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Published on: August 21, 2017
Subacute sclerosing panencephalitis presenting as neuromyelitis optica
Tushar Premraj Raut1, Maneesh Kumar Singh, Ravindra Kumar Garg
1Department of Neurology, CSMMU Lucknow, Lucknow, Uttar Pradesh, India.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a slowly progressing inflammatory and degenerative disorder of the brain caused by a mutant measles virus. The diagnosis of SSPE is based on characteristic clinical and EEG findings (periodic complexes) and demonstration of elevated antibody titres against measles in cerebrospinal fluid. SSPE can have atypical clinical features at the onset. The authors here report a case of a 3-year-old child who presented with vision loss followed 15 months later by quadriparesis with bladder involvement. These clinical features resembled that of neuromyelitis optica. However, as the disease progressed, appearance of myoclonic jerks, periodic discharges on EEG and positive cerebrospinal fluid serology for measles led to the final diagnosis of SSPE.
Insights
Subacute sclerosing panencephalitis (SSPE) is a rare brain disorder caused by measles virus. This case highlights atypical early symptoms mimicking other neurological conditions, emphasizing diagnostic challenges.
Area of Science:
- Neurology
- Virology
- Pediatrics
Background:
- Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurological disorder resulting from a persistent measles virus infection.
- Diagnosis typically relies on characteristic electroencephalogram (EEG) findings, elevated measles antibodies in cerebrospinal fluid (CSF), and clinical presentation.
Observation:
- A 3-year-old child presented with initial symptoms of vision loss, followed by quadriparesis and bladder dysfunction.
- These early clinical manifestations mimicked neuromyelitis optica, posing a diagnostic challenge.
Findings:
- The patient later developed myoclonic jerks and periodic discharges on EEG.
- CSF analysis confirmed elevated measles antibodies, leading to the definitive diagnosis of SSPE.
Implications:
- This case underscores the importance of considering SSPE in children with atypical neurological presentations, even when initial symptoms suggest other diseases.
- Early and accurate diagnosis of SSPE is crucial for potential management strategies and understanding disease progression.
- Recognizing diverse clinical presentations of SSPE aids in differentiating it from conditions like neuromyelitis optica.
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