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Atypical electroencephalographic pattern in a patient with subacute sclerosing panencephalitis
G B Sharp1, S M Laney, B F Westmoreland
1Section of Pediatric Neurology, Mayo Clinic, Rochester, MN 55905.
Abstract:
Subacute sclerosing panencephalitis (SSPE) has become rare since the widespread use of the measles vaccine. In this patient with a 5 month history of seizures and progressive dementia due to SSPE, the atypical electroencephalographic pattern was characterized by generalized spike-and-wave discharges, maximal on the right, associated with clinical seizures. After diazepam (intravenously), more typical periodic complexes appeared.
Insights
Subacute sclerosing panencephalitis (SSPE), a rare measles complication, presents with progressive dementia and seizures. Diazepam administration revealed typical periodic complexes on EEG, aiding diagnosis in this atypical case.
Area of Science:
- Neurology
- Virology
- Pediatrics
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological complication of measles virus infection.
- Widespread measles vaccination has significantly reduced SSPE incidence globally.
- SSPE typically manifests with cognitive decline, seizures, and characteristic EEG abnormalities.
Observation:
- A patient presented with a 5-month history of seizures and progressive dementia attributed to SSPE.
- The initial electroencephalogram (EEG) showed an atypical pattern of generalized spike-and-wave discharges, predominantly on the right side, coinciding with clinical seizures.
- Intravenous diazepam administration was administered to the patient.
Findings:
- Following diazepam administration, the EEG pattern evolved to display more typical periodic complexes.
- This EEG change suggests a potential diagnostic utility of pharmacologic challenge in atypical SSPE cases.
- The findings highlight the variability in EEG presentation of SSPE.
Implications:
- Recognizing atypical EEG patterns in SSPE is crucial for timely diagnosis and management.
- Pharmacologic interventions like diazepam may help unmask characteristic EEG findings in challenging SSPE cases.
- Continued surveillance and vaccination remain paramount in preventing SSPE.