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Lewy body dementia: Case report and discussion

Article  in  The Journal of the American Board of Family Practice / American Board of Family Practice · January 2002
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MEDICAL PRACTICE

Lewy Body Dementia: Case Report and Discussion

Natan Khotianov, MD, Ranjit Singh, MBBChir, MA(Cantab), and
Sonjoy Singh MBBChir, MA(Cantab)

Background: Lewy body dementia is a common but frequently underdiagnosed cause of dementia often
mistaken for the more familiar entity of Alzheimer disease. Clinically the distinction is important, be-
cause it can have profound implications for management.
Methods: The medical literature was searched using the keywords “Lewy bodies,” “Lewy body demen-
tia,” “Alzheimer dementia,” and “parkinsonian disorders.” A case of Lewy body dementia is described.
Results: An elderly man had long-standing diagnoses of Alzheimer disease and Parkinson disease.
After he was evaluated thoroughly, the diagnosis was revised to Lewy body dementia, leading to changes
in treatment that were associated with dramatic improvement in the patient’s mental status. Evidence
from the literature suggests that Lewy body dementia can be diagnosed in primary care settings based
on clinical criteria. The physician should be alert to this diagnosis, and special attention should be paid
to dementia patients who exhibit parkinsonism, hallucinations, fluctuating cognition, or prominent
visuosperceptual deficits.
Conclusions: The diagnosis of Lewy body dementia has important implications. It is associated with
a high incidence of neuroleptic sensitivity, necessitating great caution in the use of these common anti-
psychotic agents. Early studies indicate cholinesterase inhibitors can be beneficial for treating the hallu-
cinations and behavior disturbances that afflict these patients and might also improve cognition.(J Am
Board Fam Pract 2002;15:50 – 4.)

Lewy body dementia is a common cause of demen- Lewy body dementia initially diagnosed as Alzhei-
tia in the elderly, accounting for perhaps 15% to mer dementia in which a change of diagnosis and
25% of dementia cases.1,2 Evidence suggests that treatment led to striking improvements in the pa-
Lewy body dementia might be underdiagnosed, tient’s symptoms.
often being mistaken for the more familiar Alzhei-
mer disease. Clinically the distinction is important, Case Report
as it can have profound implications for manage- A 79-year-old man was brought to the emergency
ment and prognosis. An alternative diagnosis of department of a county hospital in November 2000
Lewy body dementia can lead to a trial of treatment after having been found by the police wandering on
that can be associated with dramatic improvement the street. The patient reported no complaints with
in the patient’s symptoms. the exception of visual hallucinations, which were
of a nonthreatening nature (eg, the patient had seen
Methods objects in the room, such as flowers and bread on
The medical literature was searched using the key- the table). At this point the patient was confused
words “Lewy bodies,” “Lewy body dementia,” and unable to provide a detailed history; therefore,
“Alzheimer’s dementia,” and “parkinsonian disor- the admitting physician relied on the patient’s wife
ders.” An illustrative case report describes a case of and daughter for most of the information.
Eight years earlier, the patient had Parkinson
disease diagnosed. He was examined by a neurolo-
Submitted, revised 19 June 2001 gist and was started on a combination carbidopa-
From the Department of Family Medicine (NK, RS) levodopa medication. The dosage was titrated up to
State University of New York at Buffalo, Buffalo; and the
Family Medicine Residency Program (SS), Niagara Falls 50 mg of carbidopa and 200 mg of levodopa four
Memorial Medical Center, Niagara Falls, New York. Re- times a day. Although the prescribing physician
print requests should be addressed to Ranjit Singh, MB-
BChir, Skilled Nursing Facility, Erie County Medical Cen- noted no improvement in his parkinsonian symp-
ter, 462 Grider St, Buffalo, NY 14215. toms, the medication was continued. Less than a

