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Best disease

Best disease is a form of juvenile macular degeneration. While there is no cure for it, there are options that can slow the progression of the disease and improve visual function.

Best disease in Bellflower

What is best disease?

Best disease, also known as Best vitelliform macular dystrophy, is a genetic form of juvenile macular degeneration. In macular degeneration (AMD), the macula, the central part of the retina, is progressively damaged, causing loss of central vision. The prevalence of Best disease is estimated at 1 in 16,500 to 1 in 21,000 people. According to another estimate, about 1 in 15,000 people suffer from it.

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How can a low vision optometrist help?

People with Best disease and other conditions that cause vision loss may benefit from low vision optometrists who undergo advanced training in this field. Their advanced knowledge of the latest vision technologies helps improve vision both practically and functionally.

They may recommend you use low vision aids such as magnifying devices, CCTVs, tinted lenses, prisms, and digital devices. Once the doctor understands what matters to you, they will help you find the devices that will maximize your vision for the tasks you care about. People with this disease and similar conditions have now been able to do things they never thought they could with the help of state-of-the-art custom low vision optical glasses and devices.

Can you drive if you have Best disease?

With Best disease, the macula which is responsible for central vision is affected. It is vital to have a healthy macula so that you can drive safely and read road signs. Even though vision is affected by the disease, for most people it does not impair their ability to drive. The use of low vision glasses allows many patients with this condition to keep their licenses until late in life.

In the majority of states, low vision optometrists can assist you in maintaining your license and regaining your driving ability through bioptic telescopes customized to your prescription.

A bioptic telescope consists of a pair of glasses with a telescope attached above your normal line of sight. These can be attached to your regular eyeglasses and can be used either for one or both of your eyes. Bioptic lenses magnify images just like binoculars and help you see things better. Using these lenses allows you to see things that are much further away, which is one of their main benefits.

See our page on the driving laws in California with low vision.

Can you drive if you have Best disease?
What are the common symptoms of Best disease?

What are the common symptoms of Best disease?

The average age at which Best disease begins is between 5 and 10 years old, but it can vary from person to person. In the beginning, individuals with this condition have normal vision, but may experience the following symptoms with time:

  • Blurred vision
  • Reduced sharpness or clarity of vision
  • Objects that appear distorted (metamorphosis)
  • Loss of central vision

Many people with this disorder are unaware that their vision is declining and may be diagnosed during a routine eye examination. Others experience significant loss of vision, due to blood vessels forming under the macula and retina. It is possible for each eye to suffer from different degrees of visual loss. In most cases, individuals with Best disease have one eye more severely affected than the other, but with the assistance of a low vision optometrist can still engage in daily activities such as driving well into their old age.

Causes of Best disease

Best is inherited genetically from one parent through an autosomal dominant pattern. That means that a faulty gene is responsible for the condition. A defective gene inherited from one parent dominates the healthy gene inherited from the other parent. Another possibility is that the mutated gene was not passed down from the parent to the child, in which case the child would not inherit the disease. If one parent has the disease, there is a 50/50 chance of inheriting it.

What are the common symptoms of Best disease?

How is Best disease diagnosed?

Best disease can be diagnosed by looking for lesions that resemble egg yolks at the back of the eye, seen in the vitelliform stage. There are other tests available to confirm the diagnosis.

  • OCT is useful for localizing the lesion and determining its thickness, which may reveal lipofuscin and fluid buildup in the RPE.
  • Autofluorescent imaging can detect the build-up of fatty deposits in the back of the eye.
  • Best disease can also be detected in patients through an electrophysiological test called an EOG. An EOG test measures the health and activity of the RPE cells in the back of the eye. EOG recordings are used to calculate Arden ratios, which are generally 1.5 or lower in patients with Best disease.
  • The ERG test is also used in the diagnosis of patients with the Best disease. It is used to measure retinal function.
  • Another way to diagnose the Best disease is by genetic testing.

What are the different stages of Best disease?

Best disease progresses through six stages. They are discussed below:

Stage I - Pre Vitelliform

At this point, you are likely to not be experiencing symptoms and you have not yet developed the yellow material below your retina.

Stage II - Vitelliform

This term vitelliform means shaped like an egg. At this stage, yellow material is forming an egg-like shape. You may still have good vision.

Stage III - Pseudohypopyon

It is possible that the yellow material that was forming under the retina at stage 2 will develop into a cyst.

Stage IV - Vitelliruptive

Your retina may begin to be damaged by the yellow material, and your vision may be affected.

Stage V- Atrophic

As the yellow material disappears, it leaves scars and damaged cells behind. This stage is considered to be the final phase of Best disease by some researchers.

Stage VI - Choroidal neovascularization or CNV

There are some researchers who believe that CNV is the final stage of Best disease, while there are others who believe that it is a complication of it. About 20% of people with Best disease develop CNV. Choroid neovascularization refers to the development of new blood vessels. Those blood vessels may leak, causing your vision to deteriorate.

Can you drive if you have Best disease?
What are the common symptoms of Best disease?

