Early Diagnosis of Dementia

Page created by Maria Webster
 
CONTINUE READING
Early Diagnosis of Dementia
KAREN S. SANTACRUZ, M.D., and DANIEL SWAGERTY, M.D., M.P.H.
University of Kansas Medical Center, Kansas City, Kansas

Until recently, the most significant issue facing a family physician regarding the diag-
nosis and treatment of dementia was ruling out delirium and potentially treatable eti-                      O A patient informa-
ologies. However, as more treatment options become available, it will become increas-                       tion handout on
ingly important to diagnose dementia early. Dementia may be suspected if memory                             dementia, provided by
                                                                                                            an AAFP staff patient
deficits are exhibited during the medical history and physical examination. Informa-                        education writer, is
tion from the patient’s family members, friends and caregivers may also point to signs                      presented on page
of dementia. Distinguishing among age-related cognitive decline, mild cognitive                             717.
impairment and Alzheimer’s disease may be difficult and requires evaluation of cogni-
tive and functional status. Careful medical evaluation to exclude treatable causes of
cognitive impairment is important. Patients with early dementia may benefit from for-
mal neuropsychologic testing to aid in medical and social decision-making. Follow-up
by the patient’s family physician is appropriate in most patients. However, a subspe-
cialist may be helpful in the diagnosis and management of patients with dementia
with an unusual presentation or following an atypical course. (Am Fam Physician 2001;
63:703-13,717-8.)

                         T
   See editorial                        he prevalence of dementia is             posed risk factors for dementia include a
   on page 620.                         expected to increase dramati-            family history of dementia, previous head
                                        cally in future years as life ex-        injury, lower educational level and female
                                        pectancy continues to increase           sex.2 Alzheimer’s disease is the most com-
                                        and the baby-boomer popula-              mon cause of dementia; many of the remain-
                         tion ages. The cumulative incidence of                  ing cases of dementia are caused by vascular
                         Alzheimer’s disease has been estimated to be            disease and Lewy body disease. Vascular dis-
                         as high as 4.7 percent by age 70, 18.2 percent          ease and Lewy body disease often occur in
                         by age 80 and 49.6 percent by age 90.1 Pro-             combination with Alzheimer’s disease.3,4

                         TABLE 1
                         Signs and Symptoms That May Indicate the Need for Evaluation for Dementia

                         Cognitive changes                                       Personality changes
                         New forgetfulness, more trouble understanding           Inappropriate friendliness, blunting and disinterest,
                          spoken and written communication, difficulty             social withdrawal, excessive flirtatiousness, easy
                          finding words, not knowing common facts such             frustration, explosive spells
                          as the name of the current U.S. president,             Problem behaviors
                          disorientation                                         Wandering, agitation, noisiness, restlessness, being
                         Psychiatric symptoms                                      out of bed at night
                         Withdrawal or apathy, depression, suspiciousness,       Changes in day-to-day functioning
                          anxiety, insomnia, fearfulness, paranoia, abnormal     Difficulty driving, getting lost, forgetting recipes
                          beliefs, hallucinations                                  when cooking, neglecting self-care, neglecting
                                                                                   household chores, difficulty handling money,
                                                                                   making mistakes at work, trouble with shopping

                         Reprinted with permission from Rabins PV, Lyketsos CG, Steele CD. Practical dementia care. New York: Oxford
                         University Press, 1999:23.

FEBRUARY 15, 2001 / VOLUME 63, NUMBER 4                   www.aafp.org/afp                       AMERICAN FAMILY PHYSICIAN        703
diagnostic features are constant. They are well
                        Clinical Presentation                               described in the Diagnostic and Statistical
                          A practical approach to the diagnosis of          Manual of Mental Disorders, 4th ed. (DSM-
                        dementia begins with the clinical recognition       IV) and summarized in Table 2.6
                        of a progressive decline in memory, a decrease
                        in the patient’s ability to perform activities of   HISTORY
                        daily living, psychiatric problems, personality       The early diagnosis of dementia requires
                        changes and problem behaviors (Table 1).5           careful questioning to elicit clues to the pres-
                        While the clinical presentation of dementia         ence of functional and cognitive impairment
                        may vary, depending on the etiology, the            (Table 3).5 Interviewing friends as well as

                        TABLE 2
                        Criteria for the Diagnosis of Alzheimer’s Type Dementia
                        and Age-Related Cognitive Decline

                                                   The rightsholder did not
                                                   grant rights to reproduce
                                                   this item in electronic
                                                   media. For the missing
                                                   item, see the original print
                                                   version of this publication.

