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Published on: September 3, 2020
[Electroretinogram and electrooculogram in a family with Stargardt's disease]
Dorota Pojda-Wilczek1, Katarzyna Makowiecka-Obidzińska, Ewa Herba
1Katedry i Oddziału Klinicznego Okulistyki Slaskiej Akademii Medycznej.
Insights
Early functional changes in Stargardt disease were detected in children using electroretinography (ERG) and electrooculography (EOG). These tests identified preclinical signs of retinal dystrophy, aiding in early diagnosis.
Area of Science:
- Ophthalmology
- Genetics
- Medical Imaging
Context:
- Stargardt disease is a common inherited macular dystrophy.
- Early detection of Stargardt disease is crucial for timely intervention.
- Family studies are important for understanding the genetic basis and progression of inherited retinal diseases.
Purpose:
- To identify the earliest functional changes in Stargardt disease within a family.
- To evaluate the utility of electroretinography (ERG) and electrooculography (EOG) in detecting preclinical Stargardt disease.
- To correlate functional test results with clinical findings in affected and unaffected family members.
Summary:
- A family with Stargardt disease was studied, including three affected children and one unaffected child.
- Functional testing using visual acuity, color perception, ERG, and EOG revealed subtle abnormalities in younger children, preceding significant visual loss.
- Two years later, the previously identified affected children showed a significant decline in visual acuity, confirming disease progression.
Impact:
- Electroretinography and electrooculography can diagnose retinal dystrophy in its preclinical stage.
- This study highlights the importance of comprehensive functional testing in families with a history of Stargardt disease.
- Early diagnosis through functional assessments may allow for future therapeutic strategies to slow disease progression.
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.
