reduce font size
increase font size
 

font size

Disease

Stargardt's Disease

 

Definition: Stargardt’s Disease is form of macular dystrophy that begins early in life. This is the most common form of inherited juvenile macular degeneration (usually before the age of 20). It is usually first noticeable between the ages of 8 and 12.  Boys and girls are equally affected by this condition. It is characterized by a reduction of central vision with a preservation of peripheral (side) vision.

Causes and Risk Factors: In this condition, the cone cells (at the macula) deteriorate in function and eventually die, while the rod cells generally remain intact.  Stargardt’s is usually a recessive inherited condition requiring the person to receive a gene from each parent to cause the disease. However, there have been a number of cases identified as dominant inheritance, requiring only one gene from either parent.

The diagnosis is usually made as a result of deterioration in visual acuity and typical appearances around the macula when the ophthalmologist looks into the eye. Electrical tests - such as the electro-retinogram (ERG), electro-oculogram and dark adaption testing - can measure the progress of the disease but are not usually necessary for its diagnosis.

Signs & Symptoms:

  • Loss of visual acuity or sharpness of vision
  • Decreased color vision
  • Small blind spots
  • May experience difficulties in adjusting to light

In some, this deterioration is rapid, while in others it is much slower. There is no way to detect how fast this deterioration will occur in any individual. By the age of 50, about half will have a visual acuity of 6/60 or worse (defined as "legal blindness").

Diagnostic Testing: 

Treatment: There is no cure, but research on this and other related diseases may identify a form of treatment.  Stargardt’s never causes total vision loss. Peripheral vision is left intact. Central vision is usually in the range of 20/100 to 20/400 with younger patients usually showing less loss. Low vision care can help Stargardt’s patients lead very normal lives. Following diagnosis every Stargardt’s patient should have a low vision examination by a doctor skilled in low vision rehabilitation.