Chapter 9
Neurocognitive Disorders
Mary Guerriero Austrom, Courtney B. Johnson, Daniel F. Rexroth,
and Frederick W. Unverzagt
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Neurocognitive disorders (NCDs), as defined in
the Diagnostic and Statistical Manual of Mental Dis-
orders, Fifth Edition (DSM–5; American Psychiatric
Association, 2013), are a collection of syndromes in
which the prominent clinical feature is an acquired
decline in cognitive functioning. Importantly,
dementia, a term dating to the late 18th century, is
widely understood by practitioners and the public
alike to indicate a common, usually age-related, loss
of mental abilities, has been replaced by the term
major neurocognitive disorder. Forms of NCD that
adversely affect cognition but do not limit indepen-
dence in daily activities are called mild neurocog-
nitive disorders. NCDs are subtyped according to
presumed etiology (e.g., Alzheimer’s disease [AD],
vascular disease, Lewy bodies, and frontotemporal
lobar degeneration).
This chapter describes the epidemiology; clinical
features; and assessment, diagnosis, and manage-
ment of the more common NCDs and recommend
nonpharmacological interventions for common
symptoms across NCDs.
DEFINITIONS
Before delving into the specifics of the NCDs, it is
helpful to have an understanding of their general
diagnostic criteria from a clinical psychologist’s per-
spective. First, we will consider a neurological disor-
der that is somewhat distinct from the NCDs.
Dr. Guerriero Austrom and Dr. Unverzagt were supported in part by National Institutes of Health Grant P30 AG010133.
http://dx.doi.org/10.1037/14862-009
APA Handbook of Clinical Psychology: Vol. 4. Psychopathology and Health, J. C. Norcross, G. R. VandenBos, and D. K. Freedheim (Editors-in-Chief)
Copyright © 2016 by the American Psychological Association. All rights reserved.
APA Handbook of Clinical Psychology: Psychopathology and Health, edited by J. C.
Norcross, G. R. VandenBos, D. K. Freedheim, and N. Pole
Copyright © 2016 American Psychological Association. All rights reserved.
Delirium
It may seem inconsistent to describe delirium as
a distinct disorder separate from the NCDs given
that delirium is clearly an NCD related to struc-
tural or metabolic brain dysfunction. Delirium,
however, does not lend itself to the major or minor
distinction based on severity of impairment, in part
because symptom severity in delirium fluctuates
(Ganguli et al., 2011). The essential clinical feature
of delirium is a disturbance of attention or aware-
ness that usually develops rapidly over a period of a
few hours to days. Because attention represents an
early phase of information processing that is criti-
cal to other mental abilities, there will be “down-
stream” disruptions and impairments to perception,
memory, language, spatial, and executive skills.
Fluctuation in mental status over time is typical
with an affected person appearing relatively intact
at one point and then quite impaired later in the
same day.
The very young and the very old are most vul-
nerable to delirium. The most common risk fac-
tor for delirium in the young tends to be infection
with high fever. Advanced age, comorbid physical
problems such as sleep deprivation, immobility,
dehydration, pain, and sensory impairment are the
common risk factors in adults. Dementia in older
adults increases the risk for delirium (Reuben et al.,
2013). Differential diagnoses for delirium include
major NCD, psychotic disorders or mood disorders
253
 Guerriero Austrom et al.
with psychotic features, acute stress disorder, malin-
gering disorder, or factitious disorder (American
Psychiatric Association, 2013).
Delirium is surprisingly common; an estimated
10%–15% of medical patients develop delirium in hos-
pital settings. It is particularly common among post-
surgical and elderly patients, especially those older
than 80 years. Other risk factors for delirium include
a preexisting dementia, bone fractures, systemic infec-
tions, and use of narcotics or antipsychotics (Callahan,
Austrom, & Unverzagt, 2004). Delirium is associated
with high mortality; an estimated 40%–50% of the
patients with delirium die within 1 year of developing
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the disturbance (Andreasen & Black, 2001).
Management and treatment considerations
from the clinical psychologist’s perspective include
understanding that delirium represents a medical
emergency and an emergent evaluation by a physi-
cian is essential (Callahan et al., 2004). Treatment is
focused on identification and removal of the offend-
ing agent or restoration of metabolic process. Until
the condition is corrected, measures must be taken
to maintain the patient’s health and safety, including
constant observation, consistent nursing care, and
frequent reassurance with repeated simple explana-
tions. Delirious patients are extremely sensitive to
drug side effects, so unnecessary medications should
be discontinued, including sedatives or hypnotics
(e.g., benzodiazepines). Highly agitated patients
may be calmed with low doses of high-potency anti-
psychotic medication (e.g., haloperidol); however,
drugs with significant anticholinergic effects (e.g.,
chlorpromazine, thioridazine) should be avoided
because they can worsen or prolong the delirium
(Andreasen & Black, 2001).
