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Diagnosis of Dementia

This document summarizes guidelines for diagnosing dementia. It outlines that diagnosis involves differentiating dementia from similar conditions like depression and delirium. A detailed history and exam are important to identify disease-specific symptoms and the underlying cause. Biomarkers can help but are not always available. Alzheimer's disease accounts for about half of cases so proper diagnosis is key. The document describes diagnostic steps and differential diagnosis of dementia types as well as potentially reversible causes that may be treated.

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0% found this document useful (0 votes)
91 views5 pages

Diagnosis of Dementia

This document summarizes guidelines for diagnosing dementia. It outlines that diagnosis involves differentiating dementia from similar conditions like depression and delirium. A detailed history and exam are important to identify disease-specific symptoms and the underlying cause. Biomarkers can help but are not always available. Alzheimer's disease accounts for about half of cases so proper diagnosis is key. The document describes diagnostic steps and differential diagnosis of dementia types as well as potentially reversible causes that may be treated.

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Dio Patterson
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Research and Reviews

Diagnosis of Dementia
JMAJ 56(4): 235–239, 2013

Manabu IKEDA*1

Abstract
Currently, there are a few reliable biomarkers for identifying the main underlying causes of dementia and it is
very difficult to use them in daily clinical work. Therefore, it is very important to identify disease-specific symptoms
from a detailed history taking and medical examination of the patient after correctly understanding the pathology
of each disease in order to make a comprehensive diagnosis with the help of imaging tests and other modalities.
In this paper, the differential diagnoses of dementia and similar conditions, in particular senile depression and
delirium, are described first, followed by the diagnostic steps on the basis of symptomatology and outlined the
underlying causes of dementia. Subsequently, the symptomatology of Alzheimer’s disease (AD) is discussed
in detail, as correct diagnosis of AD is the basis of dementia diagnosis and AD accounts for approximately
half of all dementia cases. As for causes of dementia other than AD, important points for differential diagnosis,
particularly with regard to AD, are also mentioned.

Key words Symptomatology, Dementia, Depression, Delirium

Introduction outlined. Because AD accounts for approximately


half of all dementia cases and its proper diagnosis
Owing to an increased interest in society in de- is the basis of dementia diagnosis, important
mentia and advancement in treatment methods, points for differential diagnosis particularly with
there is an increasing need to accurately diagnose regard to AD are also briefly described for causes
dementia at an early stage of the disease more of dementia other than AD.
than ever. Furthermore, different disease-specific
treatment and caring strategies are recommended Diagnosis of Dementia
for different causes of dementia, increasing the
importance of correctly diagnosing different types Prior to identification of the underlying cause of
of dementia in the field of long-term care as dementia, the first necessary step is to ascertain
well as medicine. However, only a few reliable if the elderly patient suspected of dementia who
biomarkers currently exist for identifying the visits a physician for examination for the first time
main underlying causes of dementia, including does, in fact, have dementia. This step involves
Alzheimer’s disease (AD). Therefore, it is very differentiating dementia from other conditions
important to identify disease-specific symptoms that are similar to dementia.
from a detailed history taking and medical exami­ Patients with dementia have significantly re-
nation of the patient after correctly understanding duced awareness of conditions, such as memory
the pathology of each disease in order to make a impairment, i.e., low insight into the disease.
comprehensive diagnosis with the help of imaging Therefore, the patient very rarely visits a phy­
tests and other modalities. sician for consultation or examination alone,
In this paper, the diagnostic steps for dementia except during the very early stages of the disease.
developed on the basis of symptomatology are On most visits, the patient will be accompanied by

*1 Professor, Department of Neuropsychiatry, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan ([email protected]).
This article is a revised English version of a paper originally published in the Journal of the Japan Medical Association (Vol. 141, No. 3, 2012,
pages 523–527).

