Clinical Characteristics of the Charles Bonnet Syndrome in Patients with Neovascular Age-Related Macular Degeneration: The Importance of Early Detection

Ophthalmic Res. 2020;63(5):466-473. doi: 10.1159/000506137. Epub 2020 Jan 27.

Abstract

Purpose: We investigated the characteristics, prognosis, and clinical outcome of the Charles Bonnet syndrome (CBS) in patients with neovascular age-related macular degeneration (AMD).

Methods: Five hundred psychiatrically healthy patients with neovascular AMD were screened for CBS. The individuals that fulfilled the inclusion criteria were systematically interviewed using a structured questionnaire that covered the impact, prognosis, risk factors, phenomenology, symptoms, and knowledge about the syndrome. A control group of 45 patients was used for comparison. Demographic data, current medication, and ocular risk factors were collected in all patients.

Results: Forty-five patients with CBS were identified. The majority of patients reported images that consisted of colored (62%) animals (44%) or faces (42%) that lasted for seconds (53%). Most patients reported a self-limited disease with a median duration of symptoms between 9 and 11.5 months, with only 7% knowing about CBS at symptom onset. The degree of visual deficit did not predict the characteristics, complexity, frequency, duration, or impact of visual hallucinations. One-third of patients reported negative outcome, which was associated with shorter duration of CBS (p = 0.023), fear-inducing images (p < 0.001), and impact on daily activities (p = 0.015).

Conclusion: The prevalence of CBS in neovascular AMD patients is high and clinically relevant. Patients with recent onset of visual hallucinations and describing fear-inducing images are at greater risk for negative outcome. Periodic screening may minimize the negative consequences of this disease.

Keywords: Age-related macular degeneration; Charles Bonnet syndrome; Low vision; Negative outcome; Visual hallucinations.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Charles Bonnet Syndrome / complications
  • Charles Bonnet Syndrome / diagnosis*
  • Charles Bonnet Syndrome / epidemiology
  • Early Diagnosis*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Portugal / epidemiology
  • Prevalence
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Surveys and Questionnaires
  • Visual Acuity*
  • Wet Macular Degeneration / complications
  • Wet Macular Degeneration / diagnosis*
  • Wet Macular Degeneration / epidemiology