Major Neurocognitive Disorder
Major NCD is defined as substantial decline or loss
in cognitive function that makes it impossible for
the affected person to independently manage daily
activities. The decline may be documented by a
complaint made by a patient or family member or it
may be based on observations made by a health care
professional. The cognitive decline should be sub-
stantial. If it is corroborated via psychometric testing
the deficits are usually on the order of 1.5 standard
254
deviations or more below normative reference sam-
ples. The cognitive deficits do not occur exclusively
in the context of delirium. A presumed etiology is
specified whenever possible (American Psychiatric
Association, 2013).
Owing to its wide understanding and general
use, the term dementia is retained and is considered
to fall under the major NCD category. Major NCD is
broader than dementia, as typically understood, and
includes conditions where the impairment affects a
single domain (e.g., amnestic disorder in the Diag-
nostic and Statistical Manual of Mental Disorders,
Fourth Edition, Text Revision; American Psychiatric
Association, 2000).
Patients presenting with symptoms of what
appears to be major NCD will often receive a revers-
ible dementia workup before the diagnosis is made.
A recent meta-analysis found 9% of cases had a
potentially reversible cause (Clarfield, 2003). Even
more important, follow-up of this subset revealed
that only 0.6% of the cases were actually reversible
(with <1% of cases reversing partially or completely;
Clarfield, 2003). Common elements of a reversible
dementia workup are presented in Table 9.1.
Patients with major NCD present with important
psychosocial management needs, including employ-
ment versus pursuit of disability claim, extent and
control of motor vehicle use, managing finances and
purchases, medication administration, and travel.
The timing and transition of decisional capacity
and long-term care are major issues. There is a criti-
cal need to offer support to patients and families
through education and social services.
Mild Neurocognitive Disorders
Mild NCD is a new diagnostic category in the
DSM–5 (American Psychiatric Association, 2013).
In mild NCD, cognitive decline is present, although
milder than what is observed in patients with
major NCD. Complex activities of daily living are
preserved—that is, there is no need for outside
assistance; however, patients may report increased
effort in completing them or reliance on compensa-
tory strategies and accommodations. Fundamen-
tally, recent research shows that neurodegenerative
diseases progress through an asymptomatic or
cognitively normal phase, then a mild NCD phase,

TABLE 9.1
Typical Features of a Reversible Dementia Workup
Screens Laboratory
Depressiona
B12
a MRI scan
Thyroid-stimulating hormone
levela
Complete blood count
Glucose
Electrolytes
Calcium
Kidney functioning
Liver functioning
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Note. Data from Knopman et al., 2001; National Institutes of Health, 2007; and Simmons, Hartmann, and
Dejoseph, 2011. MRI = magnetic resonance imaging; CT = computed tomography; PET = positron emission
tomography.
aAmerican Academy of Neurology has recommended these factors be routinely evaluated for all patients with suspected
NCD (Knopman et al., 2001).
and then a major NCD phase (Plassman et al., 2008,
2011).
The following sections present the prominent
variants of NCDs. For each, we summarize the clini-
cal features, incidence, risk factors, etiology, diag-
nostic process, and treatment approaches.
ALZHEIMER’S DISEASE
AD is characterized by insidious onset and gradual
progression of memory loss. This typically is expe-
rienced as difficulty remembering recent conversa-
tions and events, repetitive questions or statements,
misplacing items, difficulty keeping track of time
(e.g., knowing the date), difficulty tracking appoint-
ments, and getting lost in familiar locations. Aware-
ness of symptoms is variable at the early stage; many
patients will be aware of changes though they may
be reluctant to express concerns. Others will com-
plain openly of their difficulties. Others still will
be unaware or vehemently deny memory problems
altogether. Diagnosis tends to lag symptom onset by
2–3 years in many cases. As the illness progresses,
there are typically problems in word-finding, diffi-
culty in solving problems, and difficulty in managing
complex tasks in the home like finances (paying bills,
banking, insurance), digital electronic equipment
Neurocognitive Disorders
Imaging If clinically indicated
Toxicology
Noncontrast CT (especially if Human immunodeficiency virus
acute onset, patient younger test
than 65, or clinical indicators Lyme titer
suggest) Rapid plasma reagin test (e.g.,
syphilis)
Erythrocyte sedimentation rate
Lumbar puncture
Electroencephalogram
PET imaging
(television, smart phone, computer), and appliances
(clothes washer, stove). With further decline, it is
common to see problems with basic activities of daily
living like dressing, bathing, and toileting. At this
stage, most patients will have lost awareness they
might have had regarding their symptoms or illness.
Taking a history from a family member is critical to
clearly understanding clinical status at any stage of
the illness but particularly in the moderate and later
stages.
