The authors present the results of an epidemiological study of SSPE in Poland conducted since 15 years. Since the years covered by the study (1977-1991) included the first period after the introduction of obligatory vaccinations against measles in our country, the most important aim of this analysis was to find out the possible influence of the effect of these vaccinations on SSPE incidence. A comparison of the data obtained in successive stages of the study shows that a slow gradual decrease of the annual incidence of SSPE has been taking place in our country (from at least 1.6 per million before 1983 to 0.69 in 1991). The effect of the vaccinations is visible also in a significant increase of the mean age of SSPE onset which is a result of ever more widespread vaccination of the youngest children. These observations indicate unequivocally that the aetiological factor in SSPE is a wild strain of measles virus and not that used for vaccinations.
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Folia Neuropathol
August 2024
Mossakowski Medical Research Institute, Polish Academy of Sciences, Warsaw, Poland.
J Neuropathol Exp Neurol
March 2024
Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Subacute sclerosing panencephalitis (SSPE) is a fatal, slowly progressive brain disorder caused by a mutated measles virus. Both subacute inflammatory and neurodegenerative mechanisms appear to play significant roles in the pathogenesis. TAR DNA-binding protein 43 (TDP-43) inclusions are a common co-pathology in several neurodegenerative disorders with diverse pathogenesis.
View Article and Find Full Text PDFJ Alzheimers Dis
October 2022
Department of Neurogenetics and Functional Genomics, Mossakowski Medical Research Institute, Polish Academy of Sciences, Warsaw, Poland.
Background: Homozygous variants of the TREM2 and TYROBP genes have been shown to be causative for multiple bone cysts and neurodegeneration leading to progressive dementia (NHD, Nasu-Hakola disease).
Objective: To determine if biallelic variants of these genes and/or oligogenic inheritance could be responsible for a wider spectrum of neurodegenerative conditions.
Methods: We analyzed 52 genes associated with neurodegenerative disorders using targeted next generation sequencing in a selected group of 29 patients (n = 14 Alzheimer's disease, n = 8 frontotemporal dementia, n = 7 amyotrophic lateral sclerosis) carrying diverse already determined rare variants in exon 2 of TREM2.
Folia Neuropathol
August 2004
Department of Neuropathology, Institute of Psychiatry and Neurology, Warszawa, Poland.
Activated forms of microglia were ultrastructurally evaluated in three neurological diseases of different aetiology (subacute sclerosing panencephalitis--SSPE, Wilson's disease and Alzheimer's disease). The occurrence of activated rod, ramified and amoeboid microglia was found in the investigated diseases. The widest ultrastructural variability of microglia was in SSPE, including the presence of mitotic chromosomes or centrioles in its cytoplasm, which indicates microglia proliferation.
View Article and Find Full Text PDFFolia Neuropathol
March 2003
Department of Neuropathology, Institute of Psychiatry and Neurology, Warszawa, Poland.
The activation of microglial cells in pathological conditions is manifested primarily by their proliferation, as well as by the occurrence of a new morphological form--rod microglia. In the present study immunohistochemical identification of rod microglial phenotype against ramified microglia was performed on segments of 17 brains derived from 7 cases of encephalitis of viral aetiology (including 5 SSPE cases), 6 cases of Wilson's disease and 4 cases of Alzheimer's disease. Segments from frontal, temporal and occipital lobes, cerebellum and brainstem were subjected to histological, histochemical and immunohistochemical reactions.
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