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Adult-onset subacute sclerosing panencephalitis
Sruthi S Nair1, K V Vysakha1, Ramshekhar N Menon1
1Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, India.
Practical Neurology
|April 14, 2021
Summary
Subacute sclerosing panencephalitis (SSPE), a rare adult-onset neurological disease, can mimic rapidly progressive dementia. Early diagnosis requires considering measles virus antibodies, especially in regions with lower vaccination rates.
Area of Science:
- Neurology
- Virology
- Neuroimmunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a fatal neurological disease caused by a defective measles virus, typically affecting children.
- Adult-onset SSPE is uncommon and can manifest as rapidly progressive dementia, complicating diagnosis.
Purpose of the Study:
- To report a case of adult-onset SSPE presenting as rapidly progressive dementia in a young man of Indian origin.
- To highlight diagnostic challenges and key indicators for SSPE in adults.
Main Methods:
- Case presentation of a young adult with dementia.
- Magnetic resonance imaging (MRI) of the brain revealing parieto-occipital white matter changes.
- Cerebrospinal fluid (CSF) analysis for high titres of anti-measles antibodies.
Main Results:
- The patient presented with rapidly progressive dementia without focal neurological signs.
- MRI showed characteristic white matter abnormalities.
- Elevated anti-measles antibody titres in CSF confirmed SSPE diagnosis.
Conclusions:
- Adult-onset SSPE is a rare but critical differential diagnosis for rapidly progressive dementia, particularly in adults from areas with suboptimal measles vaccination.
- The long latency period between measles infection and SSPE onset can be misleading.
- Prompt diagnosis relies on recognizing subtle neurological signs and specific serological markers.
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