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Subacute Sclerosing Panencephalitis (SSPE)

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A rare, slowly progressive encephalitis caused by chronic infection with the MEASLES VIRUS. The condition occurs primarily in children and young adults, approximately 2-8 years after the initial infection. A gradual decline in intellectual abilities and behavioral alterations are followed by progressive MYOCLONUS; MUSCLE SPASTICITY; SEIZURES; DEMENTIA; autonomic dysfunction; and ATAXIA. DEATH usually occurs 1-3 years after disease onset. Pathologic features include perivascular cuffing, eosinophilic cytoplasmic inclusions, neurophagia, and fibrous gliosis. It is caused by the SSPE virus, which is a defective variant of MEASLES VIRUS. (From Adams et al., Principles of Neurology, 6th ed, pp767-8)

Also Known As:

SSPE; Subacute Sclerosing Leukoencephalitis; Encephaltis, Inclusion Body, Measles; Inclusion Body Encephalitis, Measles; Leukoencephalitis, Van Bogaert's; Sclerosing Leukoencephalitis, Subacute; Sclerosing Panencephalitis, Subacute; Leukoencephalitides, Subacute Sclerosing; Leukoencephalitis, Van Bogaert; Leukoencephalitis, Van Bogaerts; Panencephalitides, Subacute Sclerosing; Sclerosing Leukoencephalitides, Subacute; Sclerosing Panencephalitides, Subacute; Subacute Sclerosing Leukoencephalitides; Subacute Sclerosing Panencephalitides; Van Bogaert Leukoencephalitis; Van Bogaerts Leukoencephalitis; Leukoencephalitis, Subacute Sclerosing; Measles Inclusion Body Encephalitis; Panencephalitis, Subacute Sclerosing; Van Bogaert's Leukoencephalitis

Networked: 718 relevant articles (28 outcomes, 63 trials/studies)

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Disease Context: Research Results

Related Diseases

1.	Subacute Sclerosing Panencephalitis (SSPE)
2.	Measles
3.	Infection
4.	Myoclonus (Nocturnal Myoclonus)
5.	Neoplasms (Cancer)

Experts

1.	Anlar, Banu: 13 articles (09/2014 - 04/2002)
2.	Hosoya, Mitsuaki: 8 articles (04/2013 - 07/2003)
3.	Ichiyama, Takashi: 8 articles (04/2012 - 01/2006)
4.	Kusuhara, Koichi: 7 articles (06/2010 - 05/2002)
5.	Kira, Ryutaro: 6 articles (04/2010 - 05/2002)
6.	Hara, Toshiro: 6 articles (04/2010 - 05/2002)
7.	Yilmaz, Gülden: 5 articles (12/2014 - 10/2005)
8.	Yapici, Zuhal: 5 articles (12/2014 - 10/2005)
9.	Saruhan-Direskeneli, Güher: 5 articles (12/2014 - 10/2005)
10.	Demirbilek, Veysi: 5 articles (12/2014 - 10/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Subacute Sclerosing Panencephalitis:

