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A 35-year-old man presents with visual problems. He has had very poor vision in the dark for a long time but is now worried as he is developing 'tunnel vision'. He states his grandfather had a similar problem and was registered blind in his 50's. What is the most likely diagnosis?
A. Leber's congenital amaurosis
B. Vitelliform macular dystrophy
C. Central serous retinopathy
D. Primary open angle glaucoma
E. Retinitis pigmentosa
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sorry,,other choices postgraduate or usmle step(10) ,,, best regards
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Retinitis pigmentosa (RP) is a group of genetic eye conditions that leads to incurable blindness. In the progression of symptoms for RP, night blindness generally precedes tunnel vision by years or even decades. Many people with RP do not become legally blind until their 40s or 50s and retain some sight all their lives. Others go completely blind from RP, in some cases as early as childhood. Progression of RP is different in each case.
RP is a type of progressive retinal dystrophy, a group of inherited disorders in which abnormalities of the photoreceptors (rods and cones) or the retinal pigment epithelium (RPE) of the retina lead to progressive visual loss. Affected individuals first experience defective dark adaptation or nyctalopia (night blindness), followed by reduction of the peripheral visual field (known as tunnel vision) and, sometimes, loss of central vision late in the course of the disease.
The diagnosis of retinitis pigmentosa relies upon documentation of progressive loss in photoreceptor cell function by electroretinography (ERG) and visual field testing.
