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Results for “Subacute Sclerosing Panencephalitis”

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At least 19 recordsLinked to original sources
PubMed1997

Alpha-interferon in the treatment of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is an inexorably progressive disease with no effective therapy. Recent trials of intrathecal and intraventricular alpha-interferon yielded controversial results. We tried intrathecal or intraventricular alpha-interferon in four patients with subacute sclerosing panencephalitis. None of them had evidence of improvement. We reviewed the previously published studies on the use of alpha-interferon in subacute sclerosing panencephalitis. Further international collaborative studies are needed to determine the role of alpha-interferon in the treatment of subacute sclerosing panencephalitis.

Adult↗
PubMed2002

Neurodegenerative mechanisms in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is caused by persistent brain infection of mutated measles virus, showing inflammation, neuronal loss, and demyelination. We neuropathologically examined six autopsy cases of subacute sclerosing panencephalitis, using in situ nick end-labeling and immunohistochemistry. Both the neurons and glial cells in the cerebral cortex showed immunoreactive nuclei in the nick end-labeling in two cases with disease duration within 2 years, whereas they were confined to the glial cells in the demyelinated cerebral white matter in three cases with disease duration ranging from 2 to 10 years. The nuclei and cytoplasm were immunoreactive for 8-hydroxy-2'-deoxyguanosine and 8-hydroxyguanosine, markers of oxidative damage to DNA and ribonucleic acid, respectively, in the cerebral cortex in three cases with disease duration within 9 years. In contrast, 4-hydroxy-2-nonenal-modified proteins, products of lipid peroxidation, were deposited in the demyelinated white matters in four cases with disease duration longer than 9 years. In three cases with long survival, the expression of glial glutamate transporters was reduced in the cerebral cortex. It is speculated in subacute sclerosing panencephalitis that apoptosis and oxidative stress to DNA can contribute to the early neuronal damage, whereas lipid peroxidation and disturbed glutamate transport may be related to the subsequent neurodegeneration.

Adolescent↗
PubMed1989

Postmortem detection of measles virus in non-neural tissues in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis, a rare, progressive, fatal central nervous system disease of children, is caused by measles virus. Clinical signs occur months to several years after recovery from acute measles infection. It is not known where the virus persists while the disease is inapparent. Involvement of organs outside the central nervous system has rarely been documented. To search for possible peripheral reservoirs of measles virus we used in situ hybridization to probe for measles virus RNA and immunocytochemical studies to localize measles virus antigens ina variety of organs taken at autopsy from confirmed cases of subacute sclerosing panencephalitis. Seven of 9 cadavers were found to contain measles virus RNA or antigens, or both, in at least one location outside the central nervous system. These sites included lymphoid organs such as thymus, spleen, lymph nodes, and tonsil, suggesting a role for lymphocytes in disease pathogenesis. Virus was also detected in kidney, lung, and glandular tissues such as pancreas, adrenal, and pituitary. These reservoirs may provide the antigenic stimulus leading to the elevated response characteristic for subacute sclerosing panencephalitis.

Adolescent↗
PubMed2002

Proton magnetic resonance spectroscopy in a case of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is an encephalopathy caused by a persistent measles virus infection. We examined a 13-year-old girl with subacute sclerosing panencephalitis and performed a magnetic resonance spectroscopic study to evaluate the in vivo pathophysiologic abnormality. The results suggested the occurrence of inflammatory processes and glial proliferation prior to neuronal loss even in magnetic resonance imaging (MRI)-negative regions as well as in MRI-positive regions. The additional resonance peaks were also detected, but further studies should be performed to determine the precise pathophysiologic mechanisms. Novel in vivo modalities such as spectroscopy would be useful as a tool to measure responses to therapy.