1.	Inosine Pranobex (Imunovir)IBA 03/01/1981 - "The presented data are insufficient for a reliable conclusion to be drawn concerning the effect of various drugs on the course of SSPE, nevertheless, it seems that the best results with slowing down of the progression of the disease were observed in the group treated with isoprinosine." 08/01/1982 - "These findings suggest that inosiplex is effective in the slowly developing or chronic form of SSPE." 04/01/1983 - "Progressive CT abnormalities despite clinical improvement in SSPE treated with inosiplex." 02/01/2006 - "Inosiplex is one of the effective drugs for subacute sclerosing panencephalitis, but our in vivo and in vitro magnetic resonance spectroscopic study indicated that inosiplex affects the spectra, suggesting a possible failure of neurologic evaluation in a patient with subacute sclerosing panencephalitis treated with inosiplex." 01/01/1994 - "The effect of inosiplex in subacute sclerosing panencephalitis: a clinical and laboratory study."
2.	InterferonsIBA 04/01/1994 - "Intraventricular interferon (IFN) administration has been shown to improve the course of SSPE. " 01/01/1987 - "The reasons for the lack of clinical efficacy of interferon in SSPE are discussed." 01/01/1998 - "Parallely with similar studies in the foremost foreign centres trials of SSPE treatment were undertaken in Poland, in recent years by means of intracerebroventricular administration of interferons. " 04/01/1986 - "Intraventricular interferon deserves further study as a potential therapeutic agent in SSPE." 01/01/1986 - "This is the first report of the beneficial therapeutic effect of interferon therapy on rapidly progressive SSPE, which is lethal and poorly responded to any kind of previous therapeutic trials."
3.	Interferon-alpha (Interferon Alfa)FDA Link 11/01/1997 - "Further international collaborative studies are needed to determine the role of alpha-interferon in the treatment of subacute sclerosing panencephalitis." 11/01/1997 - "We reviewed the previously published studies on the use of alpha-interferon in subacute sclerosing panencephalitis. " 09/01/2007 - "Continuous intrathecal interferon alpha application in subacute sclerosing panencephalitis." 06/01/2005 - "Long-term follow-up of a patient with subacute sclerosing panencephalitis successfully treated with intrathecal interferon alpha." 08/30/2004 - "Expression of the interferon-alpha/beta-inducible MxA protein in brain lesions of subacute sclerosing panencephalitis."
4.	Ribavirin (Virazole)FDA LinkGeneric 07/01/2003 - "Although we were unable to perform a double-blinded clinical study for ethical reasons, our results suggest that treatment with intraventricular ribavirin and high doses of IFN may be effective for SSPE. " 12/01/2004 - "These results suggest that intraventricular administration of ribavirin is effective against SSPE if the CSF ribavirin concentration is maintained at a high level. " 07/01/2003 - "To establish this combination therapy as a safe and effective treatment of SSPE, further studies are necessary on the role of ribavirin in the pathogenesis as well as its effects in the central nervous system." 08/01/2004 - "Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan." 10/01/2003 - "Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan."
5.	AntigensIBA 12/30/2005 - "The results did not demonstrate any by-stander cellular response against myelin antigens, implicating that CNS is not a predominant target of an autoimmune response in SSPE. " 03/28/2000 - "Furthermore, SSPE VH sequences accumulated replacement mutations preferentially in the complementary-determining regions (CDRs) relative to framework regions-features normally observed during antigen-driven selection. " 05/01/1998 - "Measles virus antigen in macrophage/microglial cells and astrocytes of subacute sclerosing panencephalitis." 01/01/1998 - "To our knowledge, this is the first report of demonstration of measles virus nucleocapsides and antigen in the peripheral nerve in SSPE." 11/01/1997 - "Within heavily infected (MV-positive) brain lesions of all five SSPE cases, CD46 was either not detected or was expressed to a lesser degree by neural cells, irrespective of whether MV antigens were detectable or not. "
6.	AntibodiesIBA 05/01/2002 - "This study detected significant amounts of anti-CD9 antibodies in the cerebrospinal fluid (CSF) of all patients with SSPE included in the study. " 08/05/1967 - "Virus antibodies in subacute sclerosing panencephalitis: a study of 22 patients." 05/22/2007 - "Expanded plasma cell clones in the CSF of patients with subacute sclerosing panencephalitis produce disease-relevant antibodies. " 01/01/2007 - "Considering the potential role of humoral immunity in the pathogenesis of SSPE, one patient was given compassionate anti-CD20 antibodies. " 11/07/2005 - "These sera contained high titers of antibodies to MV. Thus, a small animal model is now available to both molecularly probe the pathogenesis of SSPE and to test a variety of therapies to treat the disease."
7.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 07/01/1991 - "TNF alpha production by peripheral blood monocytes was studied in seventeen patients with a recent exacerbation of MS, thirteen with remission of MS and fourteen patients with SSPE. " 12/15/2008 - "We suggest that CSF NF-H levels can be used as a marker of development of neuronal degeneration in SSPE, and that TNF-alpha modifies the neurodestructive pathogenesis in SSPE." 07/01/1991 - "In SSPE spontaneous and stimulated TNF alpha release did not differ from that in the control group. " 06/01/2005 - "We measured lymphocyte subsets and intracellular TNFalpha and interleukin-4 levels in peripheral blood in SSPE patients. " 06/01/2005 - "Lymphocyte subsets, TNF alpha and interleukin-4 levels in treated and untreated subacute sclerosing panencephalitis patients."
8.	Interferon-betaIBA 11/01/2004 - "The purpose of this study was to compare the efficacy of 2 different regimens of interferon-beta(IFN-beta) in the treatment of SSPE in pediatric patients. " 11/01/1998 - "These results suggest that beta-interferon might be efficient in some patients with subacute sclerosing panencephalitis and justify its trial in larger studies with longer follow-up." 11/01/2004 - "Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencephalitis." 11/01/1987 - "Changes were observed in the cerebrospinal fluid in SSPE cases during administration of beta-interferon 1 million units in 3.3 ml of normal saline intrathecally every second day for one month. " 11/01/1987 - "[Changes in the cerebrospinal fluid in patients with subacute sclerosing panencephalitis during intrathecal administration of interferon beta]."
9.	Carbon MonoxideIBA 04/01/2010 - "Since co-inhibitory molecules are involved in the suppression of T lymphocytes, we investigated whether single nucleotide polymorphisms (SNPs) of genes encoding co-inhibitory molecules contributed to a susceptibility to SSPE. " 08/01/1999 - "Transcriptional analysis showed that yfhP, yfhQ and yfhR are transcribed during the exponential growth phase, and sspE is monocistronically transcribed in the late sporulation phase and co-transcribed with yfhQ and/or yfhR during exponential growth. " 06/01/1975 - "A cytopathic agent causing formation of syncytial giant cells was isolated by co-cultivation of human embryonic lung cells with brain cells obtained at autopsy from a patient with subacute sclerosing panencephalitis. " 12/01/1974 - "A line of cells (IP-3), persistently infected with defective measles virus, was developed from co-cultures of subacute sclerosing panencephalitis-derived brain cells with monkey kidney cells (BSC-1). " 05/01/1974 - "Diagnosis of subacute sclerosing panencephalitis by serial tissue co-cultivation."
10.	Glucose (Dextrose)FDA LinkGeneric 01/01/1987 - "A marked decrease in glucose utilization was found in the cortical gray matter of a patient with rapidly progressive SSPE, despite relatively preserved utilization in the caudate and putamen. " 01/01/1987 - "The PET study of a patient with slowly progressive SSPE revealed patterns and values of glucose utilization similar to those of the control. " 03/01/1992 - "Changing patterns of glucose metabolism during the course of subacute sclerosing panencephalitis as measured with 18FDG-positron-emission tomography." 01/01/1989 - "Cerebral glucose metabolism in the course of subacute sclerosing panencephalitis." 06/01/1987 - "Regional cerebral metabolic rate for glucose and cerebrospinal fluid monoamine metabolites in subacute sclerosing panencephalitis."

