Macular pigment and lutein supplementation in retinitis pigmentosa and Usher syndrome

PURPOSE, To determine macular pigment (MP) in patients with inherited retin al degeneration and the response of MP and vision to supplementation of lut ein. METHODS. Patients with retinitis pigmentosa (RP) or Usher syndrome and norm al subjects had MP optical density profiles measured with heterochromatic f licker photometry. Serum carotenoids, visual acuity, foveal sensitivity, an d retinal thickness (by optical coherence tomography [OCT]) were quantified . The effects oil MP and central vision of G months of lutein supplementati on at 20 mg/d were determined. RESULTS. MP density in the patients as a group did not differ from normal. Among patients with lower MP, there was a higher percentage of females, smo kers, and light-colored irides. Disease expression tended to be more severe in patients with lower MP. Inner retinal thickness by OCT correlated posit ively with MP density in the patients. After supplementation, all participa nts showed an increase in serum lutein. Only approximately half the patient s showed a statistically significant increase in MP. Retinal nonresponders had slightly greater disease severity but were otherwise not distinguishabl e from responders. Central vision was unchanged after supplementation. CONCLUSIONS. Factors previously associated with lower or higher MP density in normal subjects showed similar associations in RP and Usher syndrome. In addition, MP in patients may be affected by stage of retinal disease, espe cially that leading to abnormal foveal architecture. MP could be augmented by supplemental lutein in many but not all patients. There was no change in central vision after G months of lutein supplementation, but long-term inf luences on the natural history of these retinal degenerations require furth er study.