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Since retinitis pigmentosa begins as rod degeneration, the patient first notices increasing difficulty in night vision, followed by difficulty seeing in the periphery.

Slowly progressive constriction of the visual field leads to tunnel vision.

Generally night blindness precedes tunnel vision by years or even decades. The age of appearance of legal blindness ranges from as early as childhood to as late as the 40s.

Although the history (especially the possibility of retinitis pigmentosa appearing in other family members) and complaints of the patient may make one suspect RP, it is primarily diagnosed by examination.

Common to all of them is progressive degeneration of the retina, specifically of the light receptors, known as the rods and cones.

The rods of the retina are involved earlier in the course of the disease, and cone deterioration occurs later.

Cases that are diagnosed later in life are often milder and may progress more slowly.

A similar pattern may appear in congenital syphilis which is unrelated and must be distinguished.

Retinitis pigmentosa is the most common of a group of hereditary progressive retinal degenerations or dystrophies.

There is considerable variation and overlap among the various forms of retinitis pigmentosa.

Some cases are sporadic and lack a family history of the disease.

A thorough genetic pedigree, often with the aid of a genetic counselor, is essential in determining risk of future generations acquiring the disease.