704   AMERICAN FAMILY PHYSICIAN                     www.aafp.org/afp              VOLUME 63, NUMBER 4 / FEBRUARY 15, 2001
Early Dementia

family members is helpful, because family
members may have adopted coping strategies          Testing for a variety of cognitive abnormalities, including
to help the patient with dementia, which
                                                    aphasia, apraxia, agnosia and executive functioning, may be
sometimes conceal the patient’s impairment,
making early diagnosis difficult. For example,      useful in the evaluation of early dementia.
a caregiver may take on additional responsi-
bilities such as shopping and financial man-
agement, possibly masking the patient’s level     evaluating cognitive function. Their use may
of impairment.                                    or may not be required in the evaluation of
   During the medical history-taking, ques-       early dementia.
tions should be asked about forgetfulness
and orientation. Inquiries should also be         PHYSICAL EXAMINATION AND COGNITIVE TESTING
made regarding activities of daily living,           The findings of the physical examination
including instrumental activities such as         may suggest an etiology for dementia. For
everyday problem solving and handling of          example, dementia resulting from vascular
business and financial affairs. Independent       disease may be accompanied by focal neuro-
functioning in community affairs, such as         logic findings.
job responsibilities, shopping and participa-        Physical examination should include
tion in volunteer and social groups, should       assessment of cognitive domains, including
be assessed. Evidence of problems with            speech (aphasia), motor memory (apraxia),
home activities, hobbies and personal care        sensory recognition (agnosia) and complex
should also be sought. In the early stages of     behavior sequencing (executive function-
dementia, the patient may show restricted         ing). Aphasia may be detected by asking the
interest in hobbies and other activities, and     patient to name body parts or objects in the
may require prompting to maintain per-            room. Frequent use of vague terms such as
sonal hygiene. 7                                  “thing” and “it” may also signify deteriora-
   A variety of rating scales are available for   tion of language function. An example of a

TABLE 3
Symptom Checklist in the Evaluation of Dementia

                                          The rightsholder did not
                                          grant rights to reproduce
                                          this item in electronic
                                          media. For the missing
                                          item, see the original print
                                          version of this publication.

FEBRUARY 15, 2001 / VOLUME 63, NUMBER 4               www.aafp.org/afp                 AMERICAN FAMILY PHYSICIAN   705
test for apraxia is to ask the patient to pan-           his or her eyes and then placing an object,
                        tomime the use of a common object such as                such as a key or a coin, in the patient’s hand
                        a hammer or a toothbrush. Agnosia can be                 and asking the patient to identify it without
                        evaluated by first asking the patient to close           looking at it. Inability to recognize a com-

                                                          Mini-Mental State Examination
                        Maximum
                        score         Score

                                                 Orientation
                        5             _____      What is the (year) (season) (date) (day) (month)?
                        5             _____      Where are we: (state) (county) (town or city) (hospital) (floor)?
                                                 Registration
                        3             _____      Name three common objects (e.g., “apple,” “table,” “penny”):
                                                   Take one second to say each. Then ask the patient to repeat all three after you
                                                   have said them. Give one point for each correct answer. Then repeat them until
                                                   he or she learns all three. Count trials and record.
                                                 Trials: ____
                                                 Attention and calculation
                        5             _____      Spell “world” backwards. The score is the number of letters in correct order.
                                                   (D___L___R___O___W___)
                                                 Recall
                        3             _____      Ask for the three objects repeated above. Give one point for each correct answer.
                                                   (Note: recall cannot be tested if all three objects were not remembered during
                                                   registration.)
                                                 Language
                        2             _____      Name a “pencil” and “watch.”
                                                 Repeat the following: “No ifs, ands or buts.”
                        1             _____      Follow a three-stage command:
                        3             _____      “Take a paper in your right hand, fold it in half and put it on the floor.”
                                                 Read and obey the following:
                        1             _____      Close your eyes.
                        1             _____      Write a sentence.
                        1             _____      Copy the following design.

                              Total
                              score: _____

                        FIGURE 1. The Mini-Mental State Examination, a useful tool for assessing cognitive function and
                        documenting subsequent decline. Scores of 24 or higher are generally considered normal; see
                        Table 4 for education and age norms.
                        Adapted with permission from Folstein MF, Folstein SE, McHugh PR. “Mini-mental state”: a practical method
                        for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:196-8, and Cockrell JR,
                        Folstein MF. Mini-mental state examination (MMSE). Psychopharm Bull 1988;24(4):689-92.

