Electroretinograms in English setters with neuronal ceroid lipofuscinosis.
نویسندگان
چکیده
Full-field electroretinograms (ERGs) were recorded from three adult English setters with advanced neuronal ceroid lipofuscinosis and three normal controls. Affected setters showed 30% to 40% reductions in a-wave and b-wave amplitudes, normal cone and rod b-wave implicit times, and slightly elevated a-wave and b-wave thresholds. The ERGs of these affected setters differed from those that have been recorded from humans with neuromal ceroid lipofuscinosis (Batten's disease), humans with hereditary retinitis pigmentosa, Irish setters with rod-cone dysplasia, and miniature French poodles with progressive rod-cone degeneration.
منابع مشابه
Studies on the retina and the pigment epithelium in hereditary canine ceroid lipofuscinosis. IV. Changes in the electroretinogram and the standing potential of the eye.
The present study correlates ultrastructural abnormalities in the retinal pigment epithelium (RPE) with electrophysiologic changes demonstrated in examinations of dogs with canine ceroid-lipofuscinosis (CCL) at various stages of clinical involvement using dc electroretinograms (ERG), including the c-wave, as well as recordings of the variations of the standing potential (SP) of the eye, both of...
متن کاملPrereceptor light absorption in setters with neuronal ceroid lipofuscinosis.
To the Editor: Berson and Watson recently described measures of the electroretinogram (ERG) in English setters with neuronal ceroid lipofuscinosis. They observed 30% to 40% reductions in rod and cone ERG amplitudes, despite the absence of gross morphologic abnormalities in the photoreceptors. The latency of the b-wave peak (i.e., implicit time) was normal. The authors raised three possible hypo...
متن کاملStudies on the retina and the pigment epithelium in hereditary canine ceroid lipofuscinosis. III. Morphologic abnormalities in retinal neurons and retinal pigmented epithelial cells.
Studies of the retina in 6- and 22-month-old English setters with progressive blindness, ataxia, and muscle weakness demonstrated a marked accumulation of abnormal cytosomes within neurons and retinal pigmented epithelial cells. Ganglion cells contained abundant cytosomes with evenly spaced stacks of membranes; bipolar and amacrine cell cytosomes consisted of dense, amorphous material with clos...
متن کاملWith Neuronal Ceroid Lipofuscinosis
An 11-year-old girl with juvenile neuronal ceroid lipofuscinosis developed acute pancreatitis secondary to valproic acid treatment, which resolved after discontinuation of the medication and conservative treatment. To my knowledge, this is the first reported case of neuronal ceroid lipofuscinosis with valproate-associated pancreatitis. Children with neuronal ceroid lipofuscinosis may be suscept...
متن کاملFeasibility of gene therapy for late neuronal ceroid lipofuscinosis.
Late infantile neuronal ceroid lipofuscinosis is a progressive childhood neurodegenerative disorder characterized by intracellular accumulation of autofluorescent material resembling lipofuscin in neuronal cells. This report summarizes the new therapies under consideration for late infantile neuronal ceroid lipofuscinosis, with a focus on strategies for in vivo gene therapy for the retinal and ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Investigative ophthalmology & visual science
دوره 19 1 شماره
صفحات -
تاریخ انتشار 1980