JMAJ, July / August 2013 — Vol. 56, No. 4  235


Ikeda M

a family member. The characteristic of memory trend of circadian rhythm reversal, or prescrip-
impairment as observed in dementia (especially tion drug use (e.g., anti-anxiety drugs, antide­
in AD) is to forget an event itself. On the other pressants, and diuretics), should be considered.
hand, a person who is forgetful due to normal When drug-induced delirium is suspected, always
aging has sufficient awareness and often visits a check if more than one medical institution is
physician alone. The characteristics of memory prescribing similar drugs or if the patient is taking
impairment include inability to remember peo- the medication as directed by the prescribing
ple’s names or dates on the spot. When a patient physicians. When drug-induced delirium due to
visits a physician for the diagnosis or treatment benzodiazepines, tricyclic depressants, β-blocker,
of dementia or “forgetfulness,” the physician H2 blocker, furosemide, or solifenacin succinate
should first ask the patient to state the purpose is suspected, immediately consider switching to
of his/her visit in order to estimate the gap an alternate drug with a different mechanism of
between the patient’s words and those of his/her action.
caregiver, so that the physician can confirm the Dementia can be accompanied by delirium.
patient’s awareness about having dementia or Hence, the diagnosis must be made carefully. The
memory impairment. priority is to treat the delirium; once the patient’s
delirium improves, the presence of dementia
Differential diagnosis of senile depression should be verified.
Senile depression is often accompanied by cog­
nitive dysfunction. In many cases, complaint of Differential Diagnosis of Dementia and
sadness is not evident; however, hypochon­ Disease-Specific Treatment
driasis and somatic concern such as insomnia,
stiff shoulders, and general malaise are obvious. The next step involves the differential diag­
Consequently, senile depression is frequently nosis of the underlying causes of dementia and
misdiagnosed as an early stage of dementia. the implementation of disease-specific treatment
In the revised Hasegawa’s dementia scale (HDS- and care.
R) or mini-mental state examination (MMSE),
patients with senile depression often have scores Dementia with a possibility of radical
similar to those obtained by patients in the early ­treatment (reversible causes of dementia)
stages of dementia; therefore, the total score of First, a physician must examine the possibility of
such assessment scales cannot be used to differ- radical treatment for a given case of dementia.
entiate between depression and dementia. Radical treatment is available in approximately
A patient’s awareness of his/her disease is 10–20% of all dementia cases, but the dementia
another clue for differentiating between depres- in these cases may have many different under­
sion and dementia. A patient with depression lying causes. One difference in dementia caused
often complains of his/her forgetfulness in an by degenerative diseases such as AD (which will
exaggerated manner rather than objectively stat- be discussed next) is its rapid progression. If
ing his/her self-assessment. It is also important a symptom is rapidly deteriorating over a few
to confirm if the patient has a past history of months, a physician should first suspect poten-
­depression or manic states. However, diagnosing tially reversible causes of dementia.
the early stages of dementia is challenging even A head CT or MRI is essential in the diag­
for a specialist because it is often accompanied nosis of neurosurgical diseases such as chronic
by a state of depression. When in doubt, a phy­ subdural hematoma, normal pressure hydroceph-
sician should consider referring the patient to a alus, or brain tumor. Chronic subdural hematoma
specialist. is strongly suspected if the patient has had a head
contusion from falling, which is often undetect-
Differential diagnosis of delirium able during examination, and has experienced a
Delirium should be first suspected if the patient rapidly progressive decline of cognitive function
shows a change in the level of consciousness or over a period of a few weeks to a few months.
cognitive functions, or experiences visual halluci- If a hematoma is applying pressure to the brain,
nation/illusion. The possibility of an underlying the hematoma must be surgically removed imme­
condition, including electrolyte abnormalities, a diately. In cases of idiopathic normal pressure

