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THE PSYCHIATRIC CONSULTANT

Series Editor Gene D. Cohen, MD, PhD

The Charles Bonnet syndrome


Visual hallucinations caused by vision impairment
Barry W. Rovner, MD

Case presentation

A 75-year-old retired attorney (Mr.


M) with age-related macular degeneration (AMD), hypertension, and
benign prostatic hypertrophy presents to his primary care physician
with complaints of visions. His
medications include hydrochlorothiazide and finasteride. He has no history of psychiatric illness and does
not abuse alcohol.
During the past 2 years, bilateral
AMD has caused Mr. Ms vision to
progressively deteriorate. As a consequence of the AMD, he can no longer
read or find clothing in his closet,
food on his plate, or items on his
kitchen shelves. During the last 2
months, he has developed visual hallucinations described in this way:
people coming and going, dressed
in costumes like football or soldier
uniforms . . . they put on galas, drive
vehicles, and are very busy outside
my house. He observes the people
talking with one another and using
construction materials to build something. He has never heard them
speak. Mr. M feels nothing when he
reaches out to them.
Although Mr. M recognizes the
hallucinations as figments of my
imagination, on occasion he has ordered the people to leave. The hallucinations tend to occur in the
evening. He says that they disappear

Dr. Rovner is professor of psychiatry, department of psychiatry and


human behavior, Jefferson Medical
College and Wills Eye Hospital,
Philadelphia, PA.

when he blinks his eyes quickly or


turns his head. He has considered
asking his neighbors about the people or calling the police, but has refrained because he suspects that only
he sees the images.
Mental status examination reveals
that Mr. M does not exhibit mood
disturbance, other hallucinations or
delusions, or cognitive deficits. Complete blood cell count, chemistry
panel, thyroid function, and B12 and
folate levels are within normal
ranges. MRI of the brain reveals scattered foci of hypoattenuation in the
subcortical and deep white matter,
consistent with nonspecific small
vessel disease.
Discussion

Mr. Ms symptoms are characteristic of the Charles Bonnet syndrome


(CBS). Like most persons with CBS,
Mr. M exhibits bilateral visual impairment and vivid, life-like hallucinations, no other mental disorders,
and some understanding that his visions are not real.1 The syndrome is
named after the 18th century Swiss
philosopher Charles Bonnet, who
described visual hallucinations in his
older visually impaired grandfather.
Although once considered rare, the
number of reported CBS cases is increasing, possibly because the aging
population is growing and vision
disorders such as AMD, glaucoma,
and cataracts increase with age.
Screening

Visually impaired patients who have


conditions such as AMD can bene-

fit from routine screening for visual


hallucinations. Screening can be as
simple as asking patients with visual
impairments whether they experience visions or hallucinations.
Diagnostic criteria

Despite the increasing prevalence of


CBS, diagnostic criteria remain controversial. Most patients exhibit visual system pathology, have an understanding that their hallucinations
are not real, and do not present with
other psychiatric disorders such as
delirium, schizophrenia, or substance abuse.1 One study has shown
that cognitive impairment, stroke,
or early Alzheimers disease might
be predisposing conditions.2
Among patients attending ophthalmologic clinics in the U.S., the
prevalence of CBS is approximately
14%.3-5 Teunisse et al studied the
largest series of CBS patients to date
and have provided the richest clinical descriptions of the visual hallucinations associated with the syndrome.3 During their study:
65% of patients hallucinated either weekly or monthly and 27%
hallucinated daily
80% of the hallucinations involved people, 38% animals, 25%
plants/trees, and 15% buildings or
other scenes
63% of the hallucinations were
in color, 47% often involved movement, and 45% were seen with
greater clarity than real objects
53% of the hallucinations lasted
from 1 to 60 minutes and 13% lasted
less than 5 seconds
continued

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June 2002 Volume 57, Number 6

Geriatrics

45

PSYCHIATRIC CONSULTANT
67% of patients experienced the
hallucinations with their eyes opened.
Most patients experienced the hallucinations during the evening or at
night, under conditions of poor lighting, and when they were inactive or
alone. Keeping their eyes closed, looking or walking away from the visions,
or turning on lights helped stop the
hallucinations. Patient emotional reactions varied: 32% became anxious
or distressed and 13% were amused.
Most patients did not report their
hallucinations to others out of fear
that they would be considered mentally ill.
Risk factors

