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## Complete Summary

#### GUIDELINE TITLE

Practice parameter: diagnosis of dementia (an evidence-based review).
Report of the Quality Standards Subcommittee of the American Academy of
Neurology.

#### BIBLIOGRAPHIC SOURCE(S)

Knopman DS, DeKosky ST, Cummings JL, Chui H, Corey-Bloom J, Relkin N,
Small GW, Miller B, Stevens JC. Practice parameter: diagnosis of
dementia (an evidence-based review): report of the quality standards
subcommittee of the American Academy of Neurology. Neurology 2001 May
8;56(9):1143-53. \[147 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

According to the guideline developer, this guideline has been reviewed
and is still considered to be current as of February 2004. This review
involved new literature searches of electronic databases followed by
expert committee review of new evidence that has emerged since the
original publication date.

It updates a previously released version: Practice parameter for
diagnosis and evaluation of dementia. Report of the Quality Standards
Subcommittee of the American Academy of Neurology. Neurology 1994
Nov;44(11):2203-6.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

-   Dementia, including vascular dementia, dementia with Lewy bodies,
    and frontotemporal dementia

-   Creutzfeldt-Jakob disease

-   Alzheimer´s disease

#### GUIDELINE CATEGORY

Diagnosis

#### CLINICAL SPECIALTY

Family Practice\
Geriatrics\
Internal Medicine\
Neurology\
Pathology\
Psychiatry\
Radiology

#### INTENDED USERS

Physicians\
Psychologists/Non-physician Behavioral Health Clinicians

#### GUIDELINE OBJECTIVE(S)

To update the 1994 practice parameter for the diagnosis of dementia in
the elderly

#### TARGET POPULATION

Elderly patients (over age 65) undergoing an initial assessment for
dementia

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Use of Diagnostic Criteria for Dementia**

1.  *Diagnostic and Statistical Manual*, 3rd edition, revised (DSM-IIIR)
    definition of dementia and criteria for Alzheimer\'s disease

2.  *Diagnostic and Statistical Manual*, 4th edition (DSM-IV) definition
    of dementia

3.  National Institute of Neurologic, Communicative Disorder and
    Stroke-Alzheimer\'s Disease and Related disorders Association
    (NINCDS-ADRDA) Work Group definitions of dementia and criteria for
    Alzheimer\'s disease

4.  Hachinski Ischemic Index criteria for diagnosis of cerebrovascular
    disease in dementia

5.  Consortium for dementia with Lewy bodies diagnostic criteria

6.  Consensus diagnostic criteria for frontotemporal dementia

7.  Clinical criteria for Creutzfeldt-Jakob disease

**Structural Neuroimaging for Differential Diagnosis of Dementing
Illness** (Note: linear or volumetric magnetic resonance imaging and
computed tomography are considered but not recommended)

1.  Noncontrast computed tomography

2.  Noncontrast magnetic resonance imaging

**Functional Neuroimaging** (Note: these tests are considered but not
recommended for routine use)

1.  Single-photon emission computed tomography (SPECT)

2.  Positron emission tomography (PET)

**Other Laboratory Tests**

1.  Genetic testing, such as apolipoprotein E (APOE) genotyping, testing
    for tau mutations or Alzheimer\'s disease gene mutations (considered
    but not recommended)

2.  Immunoassay for cerebrospinal fluid 14-3-3 protein in diagnosis of
    Creutzfeldt-Jakob disease

3.  Other cerebrospinal fluid markers or biomarkers (considered but not
    recommended)

**Screening for Comorbid Conditions**

1.  Screening for depression using validated instruments, e.g. Geriatric
    Depression Scale, Centers for Epidemiologic Studies Depression
    Scale, and Hamilton Depression Scale

2.  Screening for vitamin B~12~ deficiency

3.  Screening for hypothyroidism

4.  Screening for syphilis (considered but not routinely recommended)

#### MAJOR OUTCOMES CONSIDERED

-   Reliability of current criteria for diagnosis of dementia

-   Accuracy of current criteria to establish diagnosis for the
    prevalent dementias