236  JMAJ, July / August 2013 — Vol. 56, No. 4


Diagnosis  of  Dementia

hydrocephalus, a patient often shows the 3 clas- through symptoms such as repeated forgetting
sic characteristics of gait disturbance, urinary of appointments, inability to remember where
incontinence, and memory impairment, and the personal belongings (e.g., wallet or a pair of
coronal image from MRI shows narrowing of glasses) are kept, or repetition of the same con-
the subarachnoid cavity in the higher part of the versation. A physician can verify such memory
fornix and the median as well as dilatation of impairment during an examination if the patient
the sylvian fissure. cannot remember the name of their attending
Even if there is no underlying disease of the physician 5 min after being asked to recollect it,
brain, it is important to address the possibility of or if the patient cannot remember what he/she
social withdrawal due to environmental changes had for dinner the previous night.
or disuse syndrome, which can emerge from As the degenerative process of AD advances,
prolonged mild physical disease such as common the manifestation of other cognitive dysfunc-
cold or lower back pain. In modern Japanese tions, such as visual/constructional impairment,
society, many elderly people live alone, and there language disorder, dyscalculia, dysgraphia, which
are families in which the elderly are left alone are the symptoms of the parietotemporal lobe
at home during the daytime. A small trigger disorder, follows memory impairment. Visual/
such as a mild illness can lead to reduced mental constructional impairment is a disturbance in
functions (e.g., lack of motivation, lowered con- visuospatial ability, which can be examined by ask-
centration or attention) and a decline in physical ing the patient to replicate figures or imitate finger
functions (e.g., reduced stamina, fatigability, patterns. Another common symptom observed
dizziness on standing up). If left untreated, the in the relatively early stages of dementia is a
elderly may fall into a vicious circle of “do disturbance in executive functions, i.e., the ability
not want to do much” to “do not want to do to properly respond to problems and challenges
anything” and “too weak to do anything,” and that arise in various everyday situations and
eventually suffer from dementia or become bed- to solve them. The patient also begins to have
ridden. Sufficient attention must be paid to such difficulty completing complicated tasks such as
patients as dementia caused by AD or vascular work and cash management, cooking, and bank
dementia (VaD) (which will be discussed later in transactions.
this paper) can accompany such disuse syndrome Psychiatric symptoms and behavioral disorders
and will aggravate the symptoms further. Psychiatric symptoms and behavioral disorders
The best course of treatment is to break this have been previously described as peripheral
vicious circle, that is, to take advantage of the symptoms of dementia in Japan. Now, they are
national long-term care insurance and use the referred to as behavioral and psychological symp-
daytime care/nursing service or rehabilitation toms of dementia (BPSD), and their treatment
service in a short and intensive manner for and management is gaining attention.
improving the activity level. In such cases, the In AD, apathy (reduced spontaneity and lack
primary physician must inform the person in of interest) is believed to emerge in 70–80% of
charge of the care or rehabilitation program of patients during the early stages of the disease,
the patient’s reason for using the service. and they are considered to be the most common
psychiatric symptoms. When a patient develops
Alzheimer’s disease apathy, he/she often shows reduced spontaneity
Cognitive dysfunction and motivation as well as a lack of interest and
The degenerative process of AD normally begins concern for the surroundings; his/her own low
in the medial regions of the temporal lobe such levels of spontaneity and motivation do not appear
as the hippocampus and parahippocampal gyrus, to afflict or concern the patient. Depression,
and subsequently spreads to the parietal and the a mood disorder, is another symptom that is
temporal association area. Due to these lesions, ­commonly exhibited during the early stages of
the cognitive dysfunction caused by AD that the disease. Delusion is also commonly exhibited
manifests the earliest is the impairment of recent in the early stages of the disease; the most
memory, which becomes the core symptom of common delusion is to imagine oneself as a theft
AD. People in association with the patient in victim (delusions of theft). On the other hand,
his/her everyday life commonly notice the change the frequency of hallucinations is much lower

JMAJ, July / August 2013 — Vol. 56, No. 4  237


Ikeda M

than that of delusion. to that in AD. On the other hand, visual percep-
Wandering behavior, agitation, and irritability tion and attentional function in DLB are more
become evident in the middle and later stages of extensively impaired as compared to that in AD.
AD, and the patient often shows hyperactivity, Fluctuating cognition with pronounced varia-
restlessness, and repetitive behaviors of walking tions in attention and alertness, is one of the
back and forth and opening and closing a drawer. core features of DLB. Some fluctuations are
Neurological symptoms observed within a day or from day to day, but
In AD, focal neurological symptoms are rarely some may be long-term fluctuations over a span
observed in the early stages except in some cases of months. The family would report that the
of familial AD. The presence of parkinsonism ­patient “is sometimes completely normal, but
must be examined by signs of solidification in the at other times is so ill, it is as if it is a different
wrist, which is also helpful in differentiating it person.” A physician may observe a fluctuation
from other diseases such as dementia with Lewy in the patient’s behaviors during the exami­
bodies (DLB) or corticobasal degeneration. In nation, where the patient “is talking normally
the progressive stage of AD, the patient exhibits but suddenly becomes absentminded and cannot
neurological symptoms such as parkinsonism or continue a conversation.”
myoclonus. Recurrent visual hallucinations are another
Vascular dementia core feature of DLB. The visual hallucinations
The clinical conditions of vascular dementia often involve people, animals, or insects, and
(VaD) are variable, reflecting the sites of vascular the contents are well formed and detailed, for
lesions and pathological heterogeneity. The most example, “there are 3 people cleaning the rest-
common condition accounting for more than half room, but they won’t answer me when I talk to
of all cases is subcortical VaD, primarily con­ them.” Visual illusions (visual misjudgment) are
sisting of lacunar infarction and ischemic white also frequently observed, such as mistaking a long
matter lesions; its clinical conditions as well as rope for a snake or a cloth hanging on a hanger
pathology are relatively homogeneous. The onset for a person. The patient often remembers expe-
of VaD can be acute, but in many cases, it is latent riencing visual hallucinations/illusions, and this
and the progress is slow. Therefore, differential becomes a very important clue in differentiating
diagnosis with degenerative dementia is required. between DLB and delirium.
Gait disturbance, falling easily, urinary incon- In DLB, reportedly delusions are more vari-
tinence, pseudobulbar paralysis, and apathy are able and occur at a higher frequency than that in
relatively common symptoms in the relatively AD. Unlike patients with AD, a patient with DLB
early stage of VaD. These symptoms are helpful often misidentifies a person or place, claiming,
in differentiating VaD from AD, which shows for example, that “she (who is truly his wife)
no neurological abnormalities during the early looks identical to my wife but is a different person
stages of the disease. impersonating my wife (Capgras syndrome).”
Executive dysfunction and attentional dys- The visual hallucinations-induced delusion also
function are the main characteristics of VaD commonly occurs in DLB.
in terms of cognitive dysfunction. The memory Depressed states are observed at a high fre-
impairment in VaD is believed to be milder than quency. It has been pointed out that depression
that in AD. appears as a precursor of DLB, and that the fre-
The main psychiatric symptoms are emotional quency of transition from refractory depression
disorders such as emotional lability, depressed into DLB in the elderly is also high.
state, apathy, anxiety, irritability, hypochondriasis, REM sleep-related behavioral disorders, in-
and psychomotor slowing. Patients with VaD cluding shouting out loud or moving the body
reportedly show a more pronounced degree of to a dream, also appear often. When suspecting
slowed thinking and movements, depressed states, DLB, the physician should always ask the family
and anxiety than patients with AD. members if the patient is talking or moving his/
Dementia with Lewy bodies her body while sleeping.
Progressive cognitive impairment is the main Parkinsonism is also one of the core features
feature of DLB, and the degree of memory of DLB, and its incidence rate is very high as
impairment is believed to be mild as compared compared to those of AD and VaD. While akinesia

