Document Detail

Familial cerebro-macular degeneration (the Stengel-Batten-Mayou-Spielmeyer-Vogt-Stock disease). Evaluation of the photoreceptors.
MedLine Citation:
PMID:  474085     Owner:  NLM     Status:  MEDLINE    
In a material comprising seven patients with familial cerebro-macular degeneration a grave visual disability had developed within a few years after the onset of the disease, this being at 3 1/2 to 7 years of age. The night vision was only moderately reduced. A severe red-green colour vision defect was demonstrated in three patients. This is in accord with the loss of red and green cone responses found in one patient by chromatic adaptation studies. On the other hand, a remarkably good response of the blue cone system was registered. Normal pulse amplitudes were found by dynamic tonometry in four patients indicating good choroidal circulation. This supports the theory that the degeneration of the neuroepithelium is of primary type. The selectivity in loss of response functions being demonstrated here, might, however, also be related to parallel degenerations taking place in ganglion cells.
E Hansen
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta ophthalmologica     Volume:  57     ISSN:  0001-639X     ISO Abbreviation:  Acta Ophthalmol (Copenh)     Publication Date:  1979 Jun 
Date Detail:
Created Date:  1979-10-26     Completed Date:  1979-10-26     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0370347     Medline TA:  Acta Ophthalmol (Copenh)     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  382-96     Citation Subset:  IM    
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MeSH Terms
Color Perception / physiology
Dark Adaptation
Fluorescein Angiography
Lipidoses / diagnosis,  physiopathology*
Photoreceptor Cells / physiopathology*
Tonometry, Ocular

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