Subacute sclerosing panencephalitis and chorioretinitis

This is a case report of a 10-year-old boy with subacute sclerosing panencephalitis (SSPE). He initially developed visual disturbance and macular degenerative changes of the right eye at the age of 8 years, followed by chorioretinitis of the left eye, and his neurological symptoms deteriorated rapidly from the age of 10 years. He was diagnosed as having SSPE, as judged on cerebrospinal fluid examination for measles virus RNA by reverse transcription-polymerase chain reaction (RT-PCR), at the second stage of Jabbourʹs classification on admission. Although high intensity lesions were observed in the right occipital and temporal lobes, especially around the optic radiation, on T2-weighted brain MRI before the start of intrathecal interferon-α (IFN-α) therapy, they had disappeared at about two months after the treatment. Chorioretinitis (and/or macular degeneration) should be considered in the differential diagnosis of SSPE, permitting early IFN therapy.