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Subacute sclerosing panencephalitis in an infant: diagnostic role of viral genome analysis
T Z Baram1, I Gonzalez-Gomez, Z D Xie
1Department of Neurology, University of Southern California, Los Angeles.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is related to "defective" measles virus or vaccination, though an association with parainfluenza viruses has been reported. SSPE is characterized by a slow, erratic course and elevated cerebrospinal fluid measles titers. An immunocompetent, vaccinated infant, with onset of symptoms in parainfluenza virus season and a catastrophic course is described. Cerebrospinal fluid titers were negative, but postmortem brain had typical SSPE lesions. Patient brain-derived RNA, subjected to reverse transcription followed by polymerase chain reaction yielded polymerase chain reaction products with measles virus but not parainfluenza virus genes. The sequenced fragment revealed multiple mutations, typical for SSPE. SSPE can thus present in infants, with short latency and no cerebrospinal fluid antibodies. Viral genomic analysis may be diagnostic, permitting early therapy.
Insights
Subacute sclerosing panencephalitis (SSPE) can occur in infants with rapid progression and no detectable antibodies. Measles virus genomic analysis is crucial for early diagnosis and potential therapy.
Area of Science:
- Virology
- Neurology
- Immunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurological complication associated with measles virus infection.
- While typically linked to measles, associations with parainfluenza viruses have been reported, complicating diagnosis.
Observation:
- A case of SSPE in an immunocompetent, vaccinated infant is presented, with symptom onset during parainfluenza season.
- The infant experienced a rapid, severe disease course, and cerebrospinal fluid measles antibody titers were initially negative.
Findings:
- Postmortem examination revealed characteristic SSPE lesions in the brain.
- Reverse transcription-polymerase chain reaction analysis of brain-derived RNA confirmed the presence of measles virus, not parainfluenza virus, with mutations typical for SSPE.
Implications:
- SSPE can present in infants with a short latency period and absent cerebrospinal fluid antibodies.
- Viral genomic analysis, even without detectable antibodies, is a valuable diagnostic tool for early SSPE detection and intervention.