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NR 3/2004
SUPLEMENT

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Elekroretinogram i
elektrookulogram w rodzinie z chorob± Stargardta
Electroretinogram and
electrooculogram in family with Stargardt’s disease
Dorota Pojda-Wilczek, Katarzyna
Makowiecka-Obidzińska, Ewa Herba
Z Katedry i Oddziału Klinicznego Okulistyki ¦l±skiej Akademii
Medycznej
Szpital Specjalistyczny nr 1 w Bytomiu
Kierownik: prof. dr hab. n. med. Stefan M. Pojda |
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| Summary: |
Purpose: The aim of
this study is to present the earliest functional changes
in Stargardt disease in family with three affected and
one unaffected children.
Patients and methods: Four children: three girls
(4, 7, 10, year old) and one boy (13 year old) and their
parents (34 rear old mother and 36 year old father) were
examined. Visual acuity (Snellen card), color perception
(Ishihara’s cards), electroretinography (flash
full-field ERG – standard and chromatic) and
electrooculography (EOG) were done. For ERG and EOG LKC
equipment and UTAS E-2000 program was used.
Results: 13 year old boy has had visual acuity
about 5/50 for about 3 years, nystagmus and no color
perception. On his ocular fundus abnormal macular reflex
(like beaten metal) was seen. Visual acuity about 5/12
was found in 7 year old girl during routine examination
at school, but color perception and ocular fundus were
normal. The others examined family members had visual
acuity 5/5 without correction and no changes on ocular
fundus. Color perception was normal in girls and mother.
The father had red-green deficiency. Arden ratio in EOG
was below normal (1.4) only in boy but dark trough and
light peak amplitudes were lower than normal also in
girls 4 and 7 year old and these two girls were also
suspected of Stargardt’s disease. ERG was nearly
abolished in boy and markedly abnormal in his 7 year old
sister. In father’s ERG small reduction of cone function
especially after chromatic stimulations was found. ERG
was normal and similar in mother and 10 year old
daughter. ERG was not done in the youngest girl. Two
years later loss of visual acuity to 4/50 occurred in
girl previously 7 and to 5/6 in the youngest sister.
Conclusion: Detail analysis of EOG and ERG
results may lead to diagnose retinal dystrophy in
preclinical stadium. |
| Słowa kluczowe: |
choroba Stargardta,
elektroretinogram, elektrookulogram. |
| Key words: |
Stargardt’s disease,
electroretinogram, electrooculogram. |
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