Acute fulminant SSPE: clinical and EEG features

Area of Science:

• Neurology
• Pathology
• Virology

Background:

• Subacute Sclerosing Panencephalitis (SSPE) is a rare, chronic, post-measles encephalitis.
• SSPE typically presents with a slow, progressive decline, but rapid forms exist.
• Early diagnosis and understanding of SSPE's varied clinical course are crucial.

Observation:

• A patient presented with visual disturbances, progressing to right homonymous hemianopia and myoclonic jerks.
• Electroencephalogram (EEG) revealed periodic slow-wave discharges associated with jerks.
• Measles antibodies were elevated in serum and cerebrospinal fluid (CSF); MRI showed occipital white matter hyperintensities.

Findings:

• The patient rapidly deteriorated, experiencing blindness, generalized myoclonic jerks, and progressing to deep coma.
• EEG changes evolved to diffuse slow activity without periodic complexes as the condition worsened.
• Autopsy confirmed severe neuronal loss and intranuclear inclusion bodies characteristic of SSPE.

Implications:

• This autopsy-proven case underscores the potential for rapidly progressive SSPE, leading to death within months.
• The findings emphasize the importance of considering SSPE in cases of unexplained neurological decline, even with atypical presentations.
• Understanding the pathological hallmarks of SSPE is vital for accurate diagnosis and potential therapeutic strategies.