Brain↗
PubMed1977

Imbalance of k/lambda ratios associated with high measles antibody titers in fractionated serum immunoglobulins of patients affected with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a neurological disease which is characterized not only by the appearance of oligoclonal immunoglobulins in cerebrospinal fluid (CSF) and serum but also by high measles antibody titers and disturbed k/lambda ratios, as well in serum as in CSF. These findings lead to the hypothesis that specific immunoglobulins are produced by hyperimmunisation against measles and/or measles-like virus. Therefore the serum IgG from subacute sclerosing panencephalitis patients were fractionated by column isoelectric focusing and the k/lambda light chain ratio and the measles antibody titer of each fration were determined. The highest k/lambda ratios and the highest measles antibody titers were found mainly in two fractions at a pH ranging from 8.94-8.51 for the first and 8.88-8.24 for the second.

Antibodies, Viral↗
PubMed2006

Nonconvulsive status epilepticus on electroencephalography in a case with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a neurodegenerative disease with a poor prognosis. We report a case of a 5 1/2-year-old boy who had emotional lability, cognitive difficulties, and myoclonia after a mild closed head injury. The magnetic resonance image of the brain and computed tomographic scan of the head were normal. His electroencephalogram (EEG) showed continuous nonconvulsive status epilepticus activity, which could not be suppressed with intravenous diazepam. After treatment with phenytoin for 2 days, an EEG showed periodic high-amplitude sharp-and-slow-wave complexes, which were also not suppressed with intravenous diazepam. Since the patient had measles at 5 months of age, subacute sclerosing panencephalitis was considered, and the diagnosis was confirmed by the presence of measles antibodies in cerebrospinal fluid.

Anticonvulsants↗
PubMed1996

Use of a brief assessment examination in a study of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a degenerative disease affecting children and young adults that remains a distinct and relatively frequent problem in developing countries worldwide. Recent advances in treatment prompted a study at King Faisal Specialist Hospital, Riyadh, Saudi Arabia, using oral isoprinosine and intraventricular alpha-interferon. Initially, the Neurological Disability Assessment and Index was used for tracking patient progress. However, an additional means of assessment was deemed necessary and the Brief Assessment Examination was devised. Largely cognitive based, the Brief Assessment Examination appeared to be more sensitive to mental status changes than the Neurological Disability Assessment and Index, though it correlated modestly with the Neurologic Disability Assessment and Index and more strongly with staging. In addition, the Brief Assessment Examination can be administered by technician-level staff with a minimum of training. Though more study is needed, preliminary findings suggest that the Brief Assessment Examination should be a useful tracking tool for subacute sclerosing panencephalitis, particularly in the developing world.

Adolescent↗
PubMedDate not supplied

[Subacute sclerosing panencephalitis].

Subacute sclerosing panencephalitis is a slowly progressive neurodegenerative disorder occurring in childhood and adolescence and is characterised by dementia, ataxia, myoclonias and other neurological focal signs, with an invariably fatal outcome. The author reviews the subject, focussing on epidemiology, clinical features, measles virus behaviour, host immune reactions, measles prophylaxis and treatment proposals.

Antiviral Agents↗
PubMed1991

Atypical electroencephalographic pattern in a patient with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) has become rare since the widespread use of the measles vaccine. In this patient with a 5 month history of seizures and progressive dementia due to SSPE, the atypical electroencephalographic pattern was characterized by generalized spike-and-wave discharges, maximal on the right, associated with clinical seizures. After diazepam (intravenously), more typical periodic complexes appeared.

Adolescent↗
PubMed1996

Subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE), a neurodegenerative disease caused by a persistent "slow virus infection" with a mutated measles virus, is endemic in much of the developing world. Its incidence will increase in the USA, not only in immigrants, but also because of the 1988-1990 measles epidemic. This report reviews the pathogenesis, clinical and laboratory diagnosis, and future perspectives in treatment and prevention.

Adolescent↗
PubMed1999

Subretinal lesions in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disorder. A 9-year-old boy was seen who had progressive neurocognitive decline, myoclonic jerking of the extremities, and an abnormal result of an electroencephalogram (EEG). Ophthalmoscopic examination revealed multifocal subretinal lesions. The diagnosis of SSPE was made on the basis of the clinical examination and elevated serum and spinal fluid measles titer. We describe subretinal lesions in a patient with SSPE.

