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Atypical presentations of SSPE: a clinical study in four cases
Ercan Demir1, Ayşe Aksoy, Banu Anlar
1Department of Pediatric Neurology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal disease of the central nervous system caused by a persistent measles virus. It is clinically characterized by insidious onset of intellectual deterioration and behavioral changes followed by myoclonias and eventually complete neurologic deterioration. The diagnosis is based on characteristic clinical features, periodic electroencephalography (EEG) complexes of high slow waves and increased antibody titer against measles in cerebrospinal fluid. Here, we report four SSPE cases, two of whom manifested with hemiparesis; in the third and fourth cases, cerebellar ataxia and acute encephalopathy with focal seizures were the presenting symptoms at the onset of disease, respectively. The typical periodic EEG complexes in our patients led to the diagnosis of SSPE. Our findings show that SSPE should be considered in the differential diagnosis of hemiparesis, cerebellar ataxia and acute encephalopathy, and highlight the diagnostic significance of EEG in unidentified cases.
Insights
Subacute sclerosing panencephalitis (SSPE), a fatal brain disease from measles virus, can present with varied neurological symptoms. Electroencephalography (EEG) is crucial for diagnosing SSPE, even with atypical initial presentations.
Area of Science:
- Neurology
- Virology
- Neuroimmunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal neurological disorder.
- It results from a persistent infection by the measles virus in the central nervous system.
- Clinical presentation typically involves intellectual decline, behavioral changes, myoclonus, and severe neurological deterioration.
Observation:
- Four cases of SSPE are presented, illustrating diverse initial clinical manifestations.
- Two patients presented with hemiparesis, while others exhibited cerebellar ataxia and acute encephalopathy with focal seizures.
- These varied presentations initially suggested other neurological conditions.
Findings:
- Despite atypical initial symptoms, characteristic periodic electroencephalography (EEG) complexes were identified in all patients.
- These EEG findings were pivotal in confirming the diagnosis of SSPE.
- Elevated measles antibody titers in cerebrospinal fluid further supported the diagnosis.
Implications:
- SSPE should be included in the differential diagnosis for patients presenting with hemiparesis, cerebellar ataxia, or acute encephalopathy.
- The study underscores the diagnostic importance of electroencephalography (EEG) in identifying SSPE, especially in cases with unusual initial symptoms.
- Early and accurate diagnosis through EEG can aid in timely management and further research into SSPE pathogenesis.
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