Case of the Month | June 2023

Case of the Month
June 26, 2023

The Case

The patient was a 38-year-old woman with longstanding poor vision in the right eye. She had an infection at two months of age, and she gradually lost vision in the right eye until the age of six. There have been no recent changes in vision. The visual acuity was light perception only in the right eye and 20/20 in the left eye. The anterior chamber and vitreous were quiet in each eye. The fundus photographs are attached. What was the most likely diagnosis? Is any prophylactic treatment needed at this time?

The patient had fibrosis over the disc and optic disc pallor is visible, and there is also multifocal chorioretinal scarring. There also appears to be persistent hyperplastic primary vitreous, which might be an incident finding. She said that she was told that she had measles at the age of two months. Measles encephalitis is called subacute sclerosing panencephalitis. SSPE often leads to severe neurological damage or death when it occurs in children, and it is less hazardous when it presents in adulthood. Our patient did not have evidence of significant neurological damage. SSPE can present as a single or multiple areas of retinal whitening that evolve into areas of atrophy and chorioretinal scarring. It is usually bilateral, typically occurs in immunocompetent patients, and is more common in males. Our patient’s condition had been inactive for more than three decades, and no intervention was needed.

Another diagnostic consideration is syphilis, which can present as a multifocal chorioretinitis. It is possible that she was treated for syphilis as an infant and was later told that she had a different condition. Toxoplasmosis can also be multifocal, but the number of discrete lesions in this patient is very atypical.

Green SH, Wirtschafter JD. Ophthalmoscopic findings in subacute sclerosis panencephalitis. Br J Ophthalmol. 1973; 57:780-787.

Lee JH, Agarwal A, Mahendradas P, et al. Viral posterior uveitis. Surv Ophthalmol. 2017; 62:404-445.

Shah A, Babu R, Biswas J. Retinitis as the presenting feature of subacute sclerosing panencephalitis in an Indian male: A case report. Indian J Ophthalmol. 2018; 66:1491-1493.

Case Photos

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The patient had fibrosis over the disc and optic disc pallor is visible, and there is also multifocal chorioretinal scarring. There also appears to be persistent hyperplastic primary vitreous, which might be an incident finding. She said that she was told that she had measles at the age of two months. Measles encephalitis is called subacute sclerosing panencephalitis. SSPE often leads to severe neurological damage or death when it occurs in children, and it is less hazardous when it presents in adulthood. Our patient did not have evidence of significant neurological damage. SSPE can present as a single or multiple areas of retinal whitening that evolve into areas of atrophy and chorioretinal scarring. It is usually bilateral, typically occurs in immunocompetent patients, and is more common in males. Our patient’s condition had been inactive for more than three decades, and no intervention was needed.

Another diagnostic consideration is syphilis, which can present as a multifocal chorioretinitis. It is possible that she was treated for syphilis as an infant and was later told that she had a different condition. Toxoplasmosis can also be multifocal, but the number of discrete lesions in this patient is very atypical.

Green SH, Wirtschafter JD. Ophthalmoscopic findings in subacute sclerosis panencephalitis. Br J Ophthalmol. 1973; 57:780-787.

Lee JH, Agarwal A, Mahendradas P, et al. Viral posterior uveitis. Surv Ophthalmol. 2017; 62:404-445.

Shah A, Babu R, Biswas J. Retinitis as the presenting feature of subacute sclerosing panencephalitis in an Indian male: A case report. Indian J Ophthalmol. 2018; 66:1491-1493.

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