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)

Progressive rubella panencephalitis (PRP) is a slow virus infection of the central nervous system. PRP was first reported in 1974, and fewer than 20 cases have been reported since then. All patients were male who were between the ages of 8 and 21 years at onset, and most had signs of congenital rubella syndrome. Although PRP may exhibit clinical features resembling SSPE, the age at onset is much older and the clinical course is more benign. The main neurological features of PRP are dementia, cerebellar ataxia, and seizures. Increases in antirubella antibody titer and IgG are found in the CSF and diffuse atrophy of the brain with ventricular dilatation may be found on MRI. The pathomechanism of PRP remains unclarified.
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PMID:[Progressive rubella panencephalitis]. 910 95

Subacute sclerosing panencephalitis (SSPE) is a rare infectious central nervous system (CNS) disease with a poor prognosis. We reported on the case of an adolescent girl with SSPE and characteristic periodic electroencephalographic (EEG) complexes. Her neurological deficits including generalized myoclonic seizures improved after intraventricular interferon (IFN) treatment. However, unusual EEG patterns consisting of bisynchronous occipital spikes preceding periodic complexes developed in follow-up EEGs.
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PMID:Unusual electroencephalographic findings in subacute sclerosing panencephalitis: a case report. 913 96

An 8 year old girl presented with progressive change of personality and spastic ataxia since 4 weeks. A year before she had developed focal grand-mal-seizures; at this time laboratory and radiologic findings were normal. The EEG on admission demonstrated marked changes with partially focal, partially generalized hypersynchronic activity, but no SSPE-typical Radermecker-complexes. There were no cells in the cerebrospinal fluid (CSF), a slightly increased level of protein and a normal glucose. Isoelectric focusing showed predominantly measles-specific oligoclonal IgG bands in the CSF. In the magnetic resonance tomography multiple focal white matter lesions in the basal ganglia as well as in cortical and occipitoparietal regions could be seen. At the age of two the girl had suffered from measles, the child didn't receive any vaccination. The combination of history, CSF-, MRI-results and EEG lead to the diagnosis of subacute sclerosing panencephalitis (SSPE). After 3 months the clinical and radiological abnormalities had markedly increased. On the background of this history SSPE should be considered as differential diagnosis in patients with changes of personality.
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PMID:[Subacute sclerosing panencephalitis (SSPE) as differential diagnosis in severe personality changes and ataxia--case report and literature review]. 924 17

Subacute sclerosing panencephalitis (SSPE) is an inflammatory neurodegenerative disease related to the persistence of measles virus. Although its frequency is declining because of measles eradication, we still have some cases being diagnosed. With the aim to describe epidemiological aspects of SSPE in Brazil, we sent a protocol to Child Neurologists around the country, 48 patients were registered, 27 (56%) were from the southeast region, 34 (71%) were male and 35 (73%) white, 27 (56%) had measles, 9 (19%) had measles and were also immunized, 7 (14%) received only immunization, 1 patient had a probable neonatal form. Mean time between first symptoms and diagnosis was 12 months (22 started with myoclonus or tonic-clonic seizures, 7 (14%) with behavioral disturbances); 36 patients (75%) had EEG with pseudoperiodic complexes. Follow up performed in 28 (58%) patients showed: 12 died, 2 had complete remission and the others had variable neurological disability. Our data shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.
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PMID:Subacute sclerosing panencephalitis. Clinical aspects and prognosis. The Brazilian registry. 1041 14

The aim of this study is a clinical and electroencelographic analysis of those SSPE patients who suffered epileptic seizures in course of the disease. The material is based on an analysis of a computed database including 1180 case histories from multiple hospitalizations of 248 SSPE patients (141 males, 107 females) in years 1978-1995. The analysis was made using computer system EPI-INFO 6. The average age of SSPE onset was 12.3 +/- 4.5 (range 4-27.5). 74.2% of patients developed the disease before the age of 15. Epileptic seizures occurred in 43.5% (N = 108) of all SSPE patients in different stages of the disease. In 47 patients (M-21; F-26) they were observed among first SSPE symptoms, in 41 of those the seizures had the form of GTCS. Epileptic seizures were more frequent in patients with SSPE onset before the age of 15 (50.5%) than in patients with later onset (23.4%). More frequent occurrence of epileptic seizures and epileptiform changes in EEG in children under 15 can be associated with specificity of developmental age: immaturity of nervous and endocrine system and decreased convulsant threshold.
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PMID:[Epileptic seizures in relation to the age of SSPE onset]. 1046 23

A 5-year-old boy presented with an acute ataxia and altered mental status. Although he initially recovered from these symptoms, he presented a second time with myoclonus and seizures and rapidly became vegetative. Cerebrospinal fluid studies, magnetic resonance imaging, and brain biopsy all confirmed the presence of subacute sclerosing panencephalitis. Despite courses of therapy with cimetidine, amantadine, ribavirin, and inosine, no clinical improvement has been seen. Clinicians need to be alert to the possibility of subacute sclerosing panencephalitis even in the vaccinated child in the appropriate clinical setting.
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PMID:Subacute encephalopathy in a 5-year-old boy. 1052 34

