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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 24-month-old male who developed rapidly progressive subacute sclerosing panencephalitis 17 months after measles infection. This patient had a history of measles infection at the age of 7 months and manifested acute encephalitis 1 month later. Developmental delay observed after encephalitis began to improve after a few months. His control electroencephalogram was normal at the age of 14 months. He was admitted to the hospital with flexor spasms and sudden head drops at the age of 24 months. His electroencephalogram revealed slow waves in the posterior regions of the brain. Vigabatrin was begun; his seizures increased with vigabatrin, and adrenocorticotropic hormone was added to the therapy. After five doses of adrenocorticotropic hormone, his clinical findings deteriorated rapidly. His second electroencephalogram revealed periodic discharges synchronized with myoclonias. He was diagnosed as having subacute sclerosing panencephalitis on the basis of clinical and laboratory findings. He lapsed into a vegetative state within a week and died at the age of 25 months. We report this rapidly progressive case to emphasize the importance of recognition of subacute sclerosing panencephalitis before applying steroids in children with myoclonic seizures.
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PMID:Subacute sclerosing panencephalitis: a case with fulminant course after ACTH. 1524 97

The aims of the present study are to establish the subacute sclerosing panencephalitis (SSPE) incidence in Bulgaria for the 25-year period 1978-2002; to analyze the SSPE incidence prior to, and in the period of, routine measles immunization; and, to analyze the clinical characteristics of SSPE. SSPE was diagnosed in a total of 40 children; 28 of were diagnosed between 1978 and 1984, and 12 between 1995 and 2002. Thirty-eight cases (95%) were non-immunized and have had an early measles infection (mean age 16 months). The SSPE onset occurred primarily between 8 and 11 years of age (52.5%) with a mean latent period of about 7 years after the measles infection. After the 10-year disease-free period (1985-1994), the SSPE incidence increased between 1995 and 2002 because of the 1991-1992 measles epidemic. During the period 1995-2002 children with earlier measles infection and earlier SSPE onset predominated, compared to the period 1978-1984. The initial clinical manifestations included intellectual deterioration in 35%, extrapyramidal hyperkinesias in 29%, epileptic seizures in 15%, hemiparesis in 10%, and visual disturbances in 10% of the cases. Nine children (22.5%) demonstrated an atypical onset. A rapidly progressive course was observed in 4 children (10%) and a chronic progressive course with pseudoremissions over 2 years-in 8 cases (20%). Our analysis of the SSPE incidence in Bulgaria for the 25-year period (1978-2002) supports the importance of early measles infection as a crucial risk factor for this persistent neuroinfection. Moreover, it confirms the role of routine measles immunization in SSPE prevention.
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PMID:Clinical and epidemiological characteristics of subacute sclerosing panencephalitis in Bulgaria during the past 25 years (1978-2002). 1525 56

Measles inclusion body encephalitis (MIBE) is a disease of the immunocompromised host and typically occurs within 1 year of acute measles infection or vaccination. We report a 13-year-old boy who had chronic granulomatous disease and presented 38 days after stem cell transplantation with afebrile focal seizures that progressed despite multiple anticonvulsants. After an extensive diagnostic evaluation, brain biopsy was performed, revealing numerous intranuclear inclusion bodies consistent with paramyxovirus nucleocapsids. Measles studies including reverse transcriptase-polymerase chain reaction and viral growth confirmed measles virus, genotype D3. Immunohistochemistry was positive for measles nucleoprotein. Despite intravenous ribavirin therapy, the patient died. MIBE has not been described in stem cell recipients but is a disease of immunocompromised hosts and typically occurs within 1 year of measles infection, exposure, or vaccination. Our case is unusual as neither the patient nor the stem cell donor had apparent recent measles exposure or vaccination, and neither had recent travel to measles-endemic regions. The patient had an erythematous rash several weeks before the neurologic symptoms; however, skin biopsy was consistent with graft-versus-host disease, and immunohistochemistry studies for measles nucleoprotein were negative. As measles genotype D3 has not been seen in areas where the child lived since his early childhood, the possibility of an unusually long latency period between initial measles infection and MIBE is raised. In addition, this case demonstrates the utility of brain biopsy in the diagnosis of encephalitis of unknown cause in the immunocompromised host.
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PMID:A new complication of stem cell transplantation: measles inclusion body encephalitis. 1552 95

The 2-year history of a 10-year-old boy with subacute sclerosing panencephalitis is presented. After 6 months of epilepsy manifested by atypical absences and myoclonic-atonic seizures with an electroencephalographic (EEG) correlation of generalized spike-and polyspike-wave discharges, the child developed chorioretinitis and demonstrated a dramatic intellectual decline during corticosteroid treatment. Neuroimaging investigations did not demonstrate any pathologic changes, including at the time of fully developed disease, with neurologic deficits, periodic spasms, polymorphic epileptic seizures, and dementia. The typical generalized periodic complexes and general slowing replaced the epileptic abnormalities in the EEG. The initial anticonvulsant treatment was temporarily effective, and the purposeful isoprinosine therapy had no significant beneficial effect. The subacute sclerosing panencephalitis rapidly developed to stages III to IV, and only during the vegetative state did computed tomography show marked brain atrophy. This case is among the few described in the literature with atypical absences and myoclonic-atonic seizures as the first manifestation of subacute sclerosing panencephalitis.
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PMID:Case of subacute sclerosing panencephalitis with atypical absences and myoclonic-atonic seizures as a first symptom. 1552 62