-   Accuracy of laboratory tests in the clinical diagnosis of dementia
    (sensitivity, specificity, positive predictive value of tests)

-   Incidence of comorbidities in elderly patients undergoing initial
    assessment for dementia

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Hand-searches of Published Literature (Primary Sources)\
Hand-searches of Published Literature (Secondary Sources)\
Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

*Literature review process*. A literature search was conducted using
MEDLINE, Excerpta Medica, and BIOSIS. The search included articles
published from January 1985 through November 1999. The search strategy
sought only studies published in English and studies on human disease.
The principal search term was dementia. Other terms entered into the
search included neuroimaging, diagnostic techniques, diagnostic imaging,
biologic markers, cerebrospinal fluid, diagnostic errors, differential
diagnosis, and neuropsychologic tests. The original search yielded 1,175
articles of which approximately 300 articles were identified as relevant
to the search questions. For articles on Alzheimer´s disease, the
authors of the guideline included only those based on more than 25
patients. For the less common dementias, there was no minimal sample
size. An additional 300 articles not identified by the literature search
strategy, including ones published after the initial search was
conducted, were submitted by committee members or obtained from
bibliographies of articles identified in the search.

#### NUMBER OF SOURCE DOCUMENTS

300

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

**Classification of Evidence**

I.  Evidence provided by a well-designed prospective study in broad
    spectrum of persons with the suspected condition, using a \"gold
    standard\" for case definition, in which test is applied in a
    blinded evaluation, and enabling the assessment of appropriate tests
    of diagnostic accuracy.

II. Evidence provided by a well-designed prospective study of a narrow
    spectrum of persons with the suspected condition, or a well designed
    retrospective study of a broad spectrum of persons with an
    established condition (by \"gold standard\") compared with a broad
    spectrum of controls, in which test is applied in blinded
    evaluation, and enabling the assessment of appropriate tests of
    diagnostic accuracy.

III. Evidence provided by a retrospective study in which either persons
     with the established condition or controls are of a narrow
     spectrum, and in which test is applied in a blinded evaluation.

IV. Any design in which test is not applied in blinded evaluation OR
    evidence provided by expert opinion alone or in descriptive case
    series (without controls).

#### METHODS USED TO ANALYZE THE EVIDENCE

Review of Published Meta-Analyses\
Systematic Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not applicable

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Not stated

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

**Definitions for Practice Recommendations Based on Classification of
Evidence**

*Standard*. Principle for patient management that reflects a high degree
of clinical certainty (usually this requires Class I evidence that
directly addresses the clinical question, or overwhelming Class II
evidence when circumstances preclude randomized clinical trials).

*Guideline*. Recommendation for patient management that reflects
moderate clinical certainty (usually this requires Class II evidence or
a strong consensus of Class III evidence).

*Practice Option*. Strategy for patient management for which the
clinical utility is uncertain (inconclusive or conflicting evidence or
opinion).

*Practice Advisory*. Practice recommendation for emerging and/or newly
approved therapies or technologies based on evidence from at least one
Class I study. The evidence may demonstrate only a modest statistical
effect or limited (partial) clinical response, or significant
cost--benefit questions may exist. Substantial (or potential)
disagreement among practitioners or between payers and practitioners may
exist.

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

External Peer Review\
Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

In addition to the review and final approval by the Quality Standards
Subcommittee and the Practice Committee of the American Academy of
Neurology, this Practice Parameter was reviewed by American Academy of
Neurology members who had identified themselves as interested reviewers,
by the Geriatric and Behavioral Neurology Sections of the American
Academy, by representatives of the American Geriatrics Society, and by
representatives of the Alzheimer´s Association.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

*Classification of evidence ratings, I-IV, and the definitions for
practice recommendations based on classification of evidence (Standard,
Guideline, Practice Option, Practice Advisory) are defined at the end of
the \"Major Recommendations\" field.*

**Diagnostic Criteria**

Are the current criteria for the diagnosis of dementia reliable?

