SSPE

Subacute sclerosing panencephalitis (SSPE) is a rare but fatal late neurological complication of measles, caused by persistent measles virus (MeV) infection of the central nervous system. There are no drugs approved for the treatment of SSPE. Here, we followed the clinical progression of a 5-year-old SSPE patient after treatment with the nucleoside analog remdesivir, conducted a post-mortem evaluation of the patient's brain, and characterized the MeV detected in the brain. The quality of life of the patient transiently improved after the first two courses of remdesivir, but a third course had no further clinical effect, and the patient eventually succumbed to his condition...

No abstract text is available yet for this article.

BACKGROUND: In Japan, the incidence of subacute sclerosing panencephalitis (SSPE) has reduced; however, the medical conditions and factors associated with disease progression remain unclear. METHODS: A nationwide survey of SSPE was conducted using a questionnaire in 2022. We conducted a descriptive analysis of the patients with SSPE in 2022 and Cox proportional hazards analyses for disease progression. We compared the patients with SSPE with those in a 2007 survey...

It is increasingly appreciated that pathogens can spread as infectious units constituted by multiple, genetically diverse genomes, also called collective infectious units or genome collectives. However, genetic characterization of the spatial dynamics of collective infectious units in animal hosts is demanding, and it is rarely feasible in humans. Measles virus (MeV), whose spread in lymphatic tissues and airway epithelia relies on collective infectious units, can, in rare cases, cause subacute sclerosing panencephalitis (SSPE), a lethal human brain disease...

The amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) of the island of Guam and the Kii peninsula of Japan is a fatal neurodegenerative disease of unknown cause that is characterized by the presence of abundant filamentous tau inclusions in brains and spinal cords. Here, we used electron cryo-microscopy to determine the structures of tau filaments from the cerebral cortex of three cases of ALS/PDC from Guam and eight cases from Kii, as well as from the spinal cord of two of the Guam cases...

Dystonia has been described in a few cases with SSPE, but there are only very few reports with status dystonicus and none from South India. Here, we report a six-year-old child presenting with severe dystonic posturing of all four limbs and trunk for 10 days duration following a febrile illness and initially treated elsewhere as viral encephalitis. Scalp EEG showed periodic high-amplitude slow wave discharges. MRI brain showed T2/FLAIR hyperintensity in bilateral frontal, left parietal, and deep white matter, extending across the corpus collosum with diffuse cerebral atrophy...

Sequelae Renee Buchanan, Daniel J. Bonthius Seminars in Pediatric Neurology Volume 19, Issue 3, September 2012, Pages 107-114 Worldwide, measles remains one of the most deadly vaccine-preventable diseases. In the United States, enrollment in the public schools requires that each child receives 2 doses of measles-containing vaccine before entry, essentially eliminating this once endemic disease. Recent outbreaks of measles in the United States have been associated with importation of measles virus from other countries and subsequent transmission to intentionally undervaccinated children...

Subsegmental pulmonary embolism (SSPE) is increasingly diagnosed with the growing use and technological advancements of multidetector computed tomography pulmonary angiography. Its diagnosis is challenging, and some presumed SSPE may actually represent imaging artifacts. Indirect evidence and results from small observational studies suggest that SSPE may be more benign than more proximal pulmonary embolism, and may thus not always require treatment. Therefore, guidelines suggest to consider a management strategy without anticoagulation in selected patients with SSPE at low risk of recurrent venous thromboembolism (VTE), in whom proximal deep vein thrombosis is excluded...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

The persistence of measles virus infection in childhood and early adolescence can rarely lead to a fatal progressive neurodegenerative disorder known as subacute sclerosing panencephalitis (SSPE), characterized by behavioral disturbances and intellectual disability followed by myoclonic jerks and occasional negative myoclonus. Movement disorders are rarely presenting manifestations in SSPE. We herein report a 63-year-old woman with generalized choreoathetosis as the presenting manifestation of stage-I SSPE...

No abstract text is available yet for this article.

INTRODUCTION: Sub-acute sclerosing panencephalitis (SSPE) is a chronic, progressive neurodegenerative disorder, commonly seen in measles-endemic countries leading to progressive neuronal loss and death. Currently, there is no proven cure for this devastating disease. We started a low glycemic index therapy (LGIT) in children with SSPE using the same principle as per its role in intractable epilepsy. METHODOLOGY: Low glycemic index diet was started in children with a confirmed diagnosis of SSPE based on Dyken's criteria...

Subacute sclerosing panencephalitis (SSPE) is a chronic slow progressive neurodegenerative disease that is often associated with measles complications. The disease is characterized by seizures, behavioral changes, motor deficit and eventually death. In this case report we discuss the case of an 8-year-old male who developed SSPE and was presented to our hospital with a history of generalized tonic colonic convulsion followed by gait abnormality, episodes, abnormal behaviors, and cognitive regression. On clinical exploration, the child had a history of measles at 8 months of age and meningitis at 18 months...

ETHNOPHARMACOLOGICAL RELEVANCE: In the snake-infested mountainous regions of China, Saxifraga stolonifera [L.] Meeb is widely utilized as an immediate remedy for venomous snake bites. However, the scientific understanding of S. stolonifera's efficacy in snakebite treatment remains limited and requires further investigation. AIM OF THE STUDY: The aim of this study was to assess the inhibitory effects of Saxifraga stolonifera phenolic extracts (SSPE) on Deinagkistrodon acutus venom (DAV) and explore the potential of S...

Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease caused by measles virus (MV), which typically develops 7 to 10 years after acute measles. During the incubation period, MV establishes a persistent infection in the brain and accumulates mutations that generate neuropathogenic SSPE virus. The neuropathogenicity is closely associated with enhanced propagation mediated by cell-to-cell fusion in the brain, which is principally regulated by hyperfusogenic mutations of the viral F protein...

We report an interesting case of visual loss and visual hallucinations in a 37-year-old man. He presented with decreased vision in both eyes and visual hallucinations for the last one and a half months. He also had multiple focal to bilateral tonic-clonic seizures. On examination, there was no perception of light rays in both eyes. Fundus examination revealed disc oedema with peripapillary small haemorrhages in both eyes. Initially, the discs were hyperaemic, which turned pale in the subsequent examination at 1 month...

Isolated-subsegmental-pulmonary-embolism (SSPE) is increasingly diagnosed with the use of computed-tomography-pulmonary-angiogram (CTPA). There remains clinical equipoise for management of SSPE with previous studies not accounting for frailty while determining clinical outcomes. Clinical outcomes among patients with isolated SSPE were compared with those with a more proximal PE after accounting for frailty and other risk-factors. This study included all patients with a positive CTPA for pulmonary embolism (PE) admitted between 2017 and 2021 to two Australian-tertiary-hospitals...

DndABCDE-catalyzed DNA phosphorothioation (PT), in which the nonbridging oxygen is swapped with a sulfur atom, was first identified in the bacterial genome. Usually, this modification gene cluster is paired with a restriction module consisting of DndF, DndG, and DndH. Although the mechanisms for the antiphage activity conferred by this Dnd-related restriction and modification (R-M) system have been well characterized, several features remain unclear, including the antiphage spectrum and potential interference with DNA methylation...

No abstract text is available yet for this article.