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Bonnet’s syndrome in ophthalmology: literature review

ABSTRACT

With the increase of longevity and the quality of life, the number of people with low vision due to senility tends to grow up. Charles Bonnet’s syndrome (SCB) was cited in the XVIII century by Bonnet, when his grandfather blinded by cataracts and psychologically healthy cites visions of men, women, birds, and buildings. The main risk factor is low vision. Several ocular diseases may be involved as cataract or use of some medications. Although known, it is poorly diagnosed by neurologists, ophthalmologists and psychiatrists. To date there is no consensus on the treatment of Charles Bonnet’s syndrome.

Keywords:
Low vision; Hallucination; Elderly health

RESUMO

Com o aumento da longevidade e da qualidade de vida da população, o número de pessoas com baixa visão pela senilidade tende a crescer. A síndrome de Charles Bonnet (SCB) foi citada no século XVIII por Bonnet, quando seu avô, psicologicamente saudável e cego por catarata, citava visões de homens, mulheres, pássaros e construções. O principal fator de risco é visão diminuída. Algumas doenças oftalmológicas podem estar envolvidas como catarata ou uso de algumas medicações. Apesar de ser conhecida, é pouco diagnosticada por neurologistas, oftalmologistas e psiquiatras. Até o momento não existe um consenso no tratamento da síndrome de Charles Bonnet.

Descritores:
Baixa visão; Alucinação; Saúde do idoso

INTRODUCTION

Low vision is one of the most disabling causes for people. In the elderly, the ocular structures undergo changes, and the decline of the visual function is inevitable, being the third cause of functional disability in this population.(11 Ribeiro JE, De Freitas MM, De Sousa Araújo G, Rocha TH. Associação entre aspectos depressivos e déficit visual causado por catarata em pacientes idosos. Arq Bras Oftalmol. 2004;67(5):795-9.,22 Borges SM, Cintra FA. Avaliação da função visual em idosos em seguimento ambulatorial . 2009;7(3):161-5.) Most of them do not report vision complaints because they consider this weakness typical of old age, that is why it is barely detected in the clinical practice.(33 Luiz LC, Rebelatto JR, Coimbra AMV, Ricci NA. Associação entre déficit visual e aspectos clínico-funcionais em idosos da comunidade. Rev Bras Fisioter. 2009;13(5):444-50.,44 Borges SM, Cintra FA. Relação entre acuidade visual e atividades instrumentais de vida diária em idosos em seguimento ambulatorial. Rev Bras Oftalmol. 2010;69(3):146-51.)

In the eighteenth century, the philosopher Charles Bonnet reported symptoms reported by his grandfather, Charles Lullin, a 90-year-old man blinded by cataracts, describing vision of men, women, animals, plants, and objects. Except for cataract blindness, he did not present any other pathologies. Even Bonnet got old and presented a similar clinical condition, and later his name was used to describe the syndrome.(55 Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.

6 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.

7 Arun P, Jain R, Tripathi V. Atypical charles bonnet syndrome. Indian J Psychol Med. 2013;35(4):402-4.
-88 Santos-Bueso E, Serrador-García M, Porta-Etessam J, Rodríguez-Gómez O, Martínez-De-La-Casa JM, García-Feijoo J, et al. Síndrome de Charles Bonnet. Serie de 45 casos. Rev Neurol. 2015;60(8):337-40.)

Charles Bonnet syndrome (CBS) is more common in the elderly and in association with reduced visual acuity. Most of these patients do not have cognitive or psychiatric disorders, and are aware of the unreal nature of the facts(55 Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.,77 Arun P, Jain R, Tripathi V. Atypical charles bonnet syndrome. Indian J Psychol Med. 2013;35(4):402-4.,99 Serrador-García M, Santos-Bueso E, Sáenz-Francés F, Díaz-Valle D, Martínez-De-La-Casa-Borrelia JM, García-Feijóo J. Charles Bonnet plus syndrome: Apropos of a case. Eur J Ophthalmol. 2012;22(5):836-9.)