238  JMAJ, July / August 2013 — Vol. 56, No. 4


Diagnosis  of  Dementia

and muscle rigidity are observed at a high rate, also common. The patient does not mean any
the frequency of tremors is believed to be low. offense and rarely reflects on his/her conduct. The
Additionally, the use of antipsychotic drugs in patient is unconcerned when someone points out
small amounts possibly aggravates parkinsonism his/her misconduct and repeats the same mistake
or leads to the disturbance of consciousness or a many times.
malignant syndrome. Various autonomic symp- Stereotyped behavior is repeating organized
toms are observed frequently, particularly consti- or systematic behaviors. At home, it is common
pation, frequent urination, urinary incontinence, for the patient to eat the same food day after day
and orthostatic hypotension. (stereotyped eating) or to continue to stroll the
Frontotemporal lobar degeneration same route for several kilometers (stereotyped
Frontotemporal lobar degeneration (FTLD) is walking). When stereotyped behavior develops
characterized by significant personality changes, on a certain time schedule, the day of the patient
behavioral disorder, and language disorder as starts to resemble a time-table. The presence
the main features, and comprehensively refers of such stereotyped behavior is useful in the
to degenerative dementia with the main lesion ­differential diagnosis of FTLD from AD.
in the anterior region of the cerebrum, namely, Some types of eating behavior abnormalities
the frontal and frontotemporal lobes. Memory are observed frequently, which serves as an
impairment, visuospatial cognitive impairment, important clue for differentiating between FTLD
hallucination, and delusion are not notable. AD and AD along with other stereotyped behaviors.
with outstanding BPSD is sometimes misdiag- Binge eating and changes in taste preferences are
nosed as FTLD or frontotemporal dementia typical. The patient also starts to favor heavily
(FTD), and it is important to check for these seasoned dishes, sweets, and sugary juices.
symptoms, as they are not observed in the early
stages of FTLD. In this paper, only the major Conclusion
symptoms of FTD, whose main lesion is in the
frontal lobe, are introduced. In the past, donepezil was the only drug approved
Compared with patients with other types of for treating AD in Japan. Since 2011, however, 3
dementia, patients with FTLD lack insight into additional drugs have been approved. Disease-
the disease from the early stages. The patient is modified treatment for AD is becoming a real­
not aware of his/her own symptoms, and has no ity, and the significance of correctly diagnosing
interest in them. Therefore, it is highly unlikely dementia including AD at the early stages is
that a FTD patient willingly visits a physician increasing. The total number of cases of dementia,
for consultation. including mild cases, is said to be over 4 million
Socially inappropriate behavior is apparent in Japan. From a practical viewpoint and the
from the early stages. Ill-mannered behaviors standpoint of health economics, it is already
such as bursting into laughter in the middle of ­impossible to conduct functional imaging tests
a funeral or beginning to sing during a medical such as SPECT or PET or a cerebrospinal fluid
examination, problematic behaviors that ignore test for all cases. The importance of dementia
social rules such as cutting in line, or minor diagnosis and care based on the symptomatology
­offenses such as urinating at the roadside, food will continue to grow further in the future.
theft, shoplifting, and ignoring traffic rules are

Bibliography

1. Ikeda M, ed. Dementia: The Clinical Forefront. Tokyo: Ishiyaku rated by a Specialist. Tokyo: Chuokoron-Shinsha Inc; 2010. (in
Publications Inc; 2012. (in Japanese) Japanese)
2. Ikeda M. Dementia: Diagnosis, Treatment, and Care, as Nar­

JMAJ, July / August 2013 — Vol. 56, No. 4  239

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