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Retinitis Pigmentosa

DESCRIPTION: A group of diseases which result in the degeneration of the retina; cause is unknown, but suspected to be an enzyme in the retina; most types are hereditary, but the pattern of inheritance varies. Rods are destroyed, beginning in the mid-periphery, and gradually advancing inward toward the macula; "tunnel vision" is retained in the most common types, but central acuity is also diminished in other types; many are myopic. Many develop cataracts but are not as likely to have glaucoma or detached retinas. In Usher's syndrome (5% prevalence among R.P.), central acuity is retained but there is accompanying hearing loss; this type emerges during the teen years. In the "centro-peripheral" type of R.P. (17% of cases), onset is around 6-15 years of age, there is no nystagmus, and both central and peripheral vision is affected. In Leber's Disease (26% of R.P. cases), both central and peripheral vision is affected, nystagmus is present, and cataracts do not usually develop; it is congenital. The most common type (52%) also appears during the teen years, is typified by severe and progressive peripheral loss, but good central acuity (no worse than 20/50) is maintained until around age 60. Varieties of the common type are genetically different, and have characteristic loss of central acuity at different ages (e.g., 40-60). A few syndromes have R.P. as a component (e.g., Laurence-Moon-Biedl). Night blindness is the initial symptom and occurs early; deterioration of peripheral vision follows.

TREATMENT: There is no known treatment for R.P. There is considerable research effort in this direction.

Monitoring of retinal degeneration (locus and rate of change) via ERG and ophthalmoscopy of widest retinal periphery.

A variety of optical aids may be effective (e.g., magnifiers if fields are not severely restricted, hand telescopes, CCTV, and "pocketscopes" - infra-red devices for highs use). Prism lenses may be useful. Higher levels of illumination may be helpful.

IMPLICATIONS: GENETIC COUNSELING IS ESSENTIAL.

Functional vision evaluations may need to be "as needed" (rather than annually).

Identification of the type of R.P. is essential in establishing prognosis. Retinal specialists are usually better qualified to examine the widest area of the retina viewable. Regular eye exams are desirable, to track the rate and degree of deterioration.

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