50 JABFP January–February 2002 Vol. 15 No. 1

year later, the patient developed memory problems, and quetiapine were both discontinued. Interest-
and Alzheimer disease was diagnosed. During the ingly, there was no change in the parkinsonian
next 5 years, the patient’s condition remained sta- symptoms as a result. By the end of the second
ble, and he continued to work as an attorney. week of treatment with donepezil, the patient be-
Two years before the November 2000 emer- came increasingly alert and cooperative with care-
gency department visit, the patient began experi- takers. His interactions with family and staff im-
encing frequent visual hallucinations, thought by proved considerably. His MMSE score at the end
the neurologist to be related to the antiparkinso- of the fourth week of treatment had risen to 27/30.
nian medication. The neurologist prescribed He was still unable to reproduce the intersecting
quetiapine (125 mg in the morning and 100 mg in pentagons, his recall was 2/3 after 5 minutes, and
the evening) in an attempt to control these hallu- he made one mistake when following a 3-step com-
cinations. Despite this treatment, these symptoms
mand. Because the patient’s parkinsonism and hal-
continued to occur intermittently. At the time the
lucinations persisted, the levodopa-carbidopa and
patient was seen in the emergency department, he
quetiapine were both subsequently restarted at
was still taking carbidopa-levodopa and quetiapine
lower doses. The patient was discharged from the
at the above dosages. He was taking no other med-
hospital and is currently being cared for by his
ications.
neurologist and his primary care physician.
The patient’s medical history, in addition to the
above, included a left hip replacement, glaucoma,
and a left arm fracture. The patient had worked as
an attorney in his family’s law firm for several Discussion
decades and had retired only 2 years previously. He Lewy body dementia is named after the German
was a respected and prominent member of his com- physician Fritz Heinrich Lewy, who at the begin-
munity. He had no history of smoking, alcohol, or ning of the 20th century first described distinctive
drug abuse. There was no reason to suspect expo- extranuclear inclusions on microscopic brain tissue
sure to the human immunodeficiency virus. examination. These inclusions became known as
When examined, the patient was a well-nour- Lewy bodies. It was not until the latter part of the
ished, well-hydrated elderly white man in no ap- 20th century that the importance of these lesions
parent distress. He had no fever; his pulse was 68 began to be recognized. Initially, a subgroup of
beats per minute, blood pressure was 150/70 mm Alzheimer disease patients was determined to have
Hg, and respirations were 16/min. The patient was characteristic pathologic findings of cortical or
alert and oriented to person but not to place or brainstem Lewy bodies at autopsy.1,2 Later it be-
time. He had an obvious tremor in both hands and came clear that these patients with Lewy bodies
feet. The tremor was coarse, symmetrical, and per- constituted a distinct neuropathologic and clinical
ceptible at rest and on intentional movement. His
entity, which became known as dementia with
gait was unsteady. There were no other neurolog-
Lewy bodies or Lewy body dementia.4 Various
ical findings. His score on Folstein’s Mini-Mental
subtypes have been described based on pathologic
State Examination (MMSE)3 was 20/30. We per-
findings, details of which can be found in the liter-
formed a standard workup, ruling out reversible
ature.4,5
causes of dementia (depression, hypothyroidism,
vitamin B12 and folate deficiency, neurosyphilis, The disease is more common in men than
subdural hematoma, brain tumor, and normal pres- women with a ratio of 2:1. The mean age at onset
sure hydrocephalus) and causes of delirium (includ- of symptoms is 68 years. The average time from
ing infection, electrolyte abnormalities, uremia, onset to death is 6.4 years, with the most frequent
toxic ingestion, hypoxia, stroke, and myocardial in- cause of death being aspiration pneumonia. The
farction). All laboratory test results were unremark- cause of Lewy body dementia remains unknown.
able. A computed tomogram of the head showed Some speculate that genetics plays a role, and stud-
only age-related cerebral atrophy. ies are underway to investigate this possibility. It
The provisional diagnosis of Lewy body demen- remains to be seen whether genetic testing will ever
tia was made, and the patient was given donepezil be sufficiently sensitive and specific to help in the
(Aricept) 5 mg at bedtime. The carbidopa-levodopa clinical diagnosis of Lewy body dementia.

