Document Detail

Cone dystrophies with negative photopic electroretinogram.
MedLine Citation:
PMID:  8343466     Owner:  NLM     Status:  MEDLINE    
A scotopic electroretinogram with an a-wave amplitude larger than the b-wave amplitude traditionally is termed 'negative'. Six male patients with negative photopic electroretinograms were examined; three of them suffered from progressive cone dystrophy, in which negative electroretinograms are unusual. Another patient without symptoms was the brother of a patient with cone dystrophy. These patients are compared with others who characteristically have negative electroretinograms-one patient with incomplete congenital stationary night blindness and another with X linked congenital retinoschisis. Differential diagnosis between these unusual cases of cone dystrophies and X linked retinoschisis or congenital stationary night blindness was possible with funduscopy, adaptometry, and evaluation of progression, but not with the electroretinogram. Inner retinal defects may occur in cone dystrophies as indicated by the negative electroretinogram. The waveform variations between our patients may be due to different inner retinal defects. The findings in two brothers indicate that cone dystrophy and inner retinal defects may be inherited separately.
U Kellner; M H Foerster
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The British journal of ophthalmology     Volume:  77     ISSN:  0007-1161     ISO Abbreviation:  Br J Ophthalmol     Publication Date:  1993 Jul 
Date Detail:
Created Date:  1993-09-09     Completed Date:  1993-09-09     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0421041     Medline TA:  Br J Ophthalmol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  404-9     Citation Subset:  IM    
Freie Universität Berlin, Klinikum Steglitz, Berlin, Germany.
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MeSH Terms
Dark Adaptation
Linkage (Genetics)
Middle Aged
Night Blindness / physiopathology
Photoreceptor Cells / physiopathology*
Retinitis Pigmentosa / genetics,  physiopathology*
Retrospective Studies
Visual Acuity
X Chromosome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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