Subacute Sclerosing Panencephalitis (SSPE)

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; 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; Encephalitis, Inclusion Body, Measles; Leukoencephalitis, Subacute Sclerosing; Measles Inclusion Body Encephalitis; Panencephalitis, Subacute Sclerosing; Van Bogaert's Leukoencephalitis

Networked: 956 relevant articles (42 outcomes, 101 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Subacute Sclerosing Panencephalitis (SSPE)
2.	Measles
3.	Infections
4.	Venous Thromboembolism
5.	Encephalomyelitis (Myeloencephalitis)

Experts

1.	Anlar, Banu: 20 articles (01/2022 - 04/2002)
2.	Hosoya, Mitsuaki: 14 articles (09/2022 - 07/2003)
3.	Ichiyama, Takashi: 9 articles (01/2020 - 01/2006)
4.	Hashimoto, Koichi: 7 articles (09/2022 - 04/2013)
5.	Kusuhara, Koichi: 7 articles (01/2021 - 05/2002)
6.	Cokar, Ozlem: 6 articles (01/2021 - 10/2005)
7.	Demirbilek, Veysi: 6 articles (01/2021 - 10/2005)
8.	Kira, Ryutaro: 6 articles (01/2021 - 05/2002)
9.	Saruhan-Direskeneli, Güher: 6 articles (01/2021 - 10/2005)
10.	Yapici, Zuhal: 6 articles (01/2021 - 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. " 01/01/1998 - "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." 12/01/2011 - "The structure of SSPE virus in the brains of SSPE patients is different from that of MV. The difference in interferon (IFN) production between cells infected with SSPE virus and those infected with MV remains unclear. " 12/01/2011 - "Interferon production by cells infected with subacute sclerosing panencephalitis (SSPE) virus or measles virus."
3.	Interferon-alpha (Interferon Alfa)IBA 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. " 03/01/2019 - "A long-term subacute sclerosing panencephalitis survivor treated with intraventricular interferon-alpha for 13 years." 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."
4.	Ribavirin (Virazole)FDA LinkGeneric 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 - "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. " 03/01/2006 - "We present the cases of two patients with an early-onset, fast progressing form of SSPE, who were treated with a combined regime of oral isoprenosin, intraventricular IFN-alpha and ribavirin, which was administered intravenously in one case and intraventricularly in the other. " 12/01/2004 - "Pharmacokinetics and effects of ribavirin following intraventricular administration for treatment of subacute sclerosing panencephalitis." 01/01/1998 - "Intraventricular administration of high dose IFN-alpha and ribavirin may have potential usefulness in the treatment of patients with SSPE."
5.	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." 01/01/2021 - "The typical periodic electroencephalographic (EEG) complexes, elevated cerebrospinal fluid (CSF), and serum antimeasles antibodies in our patients led to the diagnosis of SSPE. " 08/01/2020 - "To establish the index for SSPE diagnosis using EIA, we investigated the correlation between HI and EIA titers of MV antibodies in SSPE patients. " 05/22/2007 - "Expanded plasma cell clones in the CSF of patients with subacute sclerosing panencephalitis produce disease-relevant antibodies. "
6.	AntigensIBA 04/01/1996 - "Furthermore, the paucity of neurons co-expressing MHC class I and MV antigens in SSPE and SME suggests that such cells are either rapidly cleared by cytotoxic T lymphocytes (CTL), or, alternatively, lack of co-expression of MHC class I on MV infected neurons favors MV persistence in these cells by escaping CTL recognition." 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."
7.	RNA (Ribonucleic Acid)IBA 11/15/2005 - "Brain tissue samples obtained from 11 patients with a presumptive diagnosis of SSPE were tested for the presence of measles virus RNA. " 05/01/2004 - "These differences were not evident when the same primer sets were evaluated with RNA templates extracted from a brain sample of SSPE patient. " 07/01/1997 - "No hybridization was found with any of the probes in areas of SSPE brain, which were negative for measles virus RNA or in the non-neurological control cases, although expression was demonstrated in the latter by use of reverse transcription-polymerase chain reaction (RT-PCR). " 01/01/1997 - "He was diagnosed as having SSPE, as judged on cerebrospinal fluid examination for measles virus RNA by reverse transcription-polymerase chain reaction (RT-PCR), at the second stage of Jabbour's classification on admission. " 08/01/1994 - "Using a polymerase chain reaction-based amplification specific for copy-back DIs, abundant, discrete cDNAs representing different-sized MV defective RNA species were generated from each SSPE brain. "
8.	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."
9.	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 in the cerebrospinal fluid in patients with subacute sclerosing panencephalitis during intrathecal administration of interferon beta]." 11/01/1987 - "Seven patients with SSPE aged from 10 to 19 years received beta interferon intrathecally one million units every second day during one month (in all, 15 million units for one course of treatment. "
10.	Immunoglobulin G (IgG)IBA 04/01/1980 - "Samples of CSF from 48 patients with MS and 30 patients with SSPE were tested and, in both conditions, antibody activity was significantly greater than in controls, when tested at identical IgG concentrations. " 08/01/2020 - "Immunoglobulin G-EIA may be a suitable alternative method for SSPE diagnosis; however, its potential utility and the cut-off point of ≥0.49 IU/mL should be tested with additional patient cohorts." 08/01/2020 - "The serum/CSF MV antibody titer ratios determined by IgG-EIA were <20 in most SSPE patients. " 08/01/2020 - "In patients with SSPE, both serum and CSF antibody values, measured by IgG-EIA, and HI, were positively correlated, and a positive correlation was found between the serum and CSF MV antibody titers as measured by IgG-EIA. " 08/01/2020 - "In all cases diagnosed as SSPE, IgG-EIA titers in the CSF were ≥0.49 IU/mL. There was a positive correlation between serum antibody values in the controls measured by IgG-EIA and HI. "

