Charles Bonnet syndrome in Leber’s hereditary optic neuropathy

  • Hana Kolarova
  • Claudia B. Catarino
  • Claudia Priglinger
  • Thomas KlopstockEmail author
Letter to the Editors

Dear Sirs,

Charles Bonnet Syndrome (CBS) is a condition characterized by visual hallucinations in patients with visual loss and normal neuropsychiatric status. Although there is no consensus on diagnostic criteria for CBS, most experts describe stereotyped hallucinations, complex or simple, static or moving, with better clarity than expected given the residual vision [1]. The condition was first described by Swiss naturalist Charles Bonnet in his 87-year-old grandfather who had severe visual loss due to bilateral cataracts [2]. So far, only one patient with Leber’s Hereditary Optic Neuropathy (LHON) has been reported with CBS [3].

Here, we present ten patients (6 male, 4 female; age range 22–67 years) with genetically confirmed LHON who experienced CBS during the course of their disease. All patients suffered from acute-subacute onset of bilateral visual loss at age 15–65 years. The Table 1summarizes their clinical characteristics, genotype and phenomenology of the hallucinations....


Compliance with ethical standards

Conflicts of interest

Drs. Priglinger and Klopstock have received research grants from Santhera Pharmaceuticals and from GenSight Biologics. Drs. Catarino and Klopstock received travel costs from Santhera Pharmaceuticals and from GenSight Biologics. Dr. Kolarova received speaker and consulting honoraria from Santhera Pharmaceuticals. Dr. Klopstock received speaker and consulting honoraria from Santhera Pharmaceuticals and from GenSight Biologics.

Ethics statement

The data presented here were collected in the context of standard medical care. Beyond, phenotyping of patients with mitochondrial disorders and analysis of the respective data has been approved by the ethics committee of the University of Munich in the context of the German network for mitochondrial disorders (mitoNET) and has, therefore, been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Neurology, Friedrich-Baur InstituteLudwig-Maximilians-Universität MünchenMunichGermany
  2. 2.Department of Pediatrics and Adolescent Medicine, First Faculty of MedicineCharles University and General University Hospital in PraguePragueCzech Republic
  3. 3.Department of OphthalmologyLudwig-Maximilians-Universität MünchenMunichGermany
  4. 4.Munich Cluster for Systems Neurology (SyNergy)MunichGermany
  5. 5.German Center for Neurodegenerative Diseases (DZNE)MunichGermany

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