706   AMERICAN FAMILY PHYSICIAN                       www.aafp.org/afp                  VOLUME 63, NUMBER 4 / FEBRUARY 15, 2001
Early Dementia

mon object despite normal sensory function
signifies agnosia.                                     Serial Mini-Mental State examinations (or other cognitive
   Asking the patient to perform a series of
                                                       testing) can help document changes over time.
simple tasks is a way to evaluate executive
functioning. For example, the patient can be
asked to put a piece of paper in his or her
right hand, fold it in half and put it on the       unnecessary medications and optimize treat-
floor. This task would be difficult for a patient   ment of chronic diseases, physical examina-
with impairment in the ability to plan, initi-      tion and laboratory tests are recommended to
ate, sequence and monitor complex behavior.         rule out specific treatable causes of dementia.
Asking the patient to perform serial subtrac-       Hearing or vision deficits, hypothyroidism,
tion of 7s (backward from 100 to 65), to spell      vitamin B12 deficiency and depression are
the word “world” backward and to produce            among the disorders that can cause symptoms
verbal word lists, such as names of animals or      of dementia. Such disorders are relatively easy
items in a grocery store, are other ways to test    to detect and should be excluded by appropri-
executive functioning and abstract thinking.        ate laboratory tests, physical examination and
   Although the Mini-Mental State Examina-          psychologic tests. Electrocardiography and
tion (MMSE) is not diagnostic of dementia           chest radiography can sometimes be useful to
and does not distinguish well between vari-         rule out treatable systemic diseases, although
ous confusional states,8 it is useful for assess-
ing cognitive function and documenting
subsequent decline (Figure 1). Because judg-        TABLE 4
ment and insight are not tested by the              Median Scores on Mini-Mental State Examination
MMSE, many clinicians ask additional ques-          by Age and Educational Level
tions to assess these aspects of cognition.
Judgment and insight can be assessed, for                             Educational level
example, by asking the patient, “What would
you do if you were in a crowded building and        Age (years)       4th grade       8th grade     High school    College
smelled smoke?”                                     18 to   24        22              27            29             29
   When conversational skills are well pre-         25 to   29        25              27            29             29
served, an early decline in memory may be           30 to   34        25              26            29             29
difficult to detect, especially during a short,     35 to   39        23              26            28             29
focused office visit. The MMSE can detect           40 to   44        23              27            28             29
cognitive impairment by evaluating orienta-         45 to   49        23              26            28             29
tion, attention, recall, language and ability to    50 to   54        23              27            28             29
follow commands. A score higher than 23 is          55 to   59        23              26            28             29
generally considered normal, although per-          60 to   64        23              26            28             29
formance varies with the patient’s age and          65 to   69        22              26            28             29
education (Table 4).9                               70 to   74        22              25            27             28
                                                    75 to   79        21              25            27             28
Differential Diagnosis                              80 to   84        20              25            25             27
  Figure 2 summarizes an approach to the            > 84              19              23            26             27
early diagnosis of dementia. If dementia is
suspected, a medication review and assess-          Reprinted with permission from Crum RM, Anthony JC, Bassett SS, Folstein MF.
ment for chronic disease processes are war-         Population-based norms for the mini-mental state examination by age and edu-
ranted. If no improvement occurs after appro-       cational level. JAMA 1993;18:2386-91.
priate measures are taken to eliminate

FEBRUARY 15, 2001 / VOLUME 63, NUMBER 4                  www.aafp.org/afp                     AMERICAN FAMILY PHYSICIAN      707
Diagnostic Work-Up of Dementia

                               Presence of symptoms suggestive of dementia, including
                                 changes in activities of daily living or caregivers’ concerns

                        Perform clinical assessment, including history, physical examina-
                          tion, functional status and cognitive testing such as MMSE.

                                         Deficits detected on clinical assessment

                            Perform assessment for treatable causes of dementia, including
                              medication review, depression screening and laboratory testing.

                                                                                          Yes
                                       Any treatable abnormalities present?                               Treat abnormalities and reassess.

                                                               No
                                                                                                 Do symptoms of possible dementia remain?