Therapies and Procedures

1.	Therapeutics 07/01/2014 - "We consider that combination therapy in the very early stage without hypometabolism in the cerebral cortex may be effective for SSPE. " 04/01/2012 - "The therapeutic efficacy should be evaluated in the patients who are treated with the therapy at an early stage of SSPE." 08/01/2007 - "The therapeutic efficacy should be evaluated in the patients who are treated with the therapy at an early stage of SSPE." 01/01/2020 - "We included randomised controlled trials of anticoagulation therapy versus control in patients with SSPE or incidental SSPE. " 01/12/2016 - "Randomised controlled trials of anticoagulation therapy versus no intervention in patients with SSPE or incidental SSPE. "
2.	Ketogenic Diet 01/01/2021 - "Here we present a case of a 17-year-old patient affected by subacute sclerosing panencephalitis who manifest a dramatic improvement in neurological and general clinical conditions, as well as an arrest in the progression of demyelinating process in the central nervous system, after the beginning of a high ratio ketogenic diet. " 08/25/2019 - "Substantial Remission in Subacute Sclerosing Panencephalitis by Following the Ketogenic Diet: A Case Report." 01/01/2021 - "Clinical and magnetic resonance study of a case of subacute sclerosing panencephalitis treated with ketogenic diet." 04/01/2003 - "The use of the ketogenic diet in a patient with subacute sclerosing panencephalitis." 04/01/2003 - "The ketogenic diet may be useful in controlling, even temporarily, the myoclonic jerks of SSPE."
3.	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. "
4.	Duration of Therapy 04/01/1986 - "Alpha IFN has promise in the treatment of SSPE but the optimal dosage and duration of treatment are undetermined." 09/01/1997 - "The following aspects of the patients' history were recorded (a) current type of pancreatic enzyme medication; (b) total dosage per day (with reference to lipase units); (c) duration of therapy with standard-strength pancreatic enzyme (SSPE) preparations (< or = 10,000 lipase units per capsule) and HSPE preparations (> or = 20,000 lipase units per capsule); (d) gastrointestinal complication (distal intestinal obstruction syndrome, meconium ileus, abdominal surgery, intussusception), diabetes mellitus, and hepatobiliary complications. "
5.	Surgical Amputation (Amputations) 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. "