Subacute sclerosing panencephalitis is a progressive disorder which also presents with various types of seizures, mainly myoclonic jerks, atonic attacks and tonic-clonic seizures. We report two cases, documented by video-EEG that during the course of the disease also presented with tonic seizures. The differential diagnosis of non-epileptic paroxysmal events might prove to be a problem.
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PMID:Tonic seizures in subacute sclerosing panencephalitis: a video-illustration of two cases. 1616 31

Clinical and laboratory characteristics of 39 patients with adult onset subacute sclerosing panencephalitis (SSPE) are described and compared to those of juvenile onset patients regarding preceding measles, age at onset, gender, interval between onset and diagnosis, clinical profile, and course during follow up. Diagnosis was based on clinical and electroencephalographic findings and raised anti-measles antibody titres in cerebrospinal fluid. Mean age at SSPE symptom onset was 20.9+/-4.9 years and mean interval from onset to diagnosis was 6.3+/-9.6 months. Referral diagnosis was accurate in only 12 patients. Presenting symptoms included myoclonus, behavioural changes, seizures, and cognitive, visual, and extrapyramidal disturbance. All patients received symptomatic therapy; 19 also received disease modifying agents. Five of seven pregnant women had successful deliveries. The follow-up period varied widely (maximum 60 months, median 9 months). The profile of adult onset SSPE did not differ from the rest of the cohort, except for a longer interval between measles infection and symptom onset (p<0.0001). SSPE in adults poses diagnostic challenges for clinicians. A high index of suspicion and appropriate investigations are necessary for early diagnosis and counselling.
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PMID:Adult onset subacute sclerosing panencephalitis: clinical profile of 39 patients from a tertiary care centre. 1646 98

Subacute sclerosing panencephalitis (SSPE) can show variations in its clinical course. Typical initial symptoms consist of intellectual decline, deterioration in school performance, and myoclonic jerks. Atypical forms of presentation such as generalized seizures and acute or subacute coma can be observed rarely. This report describes a 5-year-old boy with an atypical feature of SSPE, generalized seizures. For 1 month his only symptom was seizures, followed by personality and behavioral changes and myoclonic jerks. A diagnosis of SSPE was made based on the boy's history of measles, characteristic electroencephalogram changes and compatible magnetic resonance, and elevated anti-measles antibody titers in the cerebrospinal fluid and serum. The case presented in this article is a good example of SSPE in which, at early stages, some of the signs and symptoms can lead to an erroneous diagnosis.
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PMID:Subacute sclerosing panencephalitis with generalized seizure as a first symptom: a case report. 1706 Jun 98

Subacute sclerosing panencephalitis (SSPE) is a progressive disease caused by wild-type measles virus leading to premature death. Early diagnosis may help in medical interventions and counseling. The aim of this study was to ascertain diagnostic errors and their possible causes. Retrospective case record analysis of patients with subacute sclerosing panencephalitis, evaluated over a 10-year period, was performed. The following data were analyzed: initial symptoms and diagnosis, interval between onset of symptoms to diagnosis, and implications of delayed diagnosis. Among the 307 patients evaluated, initial diagnosis by various health care professionals was other than subacute sclerosing panencephalitis in 242 patients (78.8%). These included seizures, absence seizures, metachromatic leukodystrophy, Schilder's disease, cerebral palsy, hemiparkinsonism, Wilson's disease, vasculitis, spinocerebellar ataxia, motor neuron disease, nutritional amblyopia, tapetoretinal degeneration, catatonic schizophrenia, and malingering, among others. The interval between precise diagnosis and first reported symptom was 6.2 +/- 11.3 months (range, 0.2-96 months; median, 3 months). Forty-four patients (14.3%) who had symptoms for more than 1 year before the precise diagnosis had a protracted course as compared to the rest of the cohort ( P = .0001). Early and accurate diagnosis of subacute sclerosing panencephalitis needs a high index of suspicion.
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PMID:Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital. 1764 Dec 52

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonias and eventually complete neurologic deterioration. The diagnosis is based on characteristic clinical features, periodic electroencephalography (EEG) complexes of high slow waves and increased antibody titer against measles in cerebrospinal fluid. Here, we report four SSPE cases, two of whom manifested with hemiparesis; in the third and fourth cases, cerebellar ataxia and acute encephalopathy with focal seizures were the presenting symptoms at the onset of disease, respectively. The typical periodic EEG complexes in our patients led to the diagnosis of SSPE. Our findings show that SSPE should be considered in the differential diagnosis of hemiparesis, cerebellar ataxia and acute encephalopathy, and highlight the diagnostic significance of EEG in unidentified cases.
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PMID:Atypical presentations of SSPE: a clinical study in four cases. 1799 May 84

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. The disease has been related to a persistent and aberrant measles virus infection and no effective treatment has been available. We report a case of SSPE with atypical features including seizures at onset and a fulminant course in a 8 years-old boy who had been previously immunized against measles.
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PMID:Atypical subacute sclerosing panencephalitis: case report. 1809 71


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