PURPOSE: To describe a previously unreported clinical entity of progressive extensive @entity194 and pseudodrusen-like appearance in middle-aged @entity1 . DESIGN: Clinical, electrophysiologic, and molecular retrospective study. METHODS: The database of an outpatient clinic unit for genetic sensory diseases was screened for @entity1 older than 40 years with uncharacterized @entity1020 . @entity1 with extensive @entity194 and pseudodrusen-like appearance were included. RESULTS: Eighteen @entity1 of 45 records (40%) matched the inclusion criteria. Bilateral polycyclic well-delineated @entity5066 extending to the temporal vascular arcades, with a larger vertical @entity2270 and without sparing of the fovea featured the macular lesion. The pseudodrusen-like appearance was widespread throughout the posterior pole and the peripheral retina. In the extreme periphery, paving stone lesions were located mostly in the inferior quadrants. In contrast to age-related @entity1020 , a rapid progression of the @entity194 was observed with an early involvement of the foveal zone, thus leading to a severe @entity227 . All the @entity1 except 2 were legally blind at the end of the follow-up. Unlike age-related @entity1020 , in none of these @entity1 did @entity1940 develop. In all @entity1 , the scotopic and photopic electroretinography responses were reduced. CONCLUSIONS: Extensive @entity194 with pseudodrusen should be considered as a possible pattern of severe @entity1020 occurring in the middle-aged adult.

Extensive XXXX with pseudodrusen-like appearance: a new clinical entity.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity227 :: ('MESH:D014786', 'Disease') :: ['visual loss'] @entity5066 :: ('MESH:C566236', 'Disease') :: ['chorioretinal atrophy'] @entity1940 :: ('MESH:D020256', 'Disease') :: ['choroidal neovascularization'] @entity194 :: ('MESH:D001284', 'Disease') :: ['macular atrophy', 'atrophy'] @entity2270 :: ('MESH:C566610', 'Disease') :: ['axis'] @entity1020 :: ('MESH:D008268', 'Disease') :: ['macular dystrophy', 'macular degeneration']
@entity194 :: (MESH:D001284,Disease) :: ['macular atrophy']