-   The *Diagnostic and Statistical Manual*, 3rd edition, revised
    (DSM-III-R) definition of dementia, which is identical to
    *Diagnostic and Statistical Manual*, 4th edition (DSM-IV)
    definition, is reliable and should be used routinely
    **(Guideline)**.

Are current diagnostic criteria able to establish a diagnosis for the
prevalent dementias (Alzheimer´s disease, vascular dementia, dementia
with Lewy bodies, frontotemporal dementia, prion diseases)?

-   The National Institute of Neurologic, Communicative Disorders and
    Stroke--Alzheimer´s disease and Related Disorders Association
    (NINCDS-ADRDA) for the diagnosis of probable Alzheimer\'s disease or
    the *Diagnostic and Statistical Manual*, 3rd edition, revised
    (DSM-III-R) criteria for dementia of the Alzheimer\'s type (DAT)
    should be routinely used **(Guideline)**.

-   The Hachinski Ischemic Index criteria may be of use in the diagnosis
    of cerebrovascular disease in dementia **(Option)**.

-   The Consortium for dementia with Lewy bodies (DLB) diagnostic
    criteria may be of use in clinical practice **(Option)**.

-   The Consensus diagnostic criteria for frontotemporal dementia (FTD)
    may be of use in clinical practice **(Option)**.

-   Clinical criteria for Creutzfeldt-Jakob disease (CJD) should be used
    in rapidly progressive dementia syndromes **(Guideline)**.

**Laboratory Tests**

Do laboratory tests improve the accuracy of clinical diagnosis of
dementing illness?

-   Structural neuroimaging with either a noncontrast computed
    tomography or magnetic resonance scan in the initial evaluation of
    patients with dementia is appropriate. **(Guideline)**.

-   Linear or volumetric magnetic resonance or computed tomography
    measurement strategies for the diagnosis of Alzheimer\'s Disease and
    are not recommended for routine use at this time **(Guideline)**.

-   For patients with suspected dementia, single photon emission
    computed tomography (SPECT) cannot be recommended for routine use in
    either initial or differential diagnosis as it has not demonstrated
    superiority to clinical criteria **(Guideline)**.

-   Positron emission tomography (PET) imaging is not recommended for
    routine use in the diagnostic evaluation of dementia at this time
    **(Guideline)**.

-   Genetic testing of patients with suspected dementia with Lewy bodies
    and Creutzfeldt-Jakob disease is not recommended **(Guideline)**.

-   Routine use of apolipoprotein E (APOE) genotyping in patients with
    suspected Alzheimer\'s disease is not recommended at this time
    **(Guideline)**.

-   There are no other genetic markers recommended for routine use in
    the diagnosis of Alzheimer´s disease **(Guideline)**.

-   Testing for tau mutations or Alzheimer\'s disease gene mutations is
    not recommended for routine evaluation in patients with
    frontotemporal dementia at this time **(Guideline)**.

-   There are no cerebrospinal fluid or other biomarkers recommended for
    routine use in determining the diagnosis of Alzheimer\'s disease at
    this time **(Guideline)**.

-   The cerebrospinal fluid 14-3-3 protein is useful recommended for
    confirming or rejecting the diagnosis of Creutzfeldt--Jakob disease
    in clinically appropriate circumstances **(Guideline)**.

**Screening for Comorbid Diseases**

What comorbidities should be screened for in elderly patients undergoing
an initial assessment for dementia?

-   Depression is a common, treatable comorbidity in patients with
    dementia and should be screened for **(Guideline)**.

-   B~12~ deficiency is common in the elderly, and B~12~ levels should
    be included in routine assessments of the elderly. **(Guideline)**.

-   Because of its frequency, hypothyroidism should be screened for in
    elderly patients. **(Guideline)**.

-   Unless the patient has some specific risk factor or evidence of
    prior syphilitic infection, or resides in one of the few areas in
    the United States with high numbers of syphilis cases, screening for
    the disorder in patients with dementia is not justified
    **(Guideline)**.