This disease is poorly diagnosed by psychiatrists, ophthalmologists and neurologists, although it has been cited since the 18th century. (55 Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.,66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,1010 Gama Marques J, Antunes AP, Barroso C. Síndrome de Charles Bonnet : caso clínico e revisão bibliográfica. 2009;11(4):48-59.) In general, the affected population reaches 0.5% to 1.4%, depending on the study, and it is said that the Asian population is more affected. In Japan, more precisely in the city of Kitakyushu, a study was carried out diagnosing CBS in elderly patients with low vision of 0.8%. In New Zealand, 500 patients were treated in the ophthalmology service, with an incidence of 11% of thre disease.(66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,1010 Gama Marques J, Antunes AP, Barroso C. Síndrome de Charles Bonnet : caso clínico e revisão bibliográfica. 2009;11(4):48-59.,1111 Shiraishi Y, Terao T, Ibi K, Nakamura J, Tawara A. The rarity of Charles Bonnet syndrome. J Psychiatr Res. 2004;38(2):207-13.)

CBS is characterized by the presence of complex and isolated, persistent or repetitive, colored visual hallucinations in which people, trees, animals in motion are visualized, but they cannot be touched or heard. The duration can be of seconds to the whole day, extending for days or years and varying in frequency and complexity. Some types may or may not go away when the eyes are closed, especially when associated with poor visual acuity. The most common ophthalmic pathologies associated are: age-related macular degeneration (AMD), followed by glaucoma and cataract. However, patients suffering from a sudden onset of vision loss and visual field associated with brain disease are more likely to experience hallucinations than those with gradual loss of vision. (55 Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.,1010 Gama Marques J, Antunes AP, Barroso C. Síndrome de Charles Bonnet : caso clínico e revisão bibliográfica. 2009;11(4):48-59.,1212 Vale TC, Fernandes LC, Caramelli P. Charles Bonnet syndrome: characteristics of its visual hallucinations and differential diagnosis. Arq Neuropsiquiatr. 2014;72(5):333-6.

13 Reichert DP, Seriès P, Storkey AJ. Charles Bonnet syndrome: Evidence for a generative model in the cortex? PLoS Comput Biol. 2013;9(7): e1003134.

14 Tamsak RL, Zaret CR, Weidenthal D. Charles Bonnet syndrome precipitated by brimonidine tartrate eye drops. Br J Ophthalmol. 2003 Jul; 87(7): 917.
-1515 Garcia-Catalan MR, Arriola-Villalobos P, Santos-Bueso E, Gil-de-Bernabe J, Diaz-Valle D, Benitez-del-Castillo JM, et al. Charles Bonnet syndrome precipitated by brimonidine. Arch Soc Esp Oftalmol. 2013;88(9):362-4.)

The main triggering factor is low lighting. Social isolation, fatigue, weak light conditions at night, and states of drowsiness or relaxation have been associated with higher rates of recurrence. There are also reports of hallucinations with the use of some drugs such as antibiotics, hormones and of cardiovascular action. Brimonidine tartrate, eyedrops frequently used in the treatment of glaucoma, is also reported to induce seizures, which are controlled after discontinuation of the drug. (66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,1414 Tamsak RL, Zaret CR, Weidenthal D. Charles Bonnet syndrome precipitated by brimonidine tartrate eye drops. Br J Ophthalmol. 2003 Jul; 87(7): 917.,1515 Garcia-Catalan MR, Arriola-Villalobos P, Santos-Bueso E, Gil-de-Bernabe J, Diaz-Valle D, Benitez-del-Castillo JM, et al. Charles Bonnet syndrome precipitated by brimonidine. Arch Soc Esp Oftalmol. 2013;88(9):362-4.)

A study published in 2015 describes women over 80 years old and with a vision to count fingers at 1 meter as the most affected ones, and that 40% of patients who hallucinated had no triggering factor. (88 Santos-Bueso E, Serrador-García M, Porta-Etessam J, Rodríguez-Gómez O, Martínez-De-La-Casa JM, García-Feijoo J, et al. Síndrome de Charles Bonnet. Serie de 45 casos. Rev Neurol. 2015;60(8):337-40.)

Among the theories attempting to explain visual hallucinations in CBS, the most accepted one is that the reduction or absence of visual stimuli would initiate the disorders by reducing the suppression of superior cortical centers, promoting the release of perception, which results in phantom visions. The possibility of “sensitive deprivation” combined with the release of the thalamic-cortical pathways and molecular and biochemical alterations that may affect GABA A and GABA B7 receptors has also been exposed was also considered. (55 Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.,66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,1010 Gama Marques J, Antunes AP, Barroso C. Síndrome de Charles Bonnet : caso clínico e revisão bibliográfica. 2009;11(4):48-59.,1616 Rojas-Rojas H, Borja-Ballesteros C, Escobar-Córdoba F. Charles Bonnet syndrome: two cases | Síndrome de Charles Bonnet: Presentación de dos casos. Rev Chil Neuropsiquiatr. 2007;45(2):161-5.)

