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Adult-Onset Subacute Sclerosing Panencephalitis With a 30-Year Latent Period
Brandon B Holmes1, Jessamyn Conell-Price1, Collin J Kreple1
1Department of Neurology, University of California, San Francisco, CA, USA.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a rare progressive neuroinfectious disease due to a late complication of the measles virus. The hallmark clinical features of this disease include behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The presence of characteristic high-amplitude periodic complexes on electroencephalography and raised antibody titers against measles in the cerebrospinal fluid help solidify the diagnosis. We present a case of a 40-year-old patient with SSPE who initially developed ophthalmologic manifestations 30 years after the primary measles infection. This case highlights both typical and atypical features of SSPE and provides a diagnostic framework for evaluating cases that fall outside of the standard scope of this disease.
Insights
Subacute sclerosing panencephalitis (SSPE), a rare measles virus complication, can present with delayed neurological and ophthalmologic symptoms. This case highlights atypical SSPE features, emphasizing a broader diagnostic approach for this progressive neuroinfectious disease.
Area of Science:
- Neurology
- Infectious Diseases
- Ophthalmology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neuroinfectious disease.
- It is a late complication of measles virus infection.
- Typical SSPE symptoms include behavioral changes, myoclonus, dementia, and neurological deficits.
Observation:
- A 40-year-old patient presented with SSPE.
- Ophthalmologic manifestations appeared 30 years after the initial measles infection.
- This presentation included both typical and atypical SSPE features.
Findings:
- Diagnosis was supported by electroencephalography showing periodic complexes and elevated cerebrospinal fluid measles antibodies.
- The case demonstrates a prolonged latency period and initial ophthalmologic presentation.
- This highlights the variability in SSPE clinical manifestation.
Implications:
- This case underscores the importance of considering SSPE in patients with neurological or ophthalmologic decline, even decades after measles.
- It suggests a need for a broader diagnostic framework for atypical SSPE presentations.
- Early and accurate diagnosis is crucial for managing this severe neurodegenerative condition.
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