• Title/Summary/Keyword: Subacute sclerosing panencephalitis

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A long-term subacute sclerosing panencephalitis survivor treated with intraventricular interferon-alpha for 13 years

  • Kwak, Minsun;Yeh, Hye-Ryun;Yum, Mi-Sun;Kim, Hyun-Jin;You, Su Jeong;Ko, Tae-Sung
    • Clinical and Experimental Pediatrics
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    • v.62 no.3
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    • pp.108-112
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    • 2019
  • Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal central nervous system disorder resulting from persistent measles virus infection. Long-term data are scarce, with a maximum follow-up period of 10 years. Interferon-alpha ($IFN-{\alpha}$) is a protein that exerts its antiviral activity via enhancement of cellular immune response and is reported to be an effective drug for the treatment of SSPE. However, there is currently no consensus regarding the optimal duration of $IFN-{\alpha}$ therapy. Here, we present a case report of a patient with SSPE treated with long-term intraventricular $IFN-{\alpha}$ therapy, which facilitated clinical improvement and neurological stabilization without causing serious adverse effects. To the best of our knowledge, this is one of the longest follow-up studies investigating a patient with SSPE receiving intraventricular $IFN-{\alpha}$ treatment. Further studies are necessary to validate the benefits and safety of long-term intraventricular $IFN-{\alpha}$ treatment in patients with SSPE.

Measles Viral Infection in PD-1 Gene Knockout Mice (PD-1 유전자 제거 마우스에서 홍역바이러스 감염)

  • Chun, Jin Kyong;Kim, Kyu Yeun;Hur, Ji Ae;Kang, Dong Won;Kim, Ki Hwan;Kim, Dong Soo
    • Pediatric Infection and Vaccine
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    • v.20 no.3
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    • pp.123-130
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    • 2013
  • Purpose: Subacute sclerosing panencephalitis (SSPE) is a neurodegerative disease due to persistent measles virus infection. We investigated the role of programmed death-1 (PD-1) molecule which is related with chronic viral infection in developing SSPE in mouse. Methods: We adopt the $PD-1^{-/-}$, $PD-1^{-/+}$, and wild type BALB/c 3 week old mice to make an animal model of SSPE by injecting measles virus (SSPE strain) intraventricularly. Three months after infusion of virus, the mice were sacrificed and examined if the typical pathologic lesions had been progressed. The sera were collected from each group of mice and the serum level of IL-21 was measured with ELISA kit. Results: The necrotic lesions on white matter and gliosis were found in focal areas in wild type BALB/c. The extent of lesion was smaller in heterotype BALB/c. Scanty lesions were found in $PD-1^{-/-}$ mice. The sera level of IL-21 was not elevated in all three groups. Conclusion: Our data suggest that the PD-1 molecule may play a role in persistent viral infection.

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