For some patients, the visual hallucinations might precede or indicate the
presence of dementia. Pliskin et al
found that 14 CBS patients referred to
a psychiatry clinic exhibited cognitive
deficits suggestive of early dementia.2
By contrast, Holroyd and Rabins conducted a 3-year follow-up study of 100
AMD patients, 13% of whom had visual hallucinations, and found that no
patients experienced cognitive decline
during the study period.5
The strongest risk factors for CBS
include bilateral visual system impairment, declining visual acuity, cognitive deficits, stroke, and Alzheimers
disease. The etiology of CBS is unknown, although one theory suggests
that deafferentation of the visual system may alter receptive fields in the visual cortex and lead to spontaneous
neuronal discharge and hallucinations.6
Treatment

Hallucinations do not cause excessive


distress for most patients who have
CBS. Education and reassurance that
they dont have a mental disorder
might be sufficient treatment for some
patients. Other patients, such as the
one described here, are extremely distressed and require aggressive pharmacologic therapy. At the very least,
all patients require screening for cognitive deficits to determine whether
46

Geriatrics

June 2002 Volume 57, Number 6

Table Atypical antipsychotic medications for the treatment of CBS


Agent

Dosage range

Comments/precautions

Olanzapine
(Zyprexa)

2.5 to 10 mg/d

May cause extrapyramidal


symptoms

Quetiapine fumarate
(Seroquel)

25 to 100 mg/d

Same as olanzapine

Risperidone
(Risperdal)

0.25 to 1.0 mg/d

Same as olanzapine

Ziprasidone
(Geodon)

20 to 40 mg/d

May cause extrapyramidal


symptoms and QT
prolongation

Source: Prepared for Geriatrics by Barry W. Rovner, MD

dementia is present and ophthalmologic evaluation to identify correctable


vision disorders including refractive
error and cataracts.
Antipsychotic medications are used
to treat hallucinations that are associated with psychotic conditions such
as schizophrenia. Thus, pharmacologic treatment for CBS has focused
on the use of antipsychotics even
though their efficacy in CBS comes
from anecdotal evidence and has not
been established in clinical trials.
Antiepileptic agents have also been
used to manage hallucinations associated with CBS.7
The table provides dosing recommendations for the available atypical
antipsychotic agents. Compared with
typical antipsychotics, the atypical antipsychotics are associated with a substantially lower risk of inducing extrapyramidal symptoms and tardive
dyskinesia. They are less likely to cause
anticholinergic side effects, excess sedation, or orthostatic hypotension;
some agents, however, are associated
with elevations in blood sugar, weight
gain, and QT prolongation.
Nonpharmacologic interventions
include increasing lighting at home in
the evening and reducing social isolation by encouraging interpersonal contact. Careful screening, ophthalmologic and neuropsychiatric evaluations,
reassurance, practical nonpharmacologic interventions, and antipsychotic
medications will help control the condition for most patients.

Case resolution

Mr. Ms physician ruled out other medical and neuropsychological disorders


and recommended that he increase
lighting at home in the evening and
spend more time with family and
friends to avoid isolation. Mr. M was
also given a low-dose atypical antipsychotic. These steps greatly diminished
the intensity and frequency of his hallucinations. G

References
1. Gold K, Rabins PV. Isolated visual
hallucinations and the Charles Bonnet
syndrome: A review of the literature and
presentation of six cases. Compr
Psychiatry 1989; 30(1):90-8.
2. Pliskin NH, Kiolbasa TA, Towle VL, et al.
Charles Bonnet syndrome: An early
marker for dementia? J Am Geriatr Soc
1996; 44(9):1055-61.
3. Teunisse RJ, Cruysberg JR, Hoefnagels
WH, Verbeek AL, Zitman FG. Visual
hallucinations in psychologically normal
people: Charles Bonnets syndrome.
Lancet 1996; 347:794-7.
4. Scott IU, Schein OD, Feuer WJ, Folstein
MF. Visual hallucinations in patients
with retinal disease. Am J Ophthalmol
2001; 131(5):590-8.
5. Holroyd S, Rabins PV. A three-year
follow-up study of visual hallucinations
in patients with macular degeneration. J
Nerv Ment Dis 1996; 184(3):188-9.
6. Rabins PV. The genesis of phantom
(deenervation) hallucinations: A
hypothesis. Int J Ger Psych 1994;
9:775-7.
7. Ranen NG, Pasternak RE, Rovner BW.
Cisapride in the treatment of visual
hallucinations caused by vision loss:
The Charles Bonnet syndrome. Am J
Geriatr Psychiatry 1999; 7(3):264-6.