                                               Do the findings meet criteria
                                                for diagnosis of dementia?                        Yes
                                                                                                                       No

                                                                                                              Provide reassurance.
                                 No                                                       Yes

         Is mild cognitive impairment present?                    Are atypical features of dementia present?

               No                               Yes                            No                       Yes

  Provide reassurance;          Consider referral to a            Diagnose Alzheimer’s       Consider referral to a
    reassess in 6 months.        subspecialist and/or              disease, vascular          subspecialist; provide
                                 neuropsychologic testing;         dementia, etc.             close follow-up.
                                 reassess in 6 months.

                                                                       Provide counseling about expected
                                                                         course and treatment options.

FIGURE 2. An approach to the early diagnosis of dementia. (MMSE = Mini-Mental State Examination)

                             their necessity should be guided by the history              virus (HIV) status, urine check for heavy met-
                             and physical examination.                                    als and toxicology screening may be indicated
                                Table 5 lists laboratory tests to consider in             in a minority of cases (Table 6).5
                             the evaluation of dementia. Tests recom-                        Lumbar puncture is usually not necessary
                             mended for the diagnostic work-up of                         except when the onset of dementia occurs
                             dementia include a complete blood cell count                 before 55 years of age or when a specific con-
                             (to exclude anemia and infection), urinalysis                dition such as infection, syphilis or vasculitis
                             (to exclude infection), serum electrolyte, glu-              is suspected.10 However, in at least one
                             cose and calcium levels, blood urea nitrogen,                prospective study it was found that cere-
                             serum creatinine level and liver function tests              brospinal fluid analysis for the 42 amino acid
                             (to investigate metabolic disease).10 Syphilis               form of -amyloid may be suggestive of
                             serology, erythrocyte sedimentation rate,                    Alzheimer’s dementia, although not diagnos-
                             serum folate level, human immunodeficiency                   tic.11 Further studies into the existence of bio-

708   AMERICAN FAMILY PHYSICIAN                              www.aafp.org/afp                     VOLUME 63, NUMBER 4 / FEBRUARY 15, 2001
Early Dementia

markers for the diagnosis of early Alzheimer’s
disease are ongoing.                                    TABLE 5
   The utility of computed tomography or                Laboratory Tests for Evaluation of Dementia
magnetic resonance imaging to rule out vas-
cular disease, tumor, subdural hematoma or              Urinalysis and microscopy                      Erythrocyte sedimentation rate*
normal-pressure hydrocephalus remains con-              Complete blood cell count                      Serologic tests for syphilis (or similar)
                                                        Serum electrolyte levels, including            Chest radiography*
troversial. Radiologic imaging of the central
                                                          magnesium                                    Electrocardiography*
nervous system is probably not necessary in             Serum chemistry panel, including liver         Toxicology screening*
patients presenting with dementia, unless                 function tests                                 Urine toxicology
localizing neurologic signs or symptoms are             Thyroid function tests                           Serum toxicology (alcohol,
noted. Clearly, it is important to search for a         Serum vitamin B12                                  salicylates, other)
reversible cause of dementia. However, in one
meta-analysis it was revealed that fewer than           *—To be considered, not universally needed.
11 percent of patients with cognitive decline           Reprinted with permission from Rabins PV, Lyketsos CG, Steele CD. Practical
had partially or fully reversible disease.12            dementia care. New York: Oxford University Press, 1999:46.

Diagnosis
DEMENTIA
   DSM-IV criteria for the diagnosis of demen-           criteria for dementia. In order to fulfill DSM-
tia require the presence of multiple cognitive           IV criteria, cognitive impairment must be of
deficits in addition to memory impairment6               the degree that social or occupational function
(Table 1). Early in the disease, memory impair-          is reduced, with the functional impairment
ment may be the only clinical finding, and this          representing a decrease in the patient’s normal
single finding would not meet the diagnostic             ability.

TABLE 6
Additional Tests to Consider in the Diagnostic Work-Up of Dementia

Test                              Indication

Electroencephalography            Possible seizures; Creutzfeldt-Jakob disease
Lumbar puncture                   Onset of dementia within the preceding six months; dementia rapidly
                                   progressive
Heavy metal screen                History of potential exposure
Human immunodeficiency virus      History of potential exposure
Lyme disease titer                History of exposure and compatible clinical picture
Ceruloplasmin, arylsulfatase,     Wilson’s disease, metachromatic leukodystrophy, multiple myeloma
 electrophoresis
Slit lamp examination             History and examination suggest Wilson’s disease
Apolipoprotein E                  Need to increase likelihood that diagnosis of Alzheimer’s disease is correct
Genetic testing for Alzheimer     Family history is strong, and confirmation is clinically necessary
 genes, other dementia genes

Reprinted with permission from Rabins PV, Lyketsos CG, Steele CD. Practical dementia care. New York: Oxford
University Press, 1999:47.

