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An interesting case of subacute sclerosing panencephalitis presenting with Balint's syndrome and dysautonomia.
Encephalitis
March 2025
Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.
Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disorder caused by persistent aberrant measles virus infection. It is characterized by behavioral changes, cognitive decline with deterioration in academic performance, visual dysfunction, focal or generalized seizures ,myoclonus, spasticity, mutism and akinesia ultimately leading to a vegetative state. Balint's syndrome, characterized by the triad of simultagnosia, optic ataxia, and oculomotor apraxia, as an initial presenting feature of SSPE is rare.
View Article and Find Full Text PDFJaw-Opening Myoclonus: A Rare and Distinctive Sign in Subacute Sclerosing Panencephalitis.
Ann Indian Acad Neurol
March 2025
Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
Prolonged Survival in Subacute Sclerosing Panencephalitis: Case Series with a Review of Literature on "Long Survivors".
Ann Indian Acad Neurol
March 2025
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Subacute sclerosing panencephalitis (SSPE) is a progressive and usually fatal disorder, arising from persistent measles virus infection in the brain. Exceptionally, prolonged survival has been noted. We report four such patients and also review current literature on long survival in SSPE.
View Article and Find Full Text PDFTREM2 in Regulating Macrophage Inflammatory Responses and Disease Pathogenesis.
Crit Rev Immunol
February 2025
Triggering receptor expressed on myeloid cells 2 (TREM2) is a cell surface receptor belonging to the TREM family that is predominantly expressed on myeloid cells such as granulocytes, monocytes, osteocytes, macrophages, and microglia. Although much of the functionality of TREM2 is not well understood at the molecular level, it is well established that TREM2 plays a significant role in the regulation of a broad definition of macrophage inflammatory responses. Dysregulation of TREM2 has been implicated in a large number of diseases including Alzheimer's disease, Nasu-Hakola disease, bone-related diseases, and atherosclerosis.
View Article and Find Full Text PDFExploring the role of the IL-12/IFN-γ immune axis in subacute sclerosing panencephalitis.
J Neuroimmunol
March 2025
Department of Human Genetics, National Institute of Mental Health and Neurosciences, Bangalore, India. Electronic address:
Background: Subacute sclerosing panencephalitis (SSPE) is a rare but fatal neurological syndrome resulting from persistent measles virus infection in the central nervous system. Despite the availability of a measles vaccine, SSPE continues to pose a significant health burden, and immunogenetic risk factors remain unclear. We aimed to study the role of the interleukin 12 (IL-12)/interferon-gamma (IFN-γ) axis, known for it's significance in antiviral defense, in the development of SSPE.
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