Child↗
PubMed1993

Synthesis of antibodies against measles virus and myelin by in vitro stimulated B-cells derived from patients with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) patients carry persistent measles virus infection in the brain. Furthermore, the blood lymphocytes contain viral RNA. Lymphocytes derived from 6 SSPE patients were stimulated with Epstein-Barr virus (EBV). Production of antibodies against measles virus of the IgG isotype was detected in the supernatants of cell cultures of all patients, regardless of the disease's activity, duration or interferon therapy. In contrast, only some of these cell cultures also produced antibodies against myelin.

Adolescent↗
PubMed2006

Proton MR spectroscopy in the diagnosis of early-stage subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurological disorder of childhood and early adolescence. In this report, we present brain magnetic resonance spectroscopy (MRS) and conventional magnetic resonance MR imaging findings of early-stage (stage II) SSPE in an 8-year-old child. MRS demonstrated increased myo-inositol/creatine and choline/creatine ratios, and a decreased N-acetyl aspartate/creatine ratio. Conventional MR imaging demonstrated nonspecific, patchy T2 hyperintensities in the periventricular white matter and a nonspecific focus of T2 hyperintensity in the right frontal subcortical white matter. The MRS findings suggested active inflammation and demyelination as is seen in early-stage SSPE.

Child↗
PubMedDate not supplied

CT of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a progressive, frequently fatal slow virus infection of the brain attacking children and young adults and caused by measles virus. Computed tomography (CT) of the brain in 15 patients with this disease was normal in seven and abnormal in eight. CT demonstrated varying degrees of cortical atrophy associated with focal and multifocal low density lesions of the white matter. Two cases demonstrated low density lesions of the caudate nuclei. The CT lesions were seen in chronic cases and reflect the gliosis and atrophy that occur in this disease. Differential diagnoses include other viral infections and demyelinating and dysmyelinating diseases. Diagnostic laboratory evaluation includes serum and cerebrospinal fluid titers for measles antibodies, cerebrospinal fluid protein electrophoresis, electroencephalography, and cranial CT.

Adolescent↗
PubMed2006

Rituximab in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurological disorder of childhood and early adolescence. It is caused by a persistent measles virus infection of the brain without any available treatment to date. The physiopathology of the disease is largely unknown. Considering the potential role of humoral immunity in the pathogenesis of SSPE, one patient was given compassionate anti-CD20 antibodies. However, disease progression under treatment led to reconsider B cell involvement in this pathology. Nevertheless, we observed that carbamazepine was useful in improving life quality in our patient, and should be considered as a first-line drug. To date, measles vaccination remains the only solution to SSPE.

Antibodies, Monoclonal↗
PubMed1996

MRI in subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease.

Adolescent↗
PubMed1978

Subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis (SSPE) is now considered to be caused by measles virus. There are four diagnostic criteria, namely the clinical picture, a characteristic EEG, serology of serum and CSF and brain histology. A register of cases in the U.K. has been kept since 1971, and up to September 1977, ninety-six patients have been reported. The male/female ratio is 2 : 1. The disease most commonly affects children between the ages of nine and eleven years who usually have had measles at a very early age. The average delay between the measles infection and onset of SSPE was 6.8 years and of the thirty-four patients known to have died the average survival times was 1.2 years. There are still many questions about the pathogenesis and epidemiology of SSPE that have yet to be answered.

Child↗
PubMed1986

Diagnostic and pathogenetic aspects of subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis, usually a rapidly progressive and fatal disease, is a slow virus infection, where measles virus persists in cells of the CNS and in lymphocytes. Four patients, 3 boys and 1 girl, are described, who presented a characteristic disease course, beginning at the age of 12 to 14 years after they had contracted measles infection during infancy or early childhood. The diagnostic criteria including clinical and laboratory CSF findings are summarized, and epidemiologic features related to measles and measles immunization are briefly discussed. In two patients specific measles virus protein antibodies in serum and CSF were analyzed. The results confirm postulated mechanisms for viral persistence in the CNS and suggest in addition a possible role of the viral protein H in the pathogenesis of SSPE.

Adolescent↗