Subacute sclerosing panencephalitis (SSPE) is a fatal disorder that occurs as a rare complication of childhood measles. Symptoms typically manifest between the ages of 5 and 15. While the incidence of SSPE is declining globally, it is still prevalent in regions where measles remains common and vaccination rates are low due to poverty and lack of health education. Diagnosing SSPE can be challenging, particularly when patients exhibit unusual symptoms. A thorough clinical evaluation, including vaccination history, physical examination, electroencephalogram (EEG) and Cerebrospinal fluid (CSF) analysis, can help in making a diagnosis. We present the case of a young woman in her early 20s who initially experienced depressive symptoms, followed by myoclonus, dementia and visual impairment. The patient was ultimately diagnosed with SSPE based on characteristic EEG findings, neuroimaging results, CSF analysis and elevated serum measles antibody levels.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11085698PMC
http://dx.doi.org/10.1136/bcr-2023-259111DOI Listing

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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.

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Article Synopsis
  • SSPE is a rare, progressive disorder caused by a lingering mutant measles virus in the brain, with unclear treatment strategies among neurologists.
  • A survey of 298 Indian neurologists revealed insights into their management practices, showing varied preferences for therapies such as Isoprinosine and valproate.
  • There is a significant gap in follow-up care for SSPE patients and an urgent need for clearer treatment guidelines to improve patient outcomes.
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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.

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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.

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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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