Subacute sclerosing panencephalitis in only one of identical twins. A seven-year follow-up
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Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurological disease. This study followed identical twins, one developing SSPE after measles and a head injury, while the other remained healthy.
Area of Science:
- Neurology
- Virology
- Immunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological complication of measles virus infection.
- Identical twins provide a unique model to study genetic versus environmental factors in disease development.
Observation:
- Primary measles infection occurred simultaneously in both twins at age 4.
- The twin who developed SSPE experienced a grade 1 closed head injury within six months of measles infection.
- SSPE diagnosis at age 13 followed personality changes and myoclonic seizures, with elevated measles antibodies in serum and cerebrospinal fluid (CSF).
Findings:
- The affected twin showed rapid neurological deterioration after five years in Stage 2, with persistently elevated measles antibodies and oligoclonal IgG in serum and CSF.
- The unaffected twin exhibited normal neurological, virological, and immunological test results.
- Despite the head injury in the affected twin, no other significant differentiating factors were identified between the twins that would easily explain the SSPE development.
Implications:
- This case highlights the potential role of head injury as a cofactor in SSPE development, even in genetically identical individuals.
- Further research is needed to elucidate the precise mechanisms and risk factors contributing to SSPE pathogenesis.
- Understanding these factors is crucial for developing preventative strategies and treatments for SSPE.
Abstract:
We report a seven-year follow-up of identical twins, in one of whom subacute sclerosing panencephalitis (SSPE) developed. Primary measles infection occurred simultaneous in both twins at age 4. The affected twin sustained a grade 1 closed head injury within six months of her primary measles infection. At age 13, SSPE was diagnosed following the onset of personality change and myoclonic seizures. Measles antibody level was elevated in the serum and CSF. After remaining in stage 2 for five years, rapid mental and neurological deterioration occurred. Measles antibody level remained elevated, and oligoclonal IgG was present in both serum and CSF. Results of neurological examination as well as virological and immunological tests were normal in the unaffected twin. Besides the occurrence of head injury, factors known to be associated with SSPE were not obviously different in the twins. We have been unable to determine a difference that would easily explain the occurrence of SSPE in only one of two identical twins.