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 micromol/L) and nitrate (median, 6.14 micromol/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 micromol/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 micromol/L) were higher than in those without myoclonus (median, 4.25 micromol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.
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http://dx.doi.org/10.1016/j.pediatrneurol.2009.04.016 | DOI Listing |
J Virol
December 2024
Mayo Clinic Graduate School of Biomedical Sciences, Mayo Clinic, Rochester, Minnesota, USA.
Subacute sclerosing panencephalitis (SSPE) is a lethal neurological disorder occurring several years after measles. Reconstruction of the evolution of the measles virus (MeV) genome in an SSPE case suggested that the matrix (M) protein mutation M-F50S, when added to other mutations, drove neuropathogenesis. However, whether and how M-F50S would promote spread independently from other mutations was in question.
View Article and Find Full Text PDFAnn Indian Acad Neurol
November 2024
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Tremor Other Hyperkinet Mov (N Y)
November 2024
Department of Neurology, King George's Medical University, Lucknow, India.
Background: Subacute Sclerosing Panencephalitis (SSPE) is a fatal disorder marked by gradual cognitive and motor deterioration, leading to death typically within 1-3 years.
Case Report: A 20-year-old woman with progressive abnormal behaviour, forgetfulness, and involuntary movements showed significant improvement after treatment with interferon and isoprinosine. Initially severely cognitively impaired and dependent, she regained independence and demonstrated marked cognitive enhancement, her MMSE improved from 15 to 28 and reduced myoclonus.
Indian J Pediatr
November 2024
Department of Pediatrics, Post Graduate Institute of Medical Education and Research (PGIMER) & Associated Dr. Ram Manohar Lohia (RML) Hospital, New Delhi, India.
Subacute sclerosing panencephalitis (SSPE) is a devastating fatal condition caused by mutated measles virus. It predominantly affects children of younger age and invariably leads to mortality. Though reported rarely, the disease continues to cause significant morbidity in children.
View Article and Find Full Text PDFJ Virol
November 2024
Department of Molecular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
During virus replication in cultured cells, copy-back defective viral genomes (cbDVGs) can arise. CbDVGs are powerful inducers of innate immune responses , but their occurrence and impact on natural infections of human hosts remain poorly defined. We asked whether cbDVGs were generated in the brain of a patient who succumbed to subacute sclerosing panencephalitis (SSPE) about 20 years after acute measles virus (MeV) infection.
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