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)

We herein report a rare case of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and diabetes mellitus with ketoacidosis. An 18-year-old female patient was diagnosed to have diabetes mellitus and insulin therapy was thereafter initiated. At 26 years of age, she was hospitalized for diabetic ketoacidosis, soon followed by a loss of consciousness, left-sided dysmetria, and ataxic speech. MELAS was diagnosed because of the presence of ragged red fibers in a muscle biopsy. At 33 years of age, she was admitted to our hospital because of ketoacidosis and partial status epilepticus. A blood gas examination revealed as follows; arterial pH, 6.88; bicarbonate, 2.1 mmol/l; base excess - 29.8 mmol/l. The serum level of glucose had also increased to 30 mmol/l. The serum levels of lactate and B-hydroxybutyrate were elevated to 11.4 mmol/l and 1,990 micromol/l, respectively. Ketoacidosis improved by fluid replacement and continuous intravenous insulin infusion. A brain MRI demonstrated hyperintensity areas on FLAIR images in the bilateral temporal lobes and the cerebellum. A proton MRS demonstrated the abnormal lactate accumulation in the bilateral temporal and occipital lobes. Since epileptic seizures are rare in patients with diabetic ketoacidosis, such seizures may indicate the existence of MELAS syndrome.
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PMID:Ketoacidosis accompanied by epileptic seizures in a patient with diabetes mellitus and mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). 1111 21

L-2-Hydroxyglutaric aciduria (L-2-HGA) is a rare organic aciduria with a slowly progressive course regarding CNS involvement. We present a 13.5-year-old female patient who presented at the Emergency Department with a generalized status epilepticus, which promptly responded to intravenous phenytoin. CT and MRI demonstrated subcortical white matter alterations. The neurological examination revealed mild mental retardation, macrocephaly and ataxic gait with cerebellar signs. Repeated urinary organic acid analysis demonstrated increased excretion of 2-hydroxyglutaric acid which was of the L-configuration. The constellation of macrocephaly in a patient with mental retardation, cerebellar tract involvement and subcortical white matter signal alterations on MRI should alert the physician to the possibility of L-2-HGA. Although rare, epileptic seizures or even status epilepticus can be among the presenting symptoms in organic acidurias with a slow course, such as L-2-HGA.
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PMID:L-2-Hydroxyglutaric aciduria presenting as status epilepticus. 1137 7

Status epilepticus (SE) represents a medical emergency that annually affects 60,000--150,000 individuals in the United States. Selective neuronal loss in vulnerable areas has been pathologically demonstrated following convulsive SE primarily affecting the limbic system, thalamus and cerebellum. Morbidity in those cases that follow refractory SE (RSE) is poorly documented. There have been anecdotal reports of surgical treatment for this condition, especially secondary to brain lesions. We report a 6-year-old patient who was in RSE for 60 days, without a brain lesion documented by MRI. The patient underwent multiple subpial transection (MST) of the sensorimotor cortex, which by ictal EEG and ictal SPECT proved to be the epileptogenic zone. We conclude that MST should be considered as an alternative treatment for refractory partial SE.
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PMID:Treatment of refractory partial status epilepticus with multiple subpial transection: case report. 1148 52

The present study was designed to investigate whether T(2)-weighted signal changes obtained by microimaging of paraformaldehyde-fixed brain correlate with the histologically quantified damage in a model of status epilepticus (SE) induced by kainic acid in the rat. Animals were killed at several time points up to 8 weeks after a single intraperitoneal kainate (KA) injection (9 mg/kg). Perfusion-fixed brains were embedded in gelatin for MR microimaging at 9.4T. After the MRI analysis, the gelatin was removed and the brains were cryoprotected and processed for quantitative histology. Severity of neuronal damage and gliosis were assessed from thionin-stained serial sections. Correlative analysis of microimaging and histology data was done in the hippocampus, amygdala, parietal rhinal cortex (PaRH), piriform cortex (Pir), and entorhinal cortex. The relative signal intensities in T(2)-weighted images correlate with the severity of neuronal damage in the matched histological sections (correlation coefficients of 0.752-0.826). Our data show that MR microimaging ex vivo detects the degree of neuronal damage and its anatomical distribution after KA-induced SE, thus providing a useful tool for detecting the dynamics of progressive neuronal damage after prolonged seizures.
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PMID:Ex vivo MR microimaging of neuronal damage after kainate-induced status epilepticus in rat: correlation with quantitative histology. 1167 47

The primary aim of the audit described was to examine antiepileptic drug use among adult in-patients with learning disability. A secondary aim was to examine investigation in those patients. For the purposes of this paper "Learning disability" is synonymous with ICD-10 "Mental Retardation", "Mental Handicap" or "Intellectual Impairment" and should not be confused with the North American use of the term which refers to discrete, specific problems in mental abilities such as reading. A total of 75 adult patients on both the Northgate and Prudhoe sites (managed by Northgate and Prudhoe NHS Trust) were identified as being treated with antiepileptic drugs (AEDs) for postulated epilepsy. Only 12 (16%) patients were being treated with three AEDs or more, the remainder being on either one or two AEDs. All patients with a history of status epilepticus were prescribed rectal diazepam or, more rarely, intranasal midazolam. The number and type of investigations performed on patients varied widely and was sometimes limited by the patient's ability to co-operate with procedures. Overall, 28 patients were identified who might benefit from a reduction in the number of AEDs prescribed. In 15 of these patients antiepileptic drug regime was currently being reduced. The increased utilization of sleep/sedated electroencephalogram (EEG) and magnetic resonance imaging (MRI--for partial seizures), where practical, may also increase diagnostic accuracy in identifying epilepsy and its aetiology. Audit will be repeated in 12 months.
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PMID:The use of antiepileptic drugs in learning disabled people with epilepsy: an audit of adult in-patients in a treatment and continuing care service. 1174 6

