Macular Retinitis as a First Sign of Subacute Sclerosing Panencephalitis: The Importance of Early Diagnosis

Abstract

Purpose: Subacute sclerosing panencephalitis (SSPE) is a subacute inflammatory and neurodegenerative encephalitis related to the measles (rubeola) virus and usually affecting children and young adults. The overwhelming majority of cases follow a progressive downhill course leading to death, although there have been a few case reports of patients who have apparently gone into remission. Ocular changes occur in up to 50% of SSPE cases. Visual complaints, if present, generally antedate the onset of neurological symptoms by a few weeks or months. Here, we report two cases of SSPE presenting with ocular findings and their prognoses. Methods: Case reports. In the first case, a 17-year-old male presenting with macular retinitis, the macular findings were mistaken for a heredodegenerative disorder and diagnosis was postponed until neurological findings took place. He died six months after the appearance of his first ophthalmic symptoms despite intravenous immune globulin and isoprinosine therapy. The second case was a 14-year-old male, who presented with only ophthalmological complaints. His diagnosis was based on both ophthalmological findings and high doses of measles IgG in the cerebrospinal fluid (CSF); isoprinosine and intramuscular beta-interferon therapy was started before the onset of neurological findings and in the follow-up time of about 18 months, neurological findings consistent with SSPE did not develop. Results: The characteristic finding of macular retinitis in SSPE patients is rapid recovery in about one month without therapy. After improvement, neurological findings take place. Once suspected, the diagnosis of SSPE is easily established by the demonstration of high levels of measles antibody in the serum and CSF. Early diagnosis can be made with typical ocular findings and high IgG titers for rubeola in CSF. Conclusion: We suppose that ophthalmic manifestations, especially macular retinitis, may be useful in the diagnosis and management of SSPE cases with elevated IgG titers for rubeola in CSF. The typical clinical findings must be familiar to every ophthalmologist so that diagnostic pitfalls can be prevented and early therapy started. It may be discussed if early diagnosis and therapy will be possible before neurological signs appear, the prognosis of this relentless disease may show a more favorable course.

KEYWORDS:

• Macular retinitis
• subacute sclerosing panencephalitis