Disease relevance of Subacute Sclerosing Panencephalitis

• Measles-virus proteins in the brain tissue of patients with subacute sclerosing panencephalitis: absence of the M protein [1].
• Elevated IgG distributed in oligoclonal bands is characteristically observed in the cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS) or subacute sclerosing panencephalitis (SSPE) [2].
• The six most-encountered diagnoses, in declining order, were: subacute sclerosing panencephalitis; neuronal ceroid lipofuscinosis; tuberous sclerosis with degeneration; West disease, or idiopathic degenerative encephalopathy associated with infantile spasms; Werdnig-Hoffmann disease, and hereditary spastic paraplegia [3].
• Attempts to find similar material in cases of chronic MS, subacute sclerosing panencephalitis and in perivenous encephalomyelitis were unsuccessful [4].
• The sensitivity of the system was determined by diluting the positive clones from this SSPE phage-displayed library at a ratio of 10(-6) into another phage-displayed library that did not contain any detectable MV Ags; after six rounds of panning, the positive clones comprised 34% of all phage and were also shown to be MV nucleocapsid specific [5].

Psychiatry related information on Subacute Sclerosing Panencephalitis

• Unexplained sudden amnesia, postencephalitic Parkinson disease, subacute sclerosing panencephalitis, and Alzheimer disease: does viral synergy produce neurofibrillary tangles [6]?
• Neurofibrillary tangle formation, a cardinal characteristic of Alzheimer's disease, is also a feature of several other neurodegenerative disorders, including subacute sclerosing panencephalitis (SSPE) [7].

High impact information on Subacute Sclerosing Panencephalitis

• Eluates from individual MS plaques have distinct IgG patterns; in contrast, those from separate SSPE brain areas have essentially identical IgG patterns [2].
• SSPE strains LEC, IP-3, Biken, and D.R., on the other hand, were mostly cell-associated in Vero and FB cultures, although atypical cell-free particles were produced by strains Biken and IP-3 [8].
• No defect was found in cytolytic measles antibody in sera or cerebrospinal fluid from patients with subacute sclerosing panencephalitis, nor was the alternative C pathway impaired in sera from these patients [9].
• Inosiplex for SSPE [10].
• Levodopa in subacute sclerosing panencephalitis [11].

Chemical compound and disease context of Subacute Sclerosing Panencephalitis

• Intravenous and intrathecal adenine arabinoside phosphate in a child with subacute sclerosing panencephalitis [12].
• In both infected systems, C6/ SSPE and C6/CDV, we observed a total loss of catecholamine-stimulated beta-adrenergic receptor-dependent methylation, whereas the beta-receptor-independent methylation of phospholipids was unchanged [13].
• Review of 38 cases of subacute sclerosing panencephalitis: effect of amantadine on the natural course of the disease [14].
• Nagahata-like sequences are found in the M genes of at least five other SSPE viruses isolated from three continents [15].
• We conclude that in MV infections, including SSPE, the glycoprotein tails are involved not only in virus envelope assembly but also in the control of virus-induced cell fusion [16].

Biological context of Subacute Sclerosing Panencephalitis

• The data show that the loss of the ET-1 signaling pathway in C6/SSPE and C6/CDV cells is due to a receptor downregulation at the transcriptional level [17].
• F(ab')2 fragments prepared from SSPE IgG retained their activity, which showed that the assay measures a true antigen-antibody reaction rather than nonspecific adherence to IgG to MBP [18].
• CONCLUSION: To our knowledge, this study is the first to demonstrate the possibility that the IL-4 promoter gene -589 T gene polymorphism with increased IL-4 synthesis in combination with IRF-1 allele 1 confers host genetic susceptibility to SSPE in Japanese subjects [19].
• For these four patients, CSF ribavirin concentrations were maintained at a level at which SSPE virus replication was almost completely inhibited in vitro and in vivo, whereas the concentration was lower in the patient without clinical improvement [20].
• The frequency of the genotype combination of IL-4 promoter -589 T and IRF-1 allele 1 (at least 1 allele) in patients with SSPE was much higher than that in the controls (47.7% vs 22.0%; P =.003, chi2 analysis) [19].

Anatomical context of Subacute Sclerosing Panencephalitis

• To further characterize the TCR V alpha and V beta repertoire in MS plaque tissue, we examined a series of 26 histologically well-characterized central nervous system (CNS) tissue specimens from six MS patients as well as samples from five normal postmortem cases and a case of subacute sclerosing panencephalitis [21].
• Indirect immunofluorescent analysis revealed that sera from five patients with subacute sclerosing panencephalitis possessed IgD antibodies directed against measles virus components in persistently infected HeLa cells [22].
• Single cell clones from the antisense-transfected C6/SSPE cells appear to be totally free of virus in cocultivation with Vero cells, suggesting that they are really cured [23].
• There were no significant differences between the CSF results in patients with NID and MS but the OKT3 lymphocytes were reduced in CSF samples from patients with SSPE [24].
• The results suggest that T-lymphocytes are involved in the pathogenesis of both MS and SSPE [25].

Gene context of Subacute Sclerosing Panencephalitis

• Thus, it is not likely that CD46 gene alteration has a role as a host susceptibility factor in the development of SSPE [26].
• Contribution of the interleukin 4 gene to susceptibility to subacute sclerosing panencephalitis [19].
• The frequencies of HLA-A, B, C, DR and DQ antigens were studied in 63 Japanese patients with subacute sclerosing panencephalitis (SSPE) [27].
• The CSF of SSPE patients also showed relatively higher levels of IL-10 than that of the NIN group [28].
• Elevated interleukin-12 and CXCL10 in subacute sclerosing panencephalitis [28].

Analytical, diagnostic and therapeutic context of Subacute Sclerosing Panencephalitis

• Survival data from these 98 patients were compared by life-table analysis with survival in three SSPE control groups drawn from SSPE patients contracting the disease in Israel, Lebanon, or the United States at about the same time as the inosiplex-treated patients but treated differently or not at all [29].
• Cocultivation of uninfected C6 cells with C6/SSPE cells (9:1 ratio) resulting in contact-mediated transmission of measles virus showed that the 125I-endothelin 1 binding activity was gradually lost as a consequence of persistent virus infection [30].
• Sequence analysis of the positive clones identified fragments of the nucleocapsid protein of MV, the cause of SSPE [5].
• The reduced anti-M antibody in sera from patients with SSPE was demonstrated whether immune precipitation was performed with wild-type measles virus or SSPE virus proteins [31].
• MHC class I mRNA expression was examined in formalin-fixed brain tissue from 6 SSPE patients by in situ hybridization [32].

References