The differential diagnosis can be made several neurological and psychiatric pathologies, such as: dementia with Lewys bodies, Alzheimer’s, Parkinson’s, delirium, schizophrenia, bipolar disorder, epilepsy, auricle migraine, hysteria, psychosis, temporal arteritis, cortical lesions, extreme exhaustion conditions.55 Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.,66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,88 Santos-Bueso E, Serrador-García M, Porta-Etessam J, Rodríguez-Gómez O, Martínez-De-La-Casa JM, García-Feijoo J, et al. Síndrome de Charles Bonnet. Serie de 45 casos. Rev Neurol. 2015;60(8):337-40.,1717 Donoso A, Silva C, Fuentes P, Gaete G. Síndrome de Charles Bonnet: presentación de tres casos y revisión de la literatura. Rev Méd Chile. 2007;135(8):1034-9.

The approach is multidisciplinary: psychiatry, ophthalmology and neurology. Fear of the stigma of psychiatric illness causes patients to hide their symptoms, making diagnosis difficult. The patient’s awareness that CBS is not a mental illness reduces their and the family’s anxiety. (66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,1818 Brucki S, Takada L, Nitrini R. Charles Bonnet Syndrome: case series. Dement Neuropsychol. 2009;3(1):61-7.,1919 Muñoz Cortés H, Vargas Rueda A. Síndrome de Charles Bonnet: revisión de tema. Rev Colomb Psiquiatr. 2007;36(2):292-306.)

The treatment aims at ceasing hallucinations. However, to date, there is no therapeutic consensus for this condition. The visions of some patients disappear without intervention. Authors suggest that treatment should be performed if hallucinations are frequent, causing anxiety or altering the patients’ quality of life. Measures such as improving home lighting and social contact, wearing corrective lenses, cataract surgery, or treating other conditions to improve vision can be effective. However, in some cases the hallucinations cease as the vision worsens or disappears completely. (1818 Brucki S, Takada L, Nitrini R. Charles Bonnet Syndrome: case series. Dement Neuropsychol. 2009;3(1):61-7.,2020 Negrete MY, Ramírez-Bermúdez J, Ruiz-Chow A, Aguilar-Venegas LC, Crail-Melendez D, Pérez-Esparza R, et al. Alucinaciones visuales en pacientes con privación sensorial: Síndrome de Charles Bonnet. Salud Ment. 2015;38(3):217-24.)

Some mention the benefit of medications such as risperidone, cisapride, valproate, carbamazepine, clonazepam, selective serotonin reuptake inhibitors, gabapentin and olanzapine. In cases of patients who developed the syndrome after using specific medications, it is necessary to interrupt their use. (66 Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.,1818 Brucki S, Takada L, Nitrini R. Charles Bonnet Syndrome: case series. Dement Neuropsychol. 2009;3(1):61-7.,2020 Negrete MY, Ramírez-Bermúdez J, Ruiz-Chow A, Aguilar-Venegas LC, Crail-Melendez D, Pérez-Esparza R, et al. Alucinaciones visuales en pacientes con privación sensorial: Síndrome de Charles Bonnet. Salud Ment. 2015;38(3):217-24.)

FINAS CONSIDERATIONS E PERSPECTIVES

Decreased visual function is associated with alterations in the quality of life and reduced functional activities in elderly patients, which may lead to isolation, disturbance, loss of health and dignity.

With increasing longevity, the number of people affected by CBS tends to grow. The diagnosis and early understanding of this form of benign hallucination is essential in the attempt to minimize the impact on the life of the patient and their relatives.

Currently, health professionals are not prepared to differentiate it from other hallucinatory senile conditions, impairing the onset of treatment or even instituting the wrong treatment.

Educational campaigns among ophthalmologists, geriatricians, and professionals dealing with senile patients implementing an active search in patients with visual complaints associated with low vision would be an effective measure to improve treatment. However, a greater number of studies is necessary to improve knowledge on the cause, and to create a standardized treatment routine.