FEBRUARY 15, 2001 / VOLUME 63, NUMBER 4                       www.aafp.org/afp                          AMERICAN FAMILY PHYSICIAN          709
to describe a condition that may or may not
  Referral to a subspecialist may be warranted when the                      eventually lead to dementia.13 One study
                                                                             showed that patients with mild cognitive
  presentation or clinical course is atypical of dementia.
                                                                             impairment had a more rapid decline in cog-
                                                                             nitive function than control patients, but a
                                                                             less rapid decline than patients with mild
                        AGE-RELATED COGNITIVE DECLINE AND MILD               Alzheimer’s disease.15
                        COGNITIVE DISORDER                                      The definitions of and the distinctions
                           Age-related cognitive decline is character-       between mild cognitive disorder, age-associ-
                        ized by memory loss without other cognitive          ated cognitive decline and mild cognitive
                        problems (the DSM-IV criteria are described          impairment are controversial. Referral for
                        in Table 2). If memory deficit is present but the    more extensive neuropsychologic testing,
                        other diagnostic criteria for dementia are not,      with follow-up intervals of six to nine
                        a diagnosis other than dementia should be            months, is warranted in patients with mild or
                        considered.6 A disorder similar to age-related       borderline cognitive deficits.16
                        cognitive decline is described as “mild cogni-
                        tive disorder” in the World Health Organiza-         Referral
                        tions ICD-10 classification (International Sta-         The decision to refer the patient with
                        tistical Classification of Diseases, 10th rev.).13   recently diagnosed dementia to a subspecialist
                           The diagnosis of mild cognitive disorder          is influenced by both practical and medical
                        can be made if the cognitive decline is tempo-       considerations. Many family physicians choose
                        rally related to cerebral or systemic disease.       to follow their patients with dementia even
                        Otherwise, the diagnosis of age-related cogni-       when clinical features are atypical or suggestive
                        tive decline should be considered. According         of less common etiologies for the dementia.
                        to the DSM-IV, age-related cognitive decline            However, a neurologist or psychiatrist can
                        represents cognitive changes that are within         sometimes assist in the diagnosis and care of
                        normal limits given the person’s age. Age-           patients with less common dementias, includ-
                        associated cognitive decline is characterized        ing Pick’s disease, dementia of frontal lobe
                        by a decline in only one of the five broad neu-      type, dementia with Lewy bodies, progressive
                        ropsychologic domains associated with                supranuclear palsy, multiple-systems atrophy
                        dementia: memory and learning; attention             and normal-pressure hydrocephalus. Consen-
                        and concentration; thinking; language; and           sus criteria have been established for the diag-
                        visuospatial functioning.14 According to the         noses of dementia with Lewy bodies and vas-
                        International Psychogeriatric Association,14         cular, or multi-infarct, dementia (Table 7).17,19
                        additional criteria should be met to make a          Symptoms that may be helpful in identifying
                        diagnosis of age-related cognitive decline.          the less common causes of dementia include
                        These criteria include the report of cognitive       significant personality changes, extrapyrami-
                        decline from a reliable source, a gradual onset      dal signs, rapid progression, gaze palsy,
                        of at least six months’ duration and a score of      parasympathetic abnormalities, cerebellar
                        more than one standard deviation below the           signs, early urinary incontinence and gait
                        norm on standardized neuropsychologic test-          abnormalities. Other reasons for referral to a
                        ing such as the MMSE.                                neurologist or psychiatrist include rapidly
                                                                             progressive dementia, dementia in a young
                        MILD COGNITIVE IMPAIRMENT                            patient or the presence of psychiatric comor-
                          The diagnosis of mild cognitive impair-            bidities or severe behavior disturbances.
                        ment is difficult and controversial. The term           In a nonresearch setting, neuropsychologic
                        “mild cognitive impairment” has been coined          testing is not considered necessary if the diag-

710   AMERICAN FAMILY PHYSICIAN                      www.aafp.org/afp              VOLUME 63, NUMBER 4 / FEBRUARY 15, 2001
Early Dementia

