partially neuroprotective in the hippocampus but not antiepileptogenic in the lithium-pilocarpine model of temporal lobe epilepsy. 1694 4

Fragile X syndrome (Fra-X) is a common cause of mental retardation that can be associated with partial epilepsy characterized by a variety of electro-clinical features. A wide spectrum of interictal activities are reported, although no data regarding ictal EEG activity have as yet been published. Drug-resistant seizures are uncommon, and the occurrence of clustering seizures or status epilepticus has only been reported anecdotally. We describe a Fra-X patient with refractory partial epilepsy related to a malformation of cortical development who experienced a partial status epilepticus that was well documented by video-EEG and MRI. We report the electro-clinical features and peri-ictal neuroimaging data.
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PMID:Status epilepticus in a patient with fragile X syndrome: electro-clinical features and peri-ictal neuroimaging. 1698 40

We report a 60-year-old man with grade III astrocytoma, who presented with status epilepticus. The initial MRI did not demonstrate typical findings of an astrocytoma but rather showed reversible posterior leukoencephalopathy syndrome (RPLS). N-Isopropil-p-[I] iodoamphetamine single photon emission computed tomography (SPECT) demonstrated hyperperfusion in this area. A brain tumor should be considered and the patient carefully followed by MRI, even if the MRI white matter lesion pattern suggests RPLS. This is especially relevant in the presence of atypical findings for RPLS on SPECT.
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PMID:Anaplastic astrocytoma presenting as reversible posterior leukoencephalopathy syndrome. 1712 27

The diagnosis and treatment of a first epileptic seizure are made by physicians with different types of expertise. Heterogeneous patterns of care are thus expected, which explain the need for shared patterns of care. These guidelines were developed by a group of experts from the Italian League against Epilepsy (LICE) in accordance with the requirements of evidence-based medicine. An accurate assessment of the seizure is required, with active questioning about circumstances of occurrence, clinical manifestations, and postictal symptoms. For seizures with loss of consciousness, the presence of cyanosis, hypersalivation, tongue biting, and postictal disorientation has a specific diagnostic value. Laboratory tests and toxicological screening should be performed only in the presence of circumstances suggesting a metabolic or toxic encephalopathy. Elevated prolactin levels 10-20 min. after the event help in differentiating generalized tonic-clonic or partial seizures from psychogenic nonepileptic seizures. Except for infants less than six months of age, CSF examination is recommended only when a cerebral infection is suspected. An EEG should be performed within 24 h. after a seizure, particularly in children. If the EEG is normal during wakefulness, a sleep EEG is recommended. A CT scan is strictly indicated when a severe structural lesion is suspected or when the etiology is unknown. MRI may not be indicated in the emergency room, but it should be preferred to CT as part of the diagnostic assessment. The added value of other diagnostic tools (neuropsychological tests, ambulatory EEG, functional MRI, SPECT, and PET) is as yet unknown. These tests may be used on a case-by-case basis. In the presence of an acute symptomatic seizure, treatment of the cause is recommended. Symptomatic therapy is not justified unless the seizure has the characteristics of status epilepticus. Long-term treatment may be considered in patients with abnormal EEG and imaging data and after consideration of the social, emotional, and personal implications of seizure relapse.
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PMID:Diagnosis and treatment of the first epileptic seizure: guidelines of the Italian League against Epilepsy. 1723 98

A patient of Wilson's disease having neurological as well as psychiatric manifestations who presented with status epilepticus is being reported. The diagnosis was confirmed by biochemical investigations and 'face of giant panda' sign was present on MRI brain.
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PMID:Wilson's disease presenting as status epilepticus. 1724 60

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

Three patients with neurosyphilis are reported. The first and third patients presented with convulsive status epilepticus and the second with non-convulsive status after penicillin administration. In all cerebrospinal fluid and the serum Venereal Disease Research Laboratory Test (VDRL) and Treponema Pallidum hemagglutination (TPHA) or fluorescent treponemal antibody absorption test (FTA-ABS) were positive, but HIV serology was negative. Their EEGs showed periodic, lateralized, epileptiform discharges (PLEDs) just after SE. The first and third patients had no history of epilepsy. Seizures started as focal motor attacks but then secondarily generalized. The first patient's cranial MRI showed cerebral atrophy and hyperintensity involving bilateral medial and anterior temporal regions, more prominent on the left and which disappeared after penicillin treatment. The second case, after receiving penicillin, had nonconvulsive SE, a clinical presentation suggesting a Jarisch-Herxheimer reaction (JHR). Her cranial MR revealed moderate cortical atrophy and widespread confluent hyperintense foci mainly in both periventricular areas, corona radiata and centrum semiovale. MRI of the third case showed a large, left sylvian, arachnoid cyst without mass effect. Executive dysfunction was observed in follow-up neuropsychological tests in all patients. When investigating status epilepticus, neurosyphilis as a cause must not be forgotten.
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PMID:Neurosyphilis presenting with status epilepticus. 1730 12


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