ERG rod a-wave in Oguchi disease

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ERG rod a-wave in Oguchi disease

We analyzed the change in the ERG rod a-wave waveform during the course of dark adaptation in two patients with Oguchi disease. Two Japanese patients showed a homozygous arrestin 1147delA mutation. Scotopic flash ERGs were recorded after different periods of dark adaptation. ERG rod a-waves were obtained after subtraction of the cone ERG contribution. The rod a-waves were fitted with a model of...

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Purpose Electroretinography (ERG) is the gold standard in clinical examinations of retinal function. Corneal ERG is widely used for diagnostics, but ERG components from the inner retina complicate quantitative investigations of the phototransduction cascade. Transretinal ERG (TERG) recorded ex vivo enables pharmacologic isolation of signals generated by photoreceptor cells, establishing an appe...

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Rod a-wave analysis using high intensity flashes adds information on rod system function in 25% of clinical ERG recordings

PURPOSE To investigate whether rod a-wave analysis using high intensity flashes adds information above that obtained with standard ERG. METHODS A total of 2,396 eyes were recorded. Patient age was 2.4 months-84.6 years. RESULTS A-wave analysis of high intensity flashes provided additional information on rod system function in 25% of eyes recorded, most importantly in subjects with midretina...

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A nonsense mutation in S-antigen (p.Glu306*) causes Oguchi disease

PURPOSE Genetic studies were performed to identify the causative mutation in a 15-year-old girl diagnosed with congenital stationary night blindness (CSNB) presenting Mizuo-Nakamura phenomenon, a typical Oguchi disease symptom. The patient also had dural sinus thrombosis (DST), thrombocytopenia, and systemic lupus erythematosus (SLE). METHODS Mutation analysis was done by sequencing two candi...

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PURPOSE To analyze cone and rod phototransduction parameters from ERG a-waves in patients with RP and to determine the relationships among these parameters, age, and mode of inheritance. METHODS Sets of four white flashes (3.2-4.4 log scotopic troland [scot td-s]) were presented in the dark. The same stimuli were later presented against a rod-saturating background and the generated cone a-wav...

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ژورنال

عنوان ژورنال: Vision Research

سال: 2004

ISSN: 0042-6989

DOI: 10.1016/j.visres.2003.06.003