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ERG and EOG in pericentral pigmentary retinopathay
1216
IUPS-ISCERG Symposium
Papers
sex-linked inheritance. (1) Their visual functions, such as visual acuity, color sense, dark adaptation or peripheral field were found to be almost normal except slight constriction of blue peripheral field. (2) Ophthalmoscopically, a retinitis pigmentosa-like fundus was observed in each of the carriers, contrasting with their normal visual functions. However, no bone-corpuscle pigment-deposit was found in any of the cases. (3) The findings obtained by fluorescence angiography of the carriers closely resembled to those observed in the patient with the disease. (4) The photopic and scotopic ERG recorded from the carriers showed slight abnormalities. Especially, depression or absence of the ERG oscillatory potential was noted in six carriers. The VEP in all cases tested was normal.
48
Amuionceatesisin a sex-iiniced retinai degenemtion J. H. KELSEY. Moorfields Eye Hospital, City Road, London, E.C.l A GIRL was seen in 1964 as her fundi appeared to be abnormal. The family history was equivocal as to the mode of inheritance. Electrodiagnostic tests were normal. Six years later she became pregnant. Repeat el~trodia~~tic tests were still normal and it was considered that the patient was a carrier of a sex-linked disorder. She understood the chances of the condition occurring in a male child. Amnioncentesis at 13 weeks pregnancy showed cells with Barr bodies and she was delivered of a healthy female child at term.
49 ERGUaaEOGinperices~@~J
WY
J. FRAN$CMand A. DE ROUCK. Department of Ophthalmology, Universityof Ghent, De Pintelaan, 135, B-9000 Ghent, Belgium VISUALfunctions were tested in three patients with pericentrai pigmentary retinopathy, aged respectively 29,41 and 54 yr. The functional tests included visual f%eld,dark a~~at~a~, coiour vision tests, ERG, EOG and an~o~uorog~phy. The ERG was performed in light-and-dark adapted states both with singfe flashes of increasing intensity, low flicker and flicker ERG. The functional state was similar in all three patients: visual acuity ranging from 0.3 to 0.6, slightly sub-normal dark adaptation curve, large pericentral scotomas, severely disturbed colour vision of the red-green type with “scotopisation”. The electrophysiologi~al investigation showed some discrepan&es in one patient: an abnormal EGG was found together with a normal ERG, while in another the EGG was normal and the ERG severely disturbed. The ERG tidings were di&ent in the three patients: one was characterized by normal photopic and scotopic responses, one by normal scotopic and severely reduced photopic responses, and one by reduced photopic and scotopic responses,
IUPS-ISCERG Symposium
Papers
sex-linked inheritance. (1) Their visual functions, such as visual acuity, color sense, dark adaptation or peripheral field were found to be almost normal except slight constriction of blue peripheral field. (2) Ophthalmoscopically, a retinitis pigmentosa-like fundus was observed in each of the carriers, contrasting with their normal visual functions. However, no bone-corpuscle pigment-deposit was found in any of the cases. (3) The findings obtained by fluorescence angiography of the carriers closely resembled to those observed in the patient with the disease. (4) The photopic and scotopic ERG recorded from the carriers showed slight abnormalities. Especially, depression or absence of the ERG oscillatory potential was noted in six carriers. The VEP in all cases tested was normal.
48
Amuionceatesisin a sex-iiniced retinai degenemtion J. H. KELSEY. Moorfields Eye Hospital, City Road, London, E.C.l A GIRL was seen in 1964 as her fundi appeared to be abnormal. The family history was equivocal as to the mode of inheritance. Electrodiagnostic tests were normal. Six years later she became pregnant. Repeat el~trodia~~tic tests were still normal and it was considered that the patient was a carrier of a sex-linked disorder. She understood the chances of the condition occurring in a male child. Amnioncentesis at 13 weeks pregnancy showed cells with Barr bodies and she was delivered of a healthy female child at term.
49 ERGUaaEOGinperices~@~J
WY
J. FRAN$CMand A. DE ROUCK. Department of Ophthalmology, Universityof Ghent, De Pintelaan, 135, B-9000 Ghent, Belgium VISUALfunctions were tested in three patients with pericentrai pigmentary retinopathy, aged respectively 29,41 and 54 yr. The functional tests included visual f%eld,dark a~~at~a~, coiour vision tests, ERG, EOG and an~o~uorog~phy. The ERG was performed in light-and-dark adapted states both with singfe flashes of increasing intensity, low flicker and flicker ERG. The functional state was similar in all three patients: visual acuity ranging from 0.3 to 0.6, slightly sub-normal dark adaptation curve, large pericentral scotomas, severely disturbed colour vision of the red-green type with “scotopisation”. The electrophysiologi~al investigation showed some discrepan&es in one patient: an abnormal EGG was found together with a normal ERG, while in another the EGG was normal and the ERG severely disturbed. The ERG tidings were di&ent in the three patients: one was characterized by normal photopic and scotopic responses, one by normal scotopic and severely reduced photopic responses, and one by reduced photopic and scotopic responses,