Therapies and Procedures

1.	Radiotherapy 03/01/1982 - "In humans, beneficial results have been reported from clinical trials testing the effects of methisoprinol in a variety of diseases including subacute sclerosing panencephalitis (SSPE), acute viral encephalitis, recurrent mucocutaneous infections due to type I and II Herpes viruses as well as in immune restoration of cancer patients with immunodepression following radiotherapy. "
2.	Amputation 11/01/1995 - "Four patients had long-term sequelae (chronic lung disease, subacute sclerosing panencephalitis, hemiplegia, and partial amputation of a limb), and seven patients recovered uneventfully. "
3.	Aftercare (After-Treatment) 04/01/1986 - "Subacute sclerosing panencephalitis: remission after treatment with intraventricular interferon."
4.	Immunomodulation 07/01/1999 - "In this study we analyzed lymphocyte subsets in peripheral blood and concentrations of interleukin-1alpha (IL-1alpha), interleukin-2 (IL-2alpha), tumor necrosis factor-alpha (TNF-alpha), and platelet activating factor in plasma and cerebrospinal fluid before and after immunomodulatory therapy (interferon-alpha plus isoprinosine) in three patients with subacute sclerosing panencephalitis. "
5.	Drug Therapy (Chemotherapy) 04/01/2012 - "Recently, ribavirin therapy has been proposed as novel antiviral chemotherapy for SSPE. " 08/01/2007 - "Recently, ribavirin therapy has been proposed as novel antiviral chemotherapy for SSPE. " 01/01/1996 - "Effective ribavirin concentration in hamster brains for antiviral chemotherapy for subacute sclerosing panencephalitis."