Electroencephalogram (EEG) helps in diagnosing seizures and various childhood epileptic syndromes that have significant treatment implications. It represents the electric activity of the brain. Activating procedures can further increase the diagnostic yield. Besides diagnosing, it has a role in discontinuation of treatment and drug monitoring. EEG has its own limitations--it can be normal in an epileptic child and abnormal in a normal one. Electrical activity of the brain varies with age and physiological states like sleep. In neonates the background activity is much more important than epileptiform discharges unless these are repetitive and focal. Pseudo-epileptiform patterns like fourteen and six Hz activity can be confusing. Common typical epileptiform patterns seen in childhood are hypsarrhythmia in infantile spasm, burst suppression in early infantile epileptic encephalopathy, generalised 3 Hz spike wave discharges in absence seizures and periodic complexes in SSPE. EEG patterns like low voltage, electro-cerebral inactivity, multifocal spikes and excessive slow background are associated with poor outcome in neonates. Newer approaches have increased the yield and made localisation more precise. Epilepsy is a clinical diagnosis but EEG has significant role in substantiating the clinical suspicion, classification and management.
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PMID:Rational use of EEG in childhood epilepsy. 1112 91

In this study we reviewed the clinical, electrophysiological and neuroimaging data of 21 patients with epilepsia partialis continua (EPC), which is a rare form of epilepsy with focal motor seizures persisting hours to years. We found infections, cerebrovascular events and tumors as the most common causes of EPC in adults. SSPE was also shown as a cause of EPC. EPC in SSPE patients was resistant to therapies and persisted more than 1 year. EPC is usually a predictor of poor outcome and 29% of patients died after EPC in this study. As prognosis is usually bad and as response to treatment is poor in patients who had EPC, early diagnosis and treatment of the underlying cause is important. Although the most common etiologies are infections, cerebrovascular events and tumors, if EPC persists several months SSPE should be kept in mind as a rare cause of EPC. MRI should be repeated in chronic cases to show dysplastic cortex, which was shown in 1 patient in this study.
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PMID:Epilepsia partialis continua: clinical and electrophysiological features of adult patients. 1120 34

This paper reports the clinico-pathological data in a French family with orthochromatic leukodystrophy. The parents were first cousins and had seven children. Among those, two sisters and one brother presented with neurological signs, with onset around the 5(th) decade, including a dementing syndrome of frontal type, a tetrapyramidal syndrome, seizures, and, in one sibling, a cerebellar syndrome. CT scan or MRI showed diffuse involvement of the white matter. The neurological signs worsened progressively leading to death within 11 and 22 months. Neuropathological examination was performed in two cases. It revealed characteristic orthochromatic leukodystrophy. In one case, the presence of pigmented macrophages and astrocytes was suggestive of Van Bogaert and Nyssen disease. However there were some atypical features including the absence of pigmented cells in the second case whose clinical course was shorter, and the cavitary appearance of the white matter changes with a relative increase in the number of oligodendrocytes raising the issue of a possible link between this condition and cavitary orthochromatic leukodystrophies.
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PMID:[Familial orthochromatic leukodystrophy: clinicopathological study of two cases]. 1128 64

Subacute Sclerosing Panencephalitis (SSPE) is becoming less frequent in Morocco since the generalization of measles vaccination in 1982. The aim of this study was first to analyze the semiological and elecrophysiological profiles of epilepsy in SSPE in both 'disease-revealing' seizures and sequellar ones, and second, to study the evolution of epilepsy and its possible prognostic value in SSPE. Among the neurological manifestations of SSPE, epilepsy is not as rare as frequently reported in the literature. In this longitudinal series concerning 70 cases of SSPE, 30 developed epilepsy. In two-thirds of our patients the epileptic seizures started in the first year of evolution; they revealed the SSPE in 23% of the cases and were sequellar in the rest. Seizures revealing the SSPE were widely dominated by partial seizures, secondarily generalized or not (86%), suggesting a focalized encephalitic process. Conversely, sequellar seizures were in most cases generalized tonic-clonic (43%), and therefore compatible with an already spread process. The EEG contributed both to the diagnosis of SSPE and to that of the epilepsy, showing epileptic abnormalities in ten patients. The outcome of epileptic seizures was very favorable under antiepileptic drugs, while that of SSPE remained severe and not modified by epilepsy. The authors underline the relative frequency of epilepsy in SSPE, the interest of the distinction between revealing and sequellar seizures, the good prognosis of epilepsy under adequate therapy, and the absence of prognostic value of epilepsy in SSPE.
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PMID:[Epileptic seizures and epilepsy in subacute sclerosing panencephalitis (report of 30 cases]. 1181 Sep 89


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