TABLE 7
Features of Multi-Infarct Dementia and Dementia with Lewy Bodies

Multi-infarct dementia
The characteristic features include stepwise deterioration and patchy distribution of deficits, focal neurologic
signs and evidence of vascular disease as indicated by history, physical examination and laboratory testing.
MODIFIED HACHINSKI ISCHEMIA SCORE:                                                           POINTS
 Abrupt onset . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
 Stepwise progression . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
 Fluctuating course . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
 Nocturnal confusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
 Relative preservation of personality . . . . . . . . . . . . . . . . . . . . . . . . 2
 Depression . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
 Somatic complaints . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
 Emotional incontinence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
 History of hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
 History of stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
 Focal neurologic signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
 Focal neurologic symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
SCORING:Dementia is not likely to be due to vascular causes if the total score is 4 or less; dementia is likely to
be due to vascular causes if the total score is 7 or more.

Dementia with Lewy bodies
The central feature is progressive cognitive decline with resultant functional impairment. Persistent memory
impairment may occur with disease progression. Deficits on tests of attention, frontal–subcortical skills and
visuospatial ability may be prominent.
ESSENTIAL FEATURES FOR DIAGNOSIS:
 Two of the following core features are essential for a diagnosis of probable dementia with Lewy bodies;
 one is essential for possible dementia with Lewy bodies.
  • Fluctuating cognition and pronounced variations in attention and alertness
  • Recurrent visual hallucinations that are typically well formed and detailed
  • Spontaneous motor features of parkinsonism

FEATURES SUPPORTIVE OF THE DIAGNOSIS:
 Repeated falls
 Syncope
 Transient loss of consciousness
 Neuroleptic sensitivity
 Systematized delusions
 Hallucinations
SCORING:A diagnosis of dementia with Lewy bodies is less likely in the presence of the following:
 Stroke disease, evident as focal neurologic signs or on brain imaging
 Evidence of any physical illness or other brain disorder sufficient to account for the clinical picture

Modified Hachinski ischemia score adapted with permission from Rosen WG, Terry RD, Field PA, et al. Patho-
logical verification of ischemic score in differentiation of dementias. Ann Neurol 1980;7:486-8. Criteria for
dementia with Lewy bodies adapted with permission from McKeith IG, Galasko D, Kosaka K, et al. Consen-
sus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the con-
sortium on DLB international workshop. Neurology 1996;47:1113-24.

