Subacute Sclerosing Panencephalitis

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Ayse Aksoy - One of the best experts on this subject based on the ideXlab platform.

Naomi Kondo - One of the best experts on this subject based on the ideXlab platform.

Pinar Altiaylik Ozer - One of the best experts on this subject based on the ideXlab platform.

Banu Anlar - One of the best experts on this subject based on the ideXlab platform.

  • Aprepitant in the Treatment of Subacute Sclerosing Panencephalitis: A Randomized, Double-Blind, Placebo-Controlled Study.
    Pediatric neurology, 2020
    Co-Authors: Ibrahim Oncel, Mesut Sancar, Bahadir Konuskan, Filiz Arioz, Songül Tezcan, Emine Arman-kandirmaz, Safak Parlak, Ekim Gumeler, Banu Anlar
    Abstract:

    Abstract Background Aprepitant is a neurokinin-1 receptor antagonist approved for the treatment of chemotherapy-induced nausea. We aimed to investigate the safety and efficacy of aprepitant in patients with Subacute Sclerosing Panencephalitis. Methods A randomized, double-blind, placebo-controlled study was conducted in patients with Subacute Sclerosing Panencephalitis assigned to receive two courses of aprepitant 250 mg/day orally or placebo for 15 days with an interval of two months between courses. Primary end points were safety and tolerability, and secondary end point was clinical improvement or stabilization assessed by Subacute Sclerosing Panencephalitis scoring system. Electroencephalography (EEG), brain magnetic resonance imaging, and cerebrospinal fluid measles-specific immunoglobulin G index were evaluated before and after treatment. Results Sixty-two patients with Subacute Sclerosing Panencephalitis were allocated to aprepitant (n = 31, median age 18 years) or placebo (n = 31, median age 22 years) group. Fifteen patients left the study within the first six months and 12 patients left between six and 12 months. Aprepitant was well tolerated and treatment-associated adverse events were similar to those described in the treatment of nausea. Clinical status at six and 12 months’ follow-up did not differ between aprepitant and placebo groups. Post-treatment EEG scores at 12 months were better in the aprepitant group (P = 0.015). Cerebral atrophy on magnetic resonance imaging increased in both groups, whereas measles-specific immunoglobulin G index decreased in the placebo group. Conclusions In this first clinical trial of aprepitant treatment in patients with Subacute Sclerosing Panencephalitis, the drug was safe and well tolerated. No clinical effect was observed. A modest improvement in EEG findings might justify trials for longer periods because EEG changes can precede clinical findings in Subacute Sclerosing Panencephalitis.

  • Cerebrospinal fluid nitric oxide levels in Subacute Sclerosing Panencephalitis.
    Pediatric neurology, 2009
    Co-Authors: Deniz Yilmaz, Nesrin Şenbil, Banu Anlar, Deniz Yüksel, Sude Eminzade, Kamer Kilinc, Y.k. Yavuz Gürer
    Abstract:

    Oxidative damage plays a role in neurodegenerative diseases. Levels of cerebrospinal fluid nitrite and nitrate levels (oxidation products that provide an indirect estimation of nitric oxide) were investigated in relation to clinical and laboratory features in Subacute Sclerosing Panencephalitis ( n = 47) and age-matched control ( n = 43) groups. Significantly decreased levels of nitrite (median, 4.91 μ mol/L) and nitrate (median, 6.14 μ mol/L) were found in the patients. Nitrite and nitrate levels did not correlate with clinical or laboratory findings, except for presence of myoclonus. Cerebrospinal fluid nitrite levels of Subacute Sclerosing Panencephalitis patients without myoclonic jerks were significantly higher than in those with myoclonus (median, 15.63 vs 4.34 μ mol/L, respectively). The higher levels of nitrite in these patients can be explained by short disease duration and early stages of disease. Nitrate levels in Subacute Sclerosing Panencephalitis patients with myoclonus (median, 9.26 μ mol/L) were higher than in those without myoclonus (median, 4.25 μ mol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.

