Unilateral retinitis pigmentosa (URP) is a rare tapetoretinal dystrophy affecting only one eye. Criteria are necessary to make a correct diagnosis of URP: exclude all infective etiologies, check that the clinical signs of retinitis are present in the affected eye and the total absence of any signs or symptoms of retinitis pigmentosa in the fellow eye. Electroretinogram (ERG) and electrooculogram (EOG) are useful for the correct diagnosis. In this paper we report one case.
RESUMORetinose pigmentar unilateral é uma distroia tapetoretiniana rara afetando somente um olho. Critérios devem ser empregados para um diagnostico correto: exclusão de todas etiologias infecciosas que cursam com quadro semelhante, presença dos sinais clínicos de retinose pigmentar no olho afetado e ausência de qualquer sinal no olho contralateral. Eletroretinograma (ERG) e eletrooculograma(EOG) são bastante úteis no diagnostico. Neste artigo relatamos o caso de uma paciente.
Palavras-chave:Retina. Células fotoreceptoras de vertebrados. Retinite pigmentosa. Visão ocular. Mutação.
Susac's syndrome is a rare disease attribuited to a microangiopathy involving the arterioles of the cochlea, retina and brain. Encefalopathy, hearing loss, and visual deficits are the hallmarks of the disease. Visual loss is due to multiple, recurrent branch arterial retinal occlusions. We report a case of a 20-year-old women with Susac syndrome presented with peripheral vestibular syndrome, hearing loss, ataxia, vertigo, and vision loss due occlusion of the retinal branch artery.
Purpose To determine the value of multifocal ERG (mfERG) and optical coherence tomography (OCT) in the evaluation of patients with unexplained visual loss.
Methods mfERG and macular OCT were obtained from six patients with unexplained visual loss (normal ophthalmoscopy, fluorescein angiography, full‐field ERG and brain CT scan) and four age and sex matched controls.
Results Patients BCVA ranged from 20/32 to 20/125. When comparing to controls, patients showed significantly depressed mfERG amplitudes in rings R1 (121.7 vs 64.8; p 0.01) and R2 (42.6 vs 19.5; p 0.02). Foveal thickness, as measured by macular OCT, was also reduced, although not significantly (p 0.1).
Conclusion Multifocal ERG allowed localization of the visual dysfunction to the central macula, in cases with hitherto unexplained visual loss.
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