In a 19-year-old patient with status epilepticus arising in the right parietal neocortex, unenhanced ictal MRI showed abnormalities mainly in the right cerebral cortex, contralateral cerebellum, and ipsilateral thalamus. The thalamus is considered a key site of functional abnormality in this patient.
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PMID:Ipsilateral thalamic MRI abnormality in an epilepsy patient. 1260 Nov 29

Diffusion-weighted MRI demonstrated bright right temporoparietal cortex, right hippocampus, and left cerebellum in a 63-year-old female suffering a focal convulsive status epilepticus. Hyperperfusion was noted in the right temporoparietal region. Two days later, a tendency to normalization of most of the diffusion and perfusion changes was noted, apart from the right hippocampus which became brighter on diffusion- and T2-weighted images. On the tenth day the apparent diffusion coefficient was slightly elevated, getting brighter on T2-weighted images with suspected mild post-contrast enhancement. We postulate that the discharging right hippocampus suffered cytotoxic edema, which later progressed to cell damage.
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PMID:Focal status epilepticus: follow-up by perfusion- and diffusion MRI. 1187 Apr 71

We reported a 37-year-old man who presented complex partial status epilepticus as the initial symptom of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). He showed fluctuating consciousness disturbance, left homonymous hemianopsia, and paroxysmal conjugated eye deviation to the left. The lactic acid level was elevated in blood and CSF, and ragged-red fibers were observed in the biopsied muscle. MRI demonstrated T2-prolonged lesions in the right occipito-parieto-temporal lobes. Since a mutation of mitochondrial DNA (A3243G) was identified, he was diagnosed as having MELAS. On an ictal record, high amplitude, rhythmic sharp waves were observed at right parieto-temporo-occipital region. High amplitude slow waves were also observed on the right hemisphere, especially in the right frontal lobe. These ictal discharges gradually decreased at their amplitude and in frequency, and then ictal EEG turned to the interictal EEG. During an ictal period, conjugated eye deviation to the left side and consciousness loss were observed. These seizures were observed once every several minutes. During the interictal period, sharp waves and sharp-wave complexes were observed frequently at right parietal and posterior temporal lobes. The venous injection of diazepam (10 mg) normalized EEG quickly. When consciousness loss, especially fluctuating, was observed in the patients of MELAS, complex partial status epilepticus should be considered.
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PMID:[A case of MELAS presenting complex partial status epilepticus]. 1188 32

Brain CT or MRI occasionally shows transient or permanent changes in the brain after status epilepticus (SE). The mechanism for these changes has not been well elucidated. We performed repeated imaging studies on a patient with febrile SE characterized by right hemiconvulsion. CT showed transient mild edema on both hemispheres immediately after the cessation of SE. The edema improved the next day. But aphasia and right hemiparesis were observed. On day 17, CT revealed edema on left hemisphere and MRI showed a high signal intensity in cortex and subcortical white matter of the left hemisphere on T2-weighted images. Although right hemiparesis and aphasia were improved, severe atrophy of the left hemisphere was noted on CT and MRI. The results suggest that brain edema observed in several days after SE but not edema observed immediately after the cessation of SE is more pathological for the permanent brain damage. Possible mechanisms of the initial brain edema and the second edema preceded severe atrophy in left hemisphere were discussed.
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PMID:Sequential changes of brain CT and MRI after febrile status epilepticus in a 6-year-old girl. 1193 19

Epileptic nystagmus (EN) is an uncommon phenomenon characterized by repetitive and rapid saccades, in association with epileptic discharges. We present a critical video-EEG recording in a patient with occipital seizures that appeared clinically as EN. The subject, male, 70 years-old, was examined because of generalized tonic-clonic seizures, preceded by left cephalic version. These were controlled using i.v. PHT, but partial seizures persisted, which we recorded using video-EEG. Clinically, we observed episodes of left conjugate deviation of the eyes, accompanied by horizontal nystagmoid movements, with a rapid leftward component and visual hallucinations. The patient did not lose consciousness. Ictal EEG: spike rhythm in the posterior right occipito-temporal region extending to adjacent and contralateral regions, followed by post-discharge of slow waves. The video-EEG was interpreted as partial oculo-clonic status epilepticus of right temporo-occipital origin. Cranial MRI: old, bilateral hemorrhaging occipital contusions associated with previous cranial injury. The picture persisted for two days, and disappeared with administration of CBZ 600 mg/d. Our patient's nystagmus seemed to be related to the critical activity recorded in the right occipito-temporal region. The co-existence of visual hallucinations and the video-EEG correlation support this possibility. This phenomenon is probably due to epileptic activation of the cortical center of saccadic movements, with a rapid phase of nystagmus, contralateral to the focus, and a slow ipsilateral phase in association with a defect in the gaze-fixing system ("leaky neural integrator") [published with videosequences].
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PMID:Epileptic nystagmus: a case study video-EEG correlation. 1196 76


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