Treatment and prevention of best disease

Unfortunately, as of now, there is no effective medical or surgical treatment for Vitelliform macular degeneration. There is currently research being conducted on gene therapy for the treatment of different types of inherited macular dystrophies. By replacing the faulty gene with a new functioning gene, gene therapy aims to correct the problem. The research is in the beginning stages and may take a while before it could potentially become a useful treatment, however this kind of research is a positive step in the right direction.

Clinical Trials using gene therapy for Best’s disease

One of the most exciting trials is in the field of gene therapy used for treating Best’s disease. Research was done at the University of Pennsylvania and the University of Florida that demonstrated the efficacy of treating best disease through gene therapy in Dogs. The approach taken has also been used in the FDA approved retinal gene therapy for Leber’s congenital amaurosis. The hope is that this approach that has been shown to work in both dogs and in other FDA approved treatments for retinal disease, will also translate into an effective treatment for Best’s disease. Clinical trials are underway, and your low vision optometrist or ophthalmologist will inform you if the treatment becomes available.

Prevention

Head trauma may increase the risk of developing the Best disease. Therefore, If you're at risk for a head injury, it's a good idea to take extra precautions, such as avoiding contact sports and wearing a helmet while cycling.

Anti-VEGF injections

Laser therapy and anti-VEGF injections can be used to treat new blood vessels. It may not lead to a significant improvement in sight, but treating blood vessels can prevent the macula and eyes from further damage.

Diet and smoking

There is evidence that smoking accelerates other forms of macular disease, so stopping smoking might help delay progression of Best disease as well. Currently, no research has indicated that diet can help to slow the progression of the Best disease. A diet high in fresh fruit and vegetables, however, may be beneficial for maintaining eye health.

Common Questions

No, while Best’s disease can lead to severe loss of central vision, it does not result in blindness as it does not harm your peripheral or side vision. Low vision optometrists can usually help maximize remaining vision in most cases. Best disease only affects the macula of your retina and does not affect peripheral vision. Therefore, your peripheral vision, which is your vision on the sides, remains intact.
Best disease
Dr. Ikeda cartoon

Summary

Best disease, also known as Best vitelliform macular dystrophy, is a genetic form of juvenile macular degeneration. In macular degeneration (AMD), the macula, the central part of the retina, is progressively damaged, causing loss of central vision. People with Best disease and other conditions that cause vision loss may benefit from low vision optometrists who undergo advanced training in this field. Their advanced knowledge of the latest vision technologies helps improve vision both practically and functionally. They may recommend you use low vision aids such as magnifying devices, CCTVs, electronic devices, lighting, tints, and prisms to help you regain your independence.

Patients visit our low vision clinic from all over California, and we are proud to be a leading low vision provider in , Long Beach, Lakewood, Los Angeles. 

You can schedule a low vision evaluation with our low vision optometrist by calling (562) 925-6591

Testimonials


I haven't actually used the optometrist side, so my review is limited to the vision therapy offered.  This office was recommended by my occupational therapist for the treatment of my double vision following a stroke.


Claire A.

Love this location. I had a brain injury accident from day one one. All the team make you feel you still important and hope in the horizon after when the medical system fell you miserably. Dr. Ikeda very professional and very understanding about your issue. Two tombs up.


Jim K.

My husband and I were immediately impressed with Dr Ikeda. I was hit by a car while cycling which caused broken bones and three brain injuries. The brain injuries caused double vision. Dr. Ikeda examined my eyes and got me started on vision therapy with his occupational therapist who specializes in vision therapy.  She (Chris) is absolutely great.  I am impressed with the array of tools used to help recover my binocular vision.  I am doing things I never thought were possible (balance boards etc).  Chris pushes me and keeps me motivated. I really enjoy my sessions with her.  The office staff is always friendly and they have a wonderful appointment reminder tool that makes it easy to keep my calendar up to date. I am happy the rehab center at Little Co. of Mary recommended them!!


Teresa S.

The Vision Therapy is handled in a separate office through a different door from the shared waiting room. Chris, the vision therapist, has a wide and varied assortment of tools, equipment and resources to best evaluate and treat most vision issues. After just a few visits, my double vision became easier to control, using exercises developed during the therapy process. It was time well-spent.


Joe M.

I have been coming here since I can remember. I love it here. The staff is so amazing and nice. They explain everything they gonna do and never make you feel rushed. Dr. Ikeda has always been my doctor and I would never want another one. He is the doctor for my whole family and is always asking how everyone is doing. I am also so crazy about picking out my frames and have to try so many and each person who helps me take the time and lets me try them all on. I would never want to go anywhere else! I definitely would recommend this office to anyone looking for a great eye doctor.


Kayla W.

This is not for the eye exam but for the frames selection portion. I brought my prescription from Kaiser here with my VSP insurance. The reception staff was very polite and professional.  Manny helped me to pick out frames and explain my coverage.  Very helpful and patient not like the individual at the other place I rated here on Yelp.  I was running late and he still helped me and was not bothered at all.  I am so glad I came here ..........very pleased.

Very professional staff and pleasant.


Daniel G.
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