**Definitions**:

**Classification of Evidence**

I.  Evidence provided by a well-designed prospective study in broad
    spectrum of persons with the suspected condition, using a \"gold
    standard\" for case definition, in which test is applied in a
    blinded evaluation, and enabling the assessment of appropriate tests
    of diagnostic accuracy.

II. Evidence provided by a well-designed prospective study of a narrow
    spectrum of persons with the suspected condition, or a well designed
    retrospective study of a broad spectrum of persons with an
    established condition (by \"gold standard\") compared with a broad
    spectrum of controls, in which test is applied in blinded
    evaluation, and enabling the assessment of appropriate tests of
    diagnostic accuracy.

III. Evidence provided by a retrospective study in which either persons
     with the established condition or controls are of a narrow
     spectrum, and in which test is applied in a blinded evaluation

IV. Any design in which test is not applied in blinded evaluation OR
    evidence provided by expert opinion alone or in descriptive case
    series (without controls).

**Practice Recommendations Based on Classification of Evidence**

*Standard*. Principle for patient management that reflects a high degree
of clinical certainty (usually this requires Class I evidence that
directly addresses the clinical question, or overwhelming Class II
evidence when circumstances preclude randomized clinical trials).

*Guideline*. Recommendation for patient management that reflects
moderate clinical certainty (usually this requires Class II evidence or
a strong consensus of Class III evidence).

*Practice Option*. Strategy for patient management for which the
clinical utility is uncertain (inconclusive or conflicting evidence or
opinion).

*Practice Advisory*. Practice recommendation for emerging and/or newly
approved therapies or technologies based on evidence from at least one
Class I study. The evidence may demonstrate only a modest statistical
effect or limited (partial) clinical response, or significant
cost--benefit questions may exist. Substantial (or potential)
disagreement among practitioners or between payers and practitioners may
exist.

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The recommendations are based on a review of the literature. The type of
supporting evidence is identified and graded for each recommendation on
the diagnosis of dementia.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

-   Appropriate use of diagnostic criteria and laboratory tests for
    dementia

-   Appropriate recognition and treatment of depression, B~12~
    deficiency, and hypothyroidism, which are common comorbid conditions
    in elderly patients with dementia

#### POTENTIAL HARMS

Not stated

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

-   The statement of this guideline is provided as an educational
    service of the American Academy of Neurology. It is based on an
    assessment of current scientific and clinical information. It is not
    intended to include all possible proper methods of care for a
    particular neurologic problem or all legitimate criteria for
    choosing to use a specific procedure. Neither is it intended to
    exclude any reasonable alternative methodologies. The American
    Academy of Neurology recognizes that specific patient care decisions
    are the prerogative of the patient and the physician caring for the
    patient, based on all of the circumstances involved.

-   The purpose of the current practice parameter is to highlight and to
    update major areas of current interest and investigation in the
    diagnosis of dementia in the elderly. It is not intended to serve as
    a comprehensive review of the differential diagnosis of dementia.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Clinical Algorithm\
Patient Resources\
Personal Digital Assistant (PDA) Downloads\
Quick Reference Guides/Physician Guides\
Resources

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Knopman DS, DeKosky ST, Cummings JL, Chui H, Corey-Bloom J, Relkin N,
Small GW, Miller B, Stevens JC. Practice parameter: diagnosis of
dementia (an evidence-based review): report of the quality standards
subcommittee of the American Academy of Neurology. Neurology 2001 May
8;56(9):1143-53. \[147 references\]

#### ADAPTATION

Not applicable: Guideline was not adapted from another source.

