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

Abstract

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.

Subacute sclerosing panencephalitis

“…Visual manifestations may precede the neurological manifestations by a few weeks or months [35][36][37]. Visual complaints may be because of involvement of the retina, optic nerve or visual cortex.…”

Ocular Findings in Subacute Sclerosing Panencephalitis

“…[5][6][7][9][10][11] The visual symptoms preceded the onset of neurologic symptoms in only 1 patient in our study and in 4 patient in the other study. 9 In spite of the case reports indicating that ocular involvement precedes 7,11,14 the two large studies (Cochereau-Massin and this study) show that ocular involvement is usually seen shortly after the neurological symptoms appear.…”

“…Ophthalmic manifestations of SSPE have previously been reported in small series or in case reports. [5][6][7] The aim of this study was to evaluate prospectively the ocular manifestations of SSPE and to investigate possible risk factors in a relatively large number of patients.…”

Bilateral Macular Retinitis as the Presenting Feature of Subacute Sclerosing Panencephalitis

“…A multitude of neuro-ophthalmic and retinal findings are associated with SSPE, and ocular involvement appears to occur in at least 50% of cases (3)(4)(5)(6)(7)(8)(9). The most characteristic lesion is a necrotizing retinitis (13).…”

“…In India, the annual incidence rate is reported to be as high as 21 per million (1,2). Ocular manifestations of SSPE include optic neuritis (3), viral retinitis (4), chorioretinitis (5)(6)(7)(8), and cortical blindness (9,10). We report two patients with SSPE who initially presented with visual loss.…”