REFERÊNCIAS

  • 1
    Ribeiro JE, De Freitas MM, De Sousa Araújo G, Rocha TH. Associação entre aspectos depressivos e déficit visual causado por catarata em pacientes idosos. Arq Bras Oftalmol. 2004;67(5):795-9.
  • 2
    Borges SM, Cintra FA. Avaliação da função visual em idosos em seguimento ambulatorial . 2009;7(3):161-5.
  • 3
    Luiz LC, Rebelatto JR, Coimbra AMV, Ricci NA. Associação entre déficit visual e aspectos clínico-funcionais em idosos da comunidade. Rev Bras Fisioter. 2009;13(5):444-50.
  • 4
    Borges SM, Cintra FA. Relação entre acuidade visual e atividades instrumentais de vida diária em idosos em seguimento ambulatorial. Rev Bras Oftalmol. 2010;69(3):146-51.
  • 5
    Bonnet C. Síndrome de Charles Bonnet : a propósito de um caso. 2009;45(2):21-4.
  • 6
    Cortizo V, Marques Rosa AA, Soriano DS, Takada LT, Nitrini R. Síndrome de Charles Bonnet: Alucinações visuais em pacientes com doenças oculares - Relato de caso. Arq Bras Oftalmol. 2005;68(1):129-32.
  • 7
    Arun P, Jain R, Tripathi V. Atypical charles bonnet syndrome. Indian J Psychol Med. 2013;35(4):402-4.
  • 8
    Santos-Bueso E, Serrador-García M, Porta-Etessam J, Rodríguez-Gómez O, Martínez-De-La-Casa JM, García-Feijoo J, et al. Síndrome de Charles Bonnet. Serie de 45 casos. Rev Neurol. 2015;60(8):337-40.
  • 9
    Serrador-García M, Santos-Bueso E, Sáenz-Francés F, Díaz-Valle D, Martínez-De-La-Casa-Borrelia JM, García-Feijóo J. Charles Bonnet plus syndrome: Apropos of a case. Eur J Ophthalmol. 2012;22(5):836-9.
  • 10
    Gama Marques J, Antunes AP, Barroso C. Síndrome de Charles Bonnet : caso clínico e revisão bibliográfica. 2009;11(4):48-59.
  • 11
    Shiraishi Y, Terao T, Ibi K, Nakamura J, Tawara A. The rarity of Charles Bonnet syndrome. J Psychiatr Res. 2004;38(2):207-13.
  • 12
    Vale TC, Fernandes LC, Caramelli P. Charles Bonnet syndrome: characteristics of its visual hallucinations and differential diagnosis. Arq Neuropsiquiatr. 2014;72(5):333-6.
  • 13
    Reichert DP, Seriès P, Storkey AJ. Charles Bonnet syndrome: Evidence for a generative model in the cortex? PLoS Comput Biol. 2013;9(7): e1003134.
  • 14
    Tamsak RL, Zaret CR, Weidenthal D. Charles Bonnet syndrome precipitated by brimonidine tartrate eye drops. Br J Ophthalmol. 2003 Jul; 87(7): 917.
  • 15
    Garcia-Catalan MR, Arriola-Villalobos P, Santos-Bueso E, Gil-de-Bernabe J, Diaz-Valle D, Benitez-del-Castillo JM, et al. Charles Bonnet syndrome precipitated by brimonidine. Arch Soc Esp Oftalmol. 2013;88(9):362-4.
  • 16
    Rojas-Rojas H, Borja-Ballesteros C, Escobar-Córdoba F. Charles Bonnet syndrome: two cases | Síndrome de Charles Bonnet: Presentación de dos casos. Rev Chil Neuropsiquiatr. 2007;45(2):161-5.
  • 17
    Donoso A, Silva C, Fuentes P, Gaete G. Síndrome de Charles Bonnet: presentación de tres casos y revisión de la literatura. Rev Méd Chile. 2007;135(8):1034-9.
  • 18
    Brucki S, Takada L, Nitrini R. Charles Bonnet Syndrome: case series. Dement Neuropsychol. 2009;3(1):61-7.
  • 19
    Muñoz Cortés H, Vargas Rueda A. Síndrome de Charles Bonnet: revisión de tema. Rev Colomb Psiquiatr. 2007;36(2):292-306.
  • 20
    Negrete MY, Ramírez-Bermúdez J, Ruiz-Chow A, Aguilar-Venegas LC, Crail-Melendez D, Pérez-Esparza R, et al. Alucinaciones visuales en pacientes con privación sensorial: Síndrome de Charles Bonnet. Salud Ment. 2015;38(3):217-24.

Publication Dates

  • Publication in this collection
    Jul-Aug 2018

History

  • Received
    09 Feb 2018
  • Accepted
    07 May 2018
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