  • Tissue inflammatory response in Subacute Sclerosing Panencephalitis (SSPE).
    Journal of child neurology, 2001
    Co-Authors: Banu Anlar, Sabiha Aysun, Figen Soylemezoglu, Gülşen Köse, Deniz Belen, Kalbiye Yalaz
    Abstract:

    The pattern of inflammatory infiltration was studied in the frontal brain biopsies of 28 cases with Subacute Sclerosing Panencephalitis (SSPE) by immunohistochemistry. Lymphocytic infiltration and gliosis were common pathologic findings. CD4+ T lymphocytes were often observed in perivascular areas and CD8+ lymphocytes in the parenchyma. B lymphocytes were located in large perivascular cuffs associated with longer and slower disease. Major histocompatibility complex antigens, interferon-γ, and tumor necrosis factor-α (TNF-α) were expressed in endothelial and glial cells. The inflammatory lesions in Subacute Sclerosing Panencephalitis consist of various cell subtypes and cytokines localized in particular areas of the brain tissue and show certain associations with clinical course. (J Child Neurol 2001;16:895-900).

  • Beta-interferon plus inosiplex in the treatment of Subacute Sclerosing Panencephalitis.
    Journal of child neurology, 1998
    Co-Authors: Banu Anlar, Kalbiye Yalaz, Gülşen Köse, Serap Saygi
    Abstract:

    We treated seven patients with Subacute Sclerosing Panencephalitis with beta-interferon and oral inosiplex for 2 to 15 months. Stabilization or improvement was observed in three patients. The effect of treatment was equivocal in two other patients who became stable. The disease continued its progression in the remaining two patients who died. Treatment shorter than 2 months was not effective. Changes in electroencephalograms (EEG), magnetic resonance images (MRI), or cerebrospinal fluid measles antibody levels did not have a close correlation with clinical course. 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.

  • Subacute Sclerosing Panencephalitis
    CNS Drugs, 1997
    Co-Authors: Banu Anlar
    Abstract:

    Subacute Sclerosing Panencephalitis (SSPE) is a chronic progressive, usually fatal disease of uncertain pathogenesis that is associated with the presence of mutant measles virus in the CNS. The diagnosis is based on clinical criteria and an elevated titre of measles antibodies in the CSF. Electroencephalography, imaging studies and measles antibody synthesis rate in the CSF provide supportive laboratory data. When CSF studies are negative, a brain biopsy is indicated to assess the presence of inclusion bodies, measles virus antigens or viral RNA. Among the many drugs and methods tried in the treatment of SSPE, the highest rate of stabilisation or improvement has been obtained with intraventricular interferon-α (interferon-alfa) and oral inosine pranobex. Further research, including multicentre clinical trials, is warranted to identify more efficient therapeutic regimens.

Robert M. Pascuzzi - One of the best experts on this subject based on the ideXlab platform.

  • Subacute Sclerosing Panencephalitis Manifesting as Viral Retinitis: Clinical and Histopathologic Findings
    American journal of ophthalmology, 1997
    Co-Authors: Donald W. Park, H. Culver Boldt, Stephen J. Massicotte, Effiong E. Akang, Karen L. Roos, Adriana Bodnar, John Pless, Bernardino Ghetti, Robert M. Pascuzzi
    Abstract:

    PURPOSE AND METHODS To describe the clinical and histopathologic features of a patient with viral retinitis secondary to Subacute Sclerosing Panencephalitis. Results The patient was a human immunodeficiency virus-negative intravenous drug abuser with an acute retinitis that later progressed to encephalitis despite aggressive treatment for possible viral, protozoal, bacterial, and rickettsial infections. The patient had many of the characteristic findings of Subacute Sclerosing Panencephalitis, including a history of measles in early childhood, myoclonus, periodic complexes on electroencephalographic testing, persistently elevated serum and cerebrospinal fluid antimeasles immunoglobulin G (IgG) titers, and a cerebrospinal fluid oligoclonal IgG gammopathy. Ultrastructural examination demonstrated numerous filamentous microtubular intranuclear viral inclusions in the nuclear layers of the retina consistent with the measles virus. This case is unusual in that our patient developed Subacute Sclerosing Panencephalitis later in life and because there was an 8-year period between presumed viral infections in the two eyes. Conclusions An acute retinitis in an intravenous drug abuser is not always caused by human immunodeficiency virus-related infections; not all viral retinitis responds to therapy; and mortality as well as the usual morbidity may be associated with viral retinitis. One might consider the diagnosis of Subacute Sclerosing Panencephalitis in a young person with an acute retinitis with little or no vitreal inflammation and lack of response to anti-cytomegalovirus and antitoxoplasmosis therapy.