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[Changes in the fundus in subacute sclerosing panencephalitis. Apropos of 23 cases]
I Cochereau-Massin1, A Gaudric, P Reinert
1Service d'Ophtalmologie, Hôpital Pitié-Salpêtrière, Paris.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a fatal slow encephalitis. It is related to a chronic infection of the central nervous system by a defective measles virus. Among 41 patients with SSPE, 23 had ocular involvement. The ophthalmologic involvement preceded the onset of neurological signs in 4 cases, they were detected at first presentation in 9 cases, and they occurred in the course of the disease in 10 cases. The ocular involvement was bilateral in 19 cases. Various ophthalmologic features were reported: retinitis (16 eyes), macular pigment disturbances (4 eyes), optical neuritis (33 eyes), visual agnosia (1 patient). The macular lesions were typical of the disease, but at the acute stage they were liable to be confused with toxoplasmosis. This complication of measles should disappear with the systematic vaccination of 12 month old children.
Insights
Subacute sclerosing panencephalitis (SSPE), a fatal brain infection linked to measles, frequently affects vision. Early eye exams in SSPE patients can aid diagnosis and reveal the disease
Area of Science:
- Neurology
- Virology
- Ophthalmology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurological disease.
- It results from a chronic central nervous system infection by a defective measles virus.
Observation:
- Ocular involvement was observed in 23 out of 41 SSPE patients.
- Ophthalmologic symptoms preceded neurological signs in 4 cases and were present at initial diagnosis in 9 cases.
- Bilateral ocular involvement occurred in 19 patients.
Findings:
- Common ocular findings included retinitis (16 eyes), optic neuritis (33 eyes), and macular pigment disturbances (4 eyes).
- Macular lesions, typical of SSPE, can be mistaken for toxoplasmosis during the acute phase.
- Visual agnosia was noted in one patient.
Implications:
- Ophthalmologic evaluation is crucial for early SSPE diagnosis.
- Systematic measles vaccination in infancy is expected to eliminate SSPE.
- Understanding ocular manifestations aids in differentiating SSPE from other neurological conditions.