Sclerosing panencephalitis

Infectious Dementias. One hundred years ago, the most common cause of dementia was an infection syphilis. Today, syphilis is well treated and seldom left to linger long enough to infect the brain and cause dementia, but a blood test for syphilis remains a routine part of the assessment for patients newly diagnosed with dementia. Today, the most common infectious cause of dementia is HIV/AIDS. Other, rare forms of infection-caused dementia include Creutzfeld-Jakob disease (spongiform encephalopathy) and subacute sclerosing panencephalitis (SSPE), the latter caused by the measles virus in unvaccinated children. 

Isoprinosine is an immunostimulant dmg that increases natural killer cell cytotoxicity as well as to increase the activity of T-cells and monocytes. The drug has some clinical activity against viral encephalitis such as subacute sclerosing panencephalitis and severe manifestations of immunodeficiencies. Because the purine (inosine) moiety of isoprinosine is rapidly catabolized to uric acid it should be used with care in patients with a history of gout. 

Aydin OF, Senbil N, Kuyucu N, Gurer YKY. Combined treatment with subcutaneous interferon-alpha, oral iso-prinosine, and lamivudine for subacute sclerosing panencephalitis. J Child Neurol 2003 18(2) 104-8. 

Shapshak, P., Tourtelotte, W. W., Nakamura, S., Graves, M. C., Darvish, M., Hoffman, D., Walsh, M. J., Fareed, G. C., Smid, P., Heinzman, C., Sidhu, K., Bedows, E., Rosenblatt, S., Berry, K., and Hawkins, S. 1985. Subacute sclerosing panencephalitis Measles virus matrix protein nucleic acid sequences detected by in situ hybridization. Neurology. 35 1605-1609. 

Okuno Y, Nakao T, Ishida N, Konno T, Mizutani H, Fukuyama Y, Sato T, Isomura S, Ueda S, Kitamura I, et al Incidence of subacute sclerosing panencephalitis... 

Cernescu C, Milea S. Epidemiology of subacute sclerosing panencephalitis (SSPE) in Romania between 1976-1982. Virologie 1983 34(4) 239-50. 

SPMS secondary-progressive multiple sclerosis SSPE subacute sclerosing panencephalitis TGF-/S transforming growth factor /3 TLI total lymphoid irradiation TNF-a tumor necrosis factor alpha WHO World Health Organization... 

Subacute sclerosing panencephalitis and milder encephalitis are caused by measles virus. Focal lymphocytic inflammation in the leptomeninges and perivascular spaces, with many CD4-positive cells, patchy GFAP-positive fibrillary astrocytosis, and occasional microglial nodules, involves the cerebral cortex. Diffuse mononuclear inflammation, gliosis, and loss of myelin occur in subcortical white matter. Inclusion bodies are Cowdry type A and may be seen on H E-stained slides their specific identification requires IHC. °... 

Subacute sclerosing panencephalitis Amyotrophic lateral sclerosis Chronic myelogenous leukemia... 

Other halogenated uridine derivatives have been reported to exhibit antiviral activity. Fluorodeoxyuridine has in vitro antiviral activity but is not used in clinical practice. Bromodeoxyuridine is used in subacute sclerosing panencephalitis, a deadly, virus-induced CNS disease. This agent appears to interfere with DNA synthesis in the same way as idoxuridine. The 5 -amino analogue of idoxuridine (5-iodo-5 -amino-2, 5 -dideoxyuridine) is a better antiviral agent than idoxuridine, and it is less toxic. It is metabolized in herpesvirus-infected cells only by thymidine kinase to di- and triphosphoramidates. These metabolites inhibit HSV-specific late RNA transcription, causing reduction of less infective abnormal viral proteins. 5-Bromo-2 -deoxyuridine has an action similar to that of other iodinated compounds. None of these compounds are commercially available in the United States. 

Liebert, U.G., Baezko, K., Budka, H., and ter Meulen, V. 1986. Restricted expression of measles virus proteins in brains from cases of subaeute sclerosing panencephalitis. J. Gen. Virol 67(Pt. 11), 2435 2444. 

Choppin PW, Richardson CD, Merz DC, Hall WW, Scheid A. The functions and inhibition of the membrane glycoproteins of paramyxoviruses and myxoviruses and the role of the measles virus M protein in subacute sclerosing panencephalitis. The Journal of Infectious Diseases 1981 143(3) 352-363... 

Hall WW, Choppin PW. Evidence for lack of synthesis of the M polypeptide of measles virus in brain cells in subacute sclerosing panencepheilitis. firology 1979 99(2) 443-447 Hall WW, Choppin PW. Measles-virus proteins in the biain tissue of patients with subacute sclerosing panencephalitis absence of the M protein. The New England Journal of Medicine 1981 304(19) 152-1155... 

Subacute sclerosing panencephalitis (SSPE) A complication of measles, nearly always fatal, that is due to the persistence of measles viruses in brain tissue. 

An interesting attempt has been made to treat viral diseases with the liposome-encapsulated fragment A of diphteria toxin. It has been shown in cell culture experiments (cells infected with the virus of sclerosing panencephalitis), that the liposome-immobilized A fragment decreases cell infection by 99%, whereas the free fragment itself does not influence infected cells at all. 

A. AUcan, K. Sarac, and R. Kutlu. Early and late state subacute sclerosing panencephalitis Chemical shift imagining and single voxel MR spectroscopy. Am. Neuroradiol. 24 501-506 (2003). 

Ando S, Toyoda Y, Nagai Y, Dcuta R Alterations in brain gangliosides and other lipids of patients with Creutzfeldt-Jakob disease and subacute sclerosing panencephalitis (SSPE). Jpn J Exp Med. 1984 54(6) 229-234. 

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