FEBRUARY 15, 2001 / VOLUME 63, NUMBER 4                                          www.aafp.org/afp          AMERICAN FAMILY PHYSICIAN   711
nosis of dementia can be made using standard                 labeled for the symptomatic treatment of
                              criteria. In general, neuropsychologic testing is            Alzheimer’s disease. Acetylcholinesterase in-
                              indicated when patients with abnormal find-                  hibitors act by delaying neurotransmitter de-
                              ings on the mental state examination show                    gradation, thereby enhancing cortical cholin-
                              normal physical functioning and when the                     ergic activity.
                              index of suspicion is clinically high but screen-               Clinical trials in patients with mild to mod-
                              ing tests are normal.16 Neuropsychologic tests               erate dementia suggest that symptomatic
                              evaluate a wide variety of intellectual domains,             improvement is possible.20,21 Cholinergic side
                              including the level of arousal, attention and                effects, such as nausea, vomiting and diar-
                              orientation, recent and remote memory, lan-                  rhea, are usually transient but may be intoler-
                              guage, praxis, visuospatial function, calcula-               able to some patients. Monitoring of serum
                              tions and judgment. Although there are pub-                  transaminase levels is recommended with use
                              lished norms for most of the commonly used                   of tacrine because of potential hepatotoxicity.
                              standardized tests, the tests are not always                 Experimental treatment options, some with
                              definitive. Serial examination may be neces-                 potentially fewer side effects than those asso-
                              sary. Neuropsychologic tests may also be useful              ciated with currently available agents, may
                              in determining competency for legal purposes,                soon be available for the treatment of
                              in distinguishing dementia from depression                   Alzheimer’s disease.
                              and in helping the patient make important                       The primary management strategy for pro-
                              decisions regarding jobs and finances.                       gressive dementia is to preserve function and
                                                                                           independence, and to maintain quality of life
                              Management and Treatment                                     for as long as possible. Frequent (every three
                                Early diagnosis and intervention allow the                 to six months) clinic visits may be indicated to
                              patient to compensate for the disability, mini-              achieve these goals by maximizing the
                              mize disease-related and medication compli-                  patient’s general health and interacting with
                              cations, improve quality of life and optimize                caregivers to optimize the patient’s social envi-
                              the use of resources. While new experimental                 ronment. Nonpharmacologic interventions,
                              cholinergic drugs for the treatment of Alz-                  including measures to ensure safety at home
                              heimer’s disease are introduced periodically,                and long-term decisions regarding finances, a
                              tacrine (Cognex) and donepezil (Aricept) are                 living will and nursing home placement, are
                              the only cholinesterase inhibitors currently                 often important considerations.
                                                                                              The management of vascular dementia
                                                                                           consists of controlling risk factors such as
The Authors                                                                                hypertension and smoking. The use of anti-
                                                                                           coagulants is indicated in many of these
KAREN S. SANTACRUZ, M.D., is an assistant professor in the departments of pathol-
ogy and neurology at the University of Kansas School of Medicine, Kansas City. She         patients. Because of its safety, aspirin is the
completed a residency at the University of California, Irvine, Medical Center, where she   most commonly used agent. Use of warfarin
trained in anatomic pathology and neuropathology. She developed an interest in             (Coumadin) may also be considered in a lim-
dementia through her interaction with the Alzheimer’s Disease Research Center at the
University of California, Irvine.                                                          ited number of patients, such as those with-
                                                                                           out a significant risk of falling but with a def-
DANIEL SWAGERTY, M.D., M.P.H., is an assistant professor in the departments of fam-
ily medicine and internal medicine in the University of Kansas School of Medicine. He      inite history of stroke.
is also associate director of the Center on Aging at the University of Kansas. Dr. Swa-       The treatment of dementia with Lewy bod-
gerty completed medical school, a family practice residency and a geriatric medicine       ies has not been well studied. However, it is
fellowship at the University of Kansas School of Medicine. He also completed a mas-
ter’s degree in public health at the University of Kansas School of Medicine.              important to note that parkinsonian features
                                                                                           in these patients rarely respond to dopamin-
Address correspondence to Karen S. SantaCruz, M.D., Department of Pathology, Uni-
versity of Kansas School of Medicine, 3901 Rainbow Blvd., Kansas City, KS 66160-           ergic drugs, and that adverse responses to
7410. Reprints are not available from the authors.                                         neuroleptic agents may occur.22

712    AMERICAN FAMILY PHYSICIAN                                www.aafp.org/afp                 VOLUME 63, NUMBER 4 / FEBRUARY 15, 2001
Early Dementia