Lewy Body Dementia 51

Diagnosis olism,9 or dopaminergic neuronal degeneration.10
Currently, definitive diagnosis of Lewy body de- It is hoped that these types of imaging studies will
mentia can be made only at autopsy. Clinically, as eventually become widely available for definitive
illustrated by this case, there is considerable overlap antemortem diagnosis.
between Lewy body dementia and both Alzheimer
disease and Parkinson disease. To improve the sen- Therapeutic Implications
sitivity and specificity of the clinical diagnosis, a set Perhaps the most clinically important feature of
of clinical criteria for Lewy body dementia was Lewy body dementia is the tendency for neurolep-
drafted in 1996 by the Consortium on Dementia tic sensitivity. Studies suggest that about 50% of
with Lewy Bodies.4 To diagnose Lewy body de- patients develop severe extrapyramidal symptoms,
mentia using these criteria, there must be progres- such as rigidity, altered consciousness, pyrexia, and
sive cognitive decline that interferes with normal collapse when exposed to neuroleptic agents. That
social or occupational functioning. Memory and these reactions can be irreversible and sometimes
visuospatial deficits are often, but not necessarily, fatal11,12 has important implications for the man-
prominent. In addition, two of the following three agement of psychotic symptoms in these patients.
features must also be present: recurrent visual hal- Other strategies for managing these symptoms
lucinations6 (which are typically well detailed), should be exhausted before resorting to medica-
fluctuating cognition, and spontaneous motor fea- tions; when neuroleptics are necessary, it might be
tures of parkinsonism. Supportive of the diagnosis wise to consider admission to the hospital for dose
are repeated falls, syncope, transient loss of con- titration. There is some debate in the literature13,14
sciousness, systematized delusions, neuroleptic sen- about whether atypical neuroleptics, such as ris-
sitivity, and hallucinations in other sensory modal- peridone and quetiapine, might provide useful an-
ities. The absence of stroke, focal neurological tipsychotic effects in Lewy body dementia without
signs, and delirium makes the diagnosis of Lewy causing substantial extrapyramidal side effects, but
body dementia more likely. this possibility has not been thoroughly investi-
These clinical criteria, when compared with the gated.
reference standard of autopsy, have been found to Despite the parkinsonian features, many patients
be highly specific (specificity 79%–100%) but to with Lewy body dementia exhibit limited clinical
lack sensitivity (sensitivity 22%–75%.),7 implying response to l-dopa. Further, it should be noted that
that these criteria are useful for confirming the l-dopa can cause or exacerbate visual hallucina-
diagnosis but cannot be relied on for screening. tions, which tend to occur in these patients. For
This limitation of the clinical criteria is due at least patients with Lewy body dementia, therefore, the
to the following two factors. First, there is consid- risks of treatment with l-dopa will frequently out-
erable overlap between the features of Lewy body weigh the benefits. A possible explanation for the
dementia and those of other dementias. Second, limited response to l-dopa lies in the finding that,
the characteristic or diagnostic features of Lewy compared with patients with Parkinson disease,
body dementia frequently do not become manifest those with Lewy body dementia have a lower den-
until a late stage in the disease. Given the high sity of dopamine D2 receptors in the corpus stria-
prevalence of Lewy body dementia and its unique tum.15 This finding might also explain the in-
implications for management, the prudent primary creased sensitivity to neuroleptics, which are
care physician must be particularly alert for this dopamine antagonists.
diagnosis. As for any dementia, a thorough history A few studies have examined the effects of cho-
(including interviews with family members and linesterase inhibitors (including donepezil and riv-
caregivers) and physical, neurological, and cogni- astigmine) in patients with Lewy body dementia.
tive examinations are essential. The main outcomes studied were hallucinations,
Although there is considerable interest in the behavior, and cognition. In two case series16,17
role of functional imaging techniques designed to where donepezil was used for 8 to 24 weeks at doses
distinguish Lewy body dementia form Alzheimer of 5 to 10 mg, there was a decrease in the frequency
disease, these techniques are not available for rou- and duration of hallucinations. One of these se-
tine use. Essentially, radioisotope scans reflect ei- ries17 also found an improvement in cognition
ther regional blood flow,8 regional glucose metab- (mean increase in MMSE score of 4.4 in 7 of 9

52 JABFP January–February 2002 Vol. 15 No. 1

patients). The response to donepezil observed in sharing it with other primary care physicians, we
our patient was thus consistent with previously re- hope that we can help them to be more cognizant
ported findings but was rather more dramatic than of Lewy body dementia as an important clinical
would typically be expected. Studies of rivastigmine entity.
include an open trial18 and a randomized double-
blind placebo-controlled study.19 Doses ranged References
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delusions, improved behavior, and in the latter and neuropathologically distinct form of Lewy body
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body variant of Alzheimer’s disease: a clinical and
linesterase inhibitors might have an important role
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3. Folstein MF, Folstein SE, McHugh PR. ‘Mini-Men-
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who had visual hallucinations and long-standing guidelines for the clinical and pathologic diagnosis of
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consortium on DLB international workshop. Neu-
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The patient responded extremely well to the treat-
Dementia with Lewy Bodies. Neurology 1999;53:
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Lewy Body Dementia 53

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54 JABFP January–February 2002 Vol. 15 No. 1

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