Subacute sclerosing panencephalitis: fundus changes and histopathologic correlations

Abstract

This paper describes the fundus changes in 2 patients with subacute sclerosing panencephalitis (SSPE). In both cases the ocular involvement preceded the neurological symptoms by several months. In one patient a localized serous detachment in the macular region was associated with retinal infiltrates and in the second patient an atypical unilateral macular chorioretinitis was first diagnosed. The diagnosis of SSPE was suspected when neurological signs appeared and it was confirmed by the typical EEG changes and especially by the high titers of measles antibodies in serum and CSF. The affected left eye of the second patient could be studied histopathologically and presented mainly pigment epithelial changes at the level of the scar, edema of the external plexiform layer, retinal folds and a detachment and rupture of the internal limiting membrane.

Influenza Retinitis: Association with Influenza Encephalitis

“…Retinitis caused by the measles virus, and which is seen as one of the ocular manifestations of subacute sclerosing panencephalitis (SSPE), sometimes exhibits similar angiography patterns to the one observed in our case. However, the ophthalmoscopic features are usually much more prominentthan what we found [4]. Life prognosis for patients with SSPE is extremely poor, so the severity of retinitis possibly depends on the damage to the central nervous system.…”

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.…”

“…The vitreous does not show inflammatory signs. 11 The retinal changes in SSPE are felt to be due to direct viral invasion since inclusion bodies have been seen in the neuronal cells of the retina and optic nerve. 12 Measles antigen has also been found in the infected retinal cells by immunofluorescence.…”

“…The lesions of SSPE chorioretinitis consist of edema, retinal folds, and sometimes superficial hemorrhages, in the macular region. 10,11 In the later stages, the lesion usually consists of patchy pigmentation surrounding an area of depigmentation. 10 Usually the lesion is solitary, but more widespread involvement is possible.…”

Unique Optical Coherence Tomography Findings in a Case of Macular Retinitis Caused by Subacute Sclerosing Panencephalitis

“…1,3,4 Histopathologically, the inner nuclear layer and the ganglion cell layer are generally more severely affected than other retinal layers in the acute stage. 5 The previous histopathologic case report also indicated an arcuate pattern of retinitis. 6 Consistently, our patient showed retinal nerve fiber layer defect.…”