                                                                state”: a practical method for grading the cogni-
Final Comment                                                   tive state of patients for the clinician. J Psychiatr
                                                                Res 1975;12:189-98.
   Physicians and patients can obtain infor-              9.    Crum RM, Anthony JC, Bassett SS, Folstein MF.
mation about potential experimental treat-                      Population-based norms for the Mini-Mental State
                                                                Examination by age and educational levels. JAMA
ment options and ongoing clinical trials at the                 1993;18:2386-91.
Alzheimer’s Disease Education and Referral                10.   Report of the Quality Standards Subcommittee of
(ADEAR) Center Web site (www.alzheimers.                        the American Academy of Neurology. Practice
                                                                parameter for diagnosis and evaluation of demen-
org) or through an Alzheimer’s disease infor-                   tia. Neurology 1994;44:2203-6.
mation specialist at ADEAR (800-438-4380).                11.   Andreason N, Hesse C, Davidson P, Minthon L,
The ADEAR Center is a service of the                            Wallin A, Winblad B, et al. Cerebrospinal fluid
                                                                beta-amyloid(1-42) in Alzheimer’s disease: differ-
National Institute on Aging (NIA).                              ences between early- and late-onset Alzheimer dis-
   Although no method of curing or arresting                    ease and stability during the course of disease.
Alzheimer’s disease is currently available,                     Arch Neurol 1999;56:673-80.
                                                          12.   Clarfield AM. The reversible dementias: do they
early diagnosis is important for several rea-
                                                                reverse? Ann Intern Med 1988;109:476-86.
sons. The most compelling reason is that                  13.   World Health Organization. The ICD-10 classifica-
early diagnosis allows the patient and family                   tion of mental and behavioral disorders. Geneva:
to plan for the future and identify outside                     World Health Organization, 1992:64-5.
                                                          14.   Levy R. Aging-associated cognitive decline. Work-
sources of assistance. Moreover, as potentially                 ing Party of the International Psychogeriatric Asso-
useful and proven treatments become avail-                      ciation in collaboration with the World Health
able, early diagnosis of dementia will become                   Organization. Int Psychogeriatr 1994;6:63-8 [Pub-
                                                                lished erratum in Int Psychogeriatr 1994;6:133].
increasingly important. Although screening                15.   Peterson RC, Smith GE, Waring SC, Ivnik RJ, Tan-
all elderly patients for dementia is not war-                   gelos EG, Kokmen E. Mild cognitive impairment:
ranted,23 being alert for cognitive and func-                   clinical characterization and outcome Arch Neurol
                                                                1999;56:303-8 [Published erratum in Arch Neurol
tional decline is a prudent way of recognizing                  1999;56:760].
dementia in its early stage.                              16.   Daly MP. Diagnosis and management of Alzheimer
                                                                Disease. J Am Board Fam Pract 1999;12:375-85.
                                                          17.   McKeith IG, Galasko D, Kosaka K, Perry EK, Dick-
REFERENCES
                                                                son DW, Hansen LA, et al. Consensus guidelines
1. Hebert LE, Scherr PA, Beckett LA, Albert MS, Pil-            for the clinical and pathologic diagnosis of demen-
   grim DM, Chown MJ, et al. Age-specific incidence             tia with Lewy bodies (DLB): report of the consor-
   of Alzheimer’s disease in a community population.            tium on DLB international workshop. Neurology
   JAMA 1995;273:1354-9.                                        1996;47:1113-24.
2. Larson EB, Kukull WA, Katzman RL. Cognitive            18.   Chui HC, Victoroff JI, Margolin D, Jagust W,
   impairment: dementia and Alzheimer’s disease.                Shankle R, Katzman R. Criteria for the diagnosis of
   Annu Rev Public Health 1992;13:431-49.                       ischemic vascular dementia proposed by the State
3. Bachman DL, Wolf PA, Linn R, Knoefel JE, Cobb J,             of California Alzheimer’s Disease Diagnostic Treat-
   Belanger A, et al. Prevalence of dementia and                ment Centers. Neurology 1992;42:473-80.
   probable senile dementia of the Alzheimer type in      19.   Rosen WG, Terry RD, Fuld PA, Katzman R, Peck A.
   the Framingham Study. Neurology 1992;42:115-9.               Pathological verification of ischemic score in differ-
4. Collerton D, Davies C, Thompson P. Lewy body                 entiation of dementias. Ann Neurol 1980;7:486-8.
   dementia in clinical practice. In: Perry RH, McKeith   20.   Rogers SL, Farlow MR, Doody RS,Mohs R, Friedhoff
   IG, Perry EK, eds. Dementia with Lewy bodies: clin-          LT. A 24-week, double-blind, placebo-controlled
   ical, pathological, and treatment issues. New York:          trial of donepezil in patients with Alzheimer’s dis-
   Cambridge University Press, 1996:171-86.                     ease. Neurology 1998;50:136-45.
5. Rabins PV, Lyketsos CG, Steele CD. Practical demen-    21.   Sano M, Ernesto C, Thomas RG, Klauber MR, Schafer
   tia care. New York: Oxford University Press, 1999.           K, Grundman M, et al. A controlled trial of selegiline,
6. American Psychiatric Association. Diagnostic and             alpha-tocopherol, or both as treatment for Alz-
   statistical manual of mental disorders. 4th ed.              heimer’s disease. N Engl J Med 1997;336:1216-22.
   Washington, D.C.: American Psychiatric Associa-        22.   McKeith I, Fairbairn A, Perry R, Thompson P, Perry E.
   tion, 1994:123-63,684.                                       Neuroleptic sensitivity in patients with senile
7. Morris JC. The clinical dementia rating (CDR): cur-          dementia of Lewy body type. BMJ 1992;305:673-8.
   rent version and scoring rules. Neurology 1993;        23.   U.S. Preventive Services Task Force. Guide to clini-
   43:2412-4.                                                   cal preventive services. 2d ed. Baltimore: Williams
8. Folstein MF, Folstein SE, McHugh PR. “Mini-mental            & Wilkins, 1996.

FEBRUARY 15, 2001 / VOLUME 63, NUMBER 4                         www.aafp.org/afp                             AMERICAN FAMILY PHYSICIAN   713
You can also read