#### DATE RELEASED

2001 May (reviewed 2004)

#### GUIDELINE DEVELOPER(S)

American Academy of Neurology - Medical Specialty Society

#### SOURCE(S) OF FUNDING

American Academy of Neurology (AAN)

#### GUIDELINE COMMITTEE

Quality Standards Subcommittee

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Authors*: D.S. Knopman, MD; S.T. DeKosky, MD; J.L. Cummings, MD; H.
Chui, MD; J. Corey-Bloom, MD, PhD; N. Relkin, MD, PhD; G.W. Small, MD;
B. Miller, MD; and J.C. Stevens, MD

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Committee members disclosed any real or potential conflicts of interest.

#### ENDORSER(S)

American Association of Neuroscience Nurses - Professional Association\
American Geriatrics Society - Medical Specialty Society

#### GUIDELINE STATUS

This is the current release of the guideline.

According to the guideline developer, this guideline has been reviewed
and is still considered to be current as of February 2004. This review
involved new literature searches of electronic databases followed by
expert committee review of new evidence that has emerged since the
original publication date.

It updates a previously released version: Practice parameter for
diagnosis and evaluation of dementia. Report of the Quality Standards
Subcommittee of the American Academy of Neurology. Neurology 1994
Nov;44(11):2203-6.

#### GUIDELINE AVAILABILITY

Electronic copies: Available from the [American Academy of Neurology
(AAN) Web site](http://aan.com/go/practice/guidelines).

Print copies: Available from the AAN Member Services Center, (800)
879-1960, or from AAN, 1080 Montreal Avenue, St. Paul, MN 55116.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   AAN encounter kit for dementia: a multi-media, web-based algorithm.
    Available from the [American Academy of Neurology (AAN) Web
    site](http://www.aan.com/go/practice/quality/dementia).

-   AAN summary of evidence-based guideline for clinicians: detection,
    diagnosis and management of dementia. St. Paul (MN): American
    Academy of Neurology. 2 p. Electronic copies: Available in Portable
    Document Format (PDF) from the [AAN Web
    site](http://www.aan.com/professionals/practice/pdfs/dementia_guideline.pdf).
    See the related QualityTool summary on the [Health Care Innovations
    Exchange Web site](http://innovations.ahrq.gov/content.aspx?id=821).

-   Practice parameter: diagnosis of dementia. American Academy of
    Neurology. 2001. 16 p. Available for personal digital assistant
    (PDA) download from the [AAN Web
    site](http://www.aan.com/professionals/practice/guidelines/pda/Dementia_diagnosis.pdf).

-   AAN guideline development process. St. Paul (MN): American Academy
    of Neurology. Electronic copies: Available from the [AAN Web
    site](http://www.aan.com/go/practice/guidelines/development).

#### PATIENT RESOURCES

The following are available:

-   AAN guideline summary for patients and their families: Alzheimer\'s
    disease. St. Paul (MN): American Academy of Neurology. 2 p.

Electronic copies: Available in Portable Document Format (PDF) from the
[American Academy of Neurology (AAN) Web
site](http://www.aan.com/professionals/practice/pdfs/dem_pat.pdf). See
the related QualityTool summary on the [Health Care Innovations Exchange
Web site](http://innovations.ahrq.gov/content.aspx?id=817).

Please note: This patient information is intended to provide health
professionals with information to share with their patients to help them
better understand their health and their diagnosed disorders. By
providing access to this patient information, it is not the intention of
NGC to provide specific medical advice for particular patients. Rather
we urge patients and their representatives to review this material and
then to consult with a licensed health professional for evaluation of
treatment options suitable for them as well as for diagnosis and answers
to their personal medical questions. This patient information has been
derived and prepared from a guideline for health care professionals
included on NGC by the authors or publishers of that original guideline.
The patient information is not reviewed by NGC to establish whether or
not it accurately reflects the original guideline\'s content.

#### NGC STATUS

This NGC summary was completed by ECRI on February 12, 2002. The
information was verified by the guideline developer on September 5,
2003.

#### COPYRIGHT STATEMENT

This NGC summary is based on the original guideline, which is
copyrighted by the American Academy of Neurology.

### DISCLAIMER

#### NGC DISCLAIMER

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Date Modified: 11/3/2008