Visual loss and visual hallucinations in patients with age-related macular degeneration (Charles Bonnet Syndrome)

PURPOSE. The condition in which visual hallucinations (VHs) are solely associated with a visual impairment is termed Charles Bonnet Syndrome (CBS). The study was undertaken to investigate whether the extent of visual acuity (VA) loss and central visual field loss predisposes a patient with age-related macular degeneration (AMD) to develop a CBS VH and, in addition, whether the progression in loss is mirrored in the complexity of the VHs reported. VH phenomenology and CBS prevalence were also examined. METHODS. Sixty-six patients (age range, 63-96 years, mean +/- SD 81.2 +/- 7.1 years) with bilateral AMD were questioned as to whether they had experienced any hallucinatory episodes exclusive to vision. The four-point primary inclusion criterion ensured that all patients had bilateral AMD, a bilateral central scotoma, best monocular VA poorer than or equal to 0.6 logMAR (logarithm of the minimum angle of resolution) and intact cognition (using the Mini Mental State Examination for the Blind and the Telephone Interview for Cognitive Status). The patients who did not report VH were classified into the non-VH group, with the remainder in the VH group. An extended Institute of Psychiatry Structural Interview characterized the phenomenology of the VH. A secondary inclusion criterion subdivided the VH group into the apparent CBS group, in which personal medical history may have contributed to VH generation, and the manifest CBS group, where VHs were solely as a result of the visual loss. RESULTS. Fifty-three patients met the primary inclusion criterion: 32 were classified into the non-VH group and 21 into the VH group. The VH group were slightly younger (median difference, 4 years, P = 0.03) and appeared to have a lower VA (median difference, 0.20 logMAR, P = 0.08) and a more extensive visual field loss (P = 0.06) than did the non-VH group. However, when these variables were evaluated simultaneously by logistic regression, only age emerged as a statistically significant predictor of VH (odds ratio 0.88, 95% confidence interval [CI] 0.8 - 0.99, P = 0.03). The prevalence of apparent CBS and manifest CBS in the AMD population was found to be 25% and 15%, respectively. With no clinical and phenomenological differences between the two CBS groups, the secondary inclusion criterion was withdrawn, the VH group was renamed the CBS group, and a prevalence of 40% was recalculated. Of the 82 visual phenomena experienced by the CBS group, 21 were classified as simple VHs and 39 as complex VHS, with the remainder classified as either entopic phenomena or visual inference. Patients who experienced both simple and complex VHs appeared to have a greater visual field loss (P = 0.06) compared with those patients who reported either solely simple or solely complex VHs. CONCLUSIONS. The extent of visual loss did not appear to be a predictor for the likelihood of a patient with AMD experiencing a CBS VH, nor was the progression of loss reflected in the complexity of the VHs reported.