Incidence and Prevalence
In 2010, an estimated 35.6 million people were
living with a diagnosis of dementia worldwide
with 3.9 million living in the United States (Prince
et al., 2013). A recent national sample showed
that AD accounted for more than 70% of all inci-
dent dementia cases (Plassman et al., 2011). In a
recent national probability sample of the United
States, 3.4 million individuals 72 years old or
older developed dementia within a 6-year follow-
up period (Plassman et al., 2011). Researchers
propose a 1%–2% incidence rate of AD annually
(Petersen et al., 1999). The majority of incident
dementia cases (75%) are composed of individuals
with a prior history of cognitive complaints (Plass-
man et al., 2011). Annual rates of progression for
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 Guerriero Austrom et al.
individuals with prodromal symptoms range from
17% to 20% (Plassman et al., 2008).
Risk Factors
Known risk factors for AD include older age, positive
family history of dementia, presence of at least one
ε4 allele for apolipoprotein E (ApoE), lower edu-
cational attainment, and history of traumatic brain
injury (TBI). In addition, possible risk factors include
diabetes, hypertension, cardiovascular disease, and
depression (Alzheimer’s Association, 2013; Plassman
et al., 2011; Shepardson, Shankar, & Selkoe, 2011).
Less than 1% of AD cases are caused by autosomal
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dominant mutations involving presenilin-1,
presenilin-2, and amyloid precursor protein
(Caselli & Reiman, 2013).
Pathology
Neuropathologically, AD is characterized by brain
atrophy, synaptic and neuronal loss, extracellular
deposits composed of diffuse and fibrillar beta-
amyloid, and intracellular accumulation of neurofi-
brillary tangles composed of hyperphosphorylated
tau. Older adults with normal cognition at autopsy
also have been shown to have AB deposition and
neurofibrillary tangles. Approximately 65% of a
very well-characterized sample had some kind
of pathology indicative of AD or other dementia
at autopsy (Bennett et al., 2006). The pathologic
diagnosis of AD requires careful neuropathological
examination of the brain postmortem. For the most
recent criteria for postmortem exam, see Hyman
et al. (2012) and Montine et al. (2012). Although
several neuroimaging methods using visual rat-
ing scales, volumetric measurements, and amyloid
and tau tracer compounds are an important area
of research, the gold standard for the diagnosis of
AD remains histological identification of amyloid
plaques and neurofibrillary tangles in the medial
temporal lobe—particularly in the entorhinal
cortex and the hippocampus, postmortem (Appel
et al., 2009).
Diagnosis
A diagnosis of possible AD is used when there is
evidence of cognitive deficits for AD dementia, but
the course of disease is atypical, for example, if there
256
is a sudden onset of cognitive impairment or there
is a lack of historical detail or objective cognitive
documentation of progressive decline. Addition-
ally, a diagnosis of possible AD is used when there
is (a) a history of stroke temporally related to the
onset or worsening of cognitive impairment, (b) the
presence of multiple or extensive infarcts or severe
white-matter hyperintensity burden, (c) features of
dementia with Lewy bodies other than the dementia
itself, or (d) evidence of another neurological dis-
ease or a non-neurological medical comorbidity or
medication use that could have a substantial effect
on cognition. These diagnostic recommendations
for probable and possible AD are consistent with the
recent guidelines outlined by the National Institute
on Aging–Alzheimer’s Association workgroups. In
addition, clarification of amnestic or nonamnes-
tic presentation as the initial and most prominent
symptom is also recommended by the workgroups
(McKhann et al., 2011).
Treatment
Currently, there are no disease-modifying treat-
ments for AD. Treatment for major NCD resulting
from AD is mainly medication management through
the use of acetylcholinesterase inhibitors, glutamate
blockers, secretase inhibitors, and passive immuni-
zation, currently in clinical trials. Specific symptoms
can be treated with selective serotonin reuptake
inhibitors (SSRIs) and atypical neuroleptics with
mixed results. At this time, with no disease-altering
therapy available, these treatments offer limited
symptomatic relief and may slow the decline of the
progression for some time, typically 6–18 months,
but the disease itself is not altered. The SSRIs and
atypical neuroleptics often are used for behavioral
and psychiatric symptoms that often accompany the
NCDs, such as depression, agitation, aggression,
sleep disturbances, and wandering.
MAJOR NCD DUE TO FRONTOTEMPORAL
DISORDER
Frontotemporal dementia (FTD) has four variants:
behavioral, semantic, logopenic, and progressive
nonfluent aphasia. These disorders generally have
a younger age of onset that often results in a delay

in obtaining an accurate diagnosis. The behavioral
variant of FTD is more likely to be associated with
motor neuron disease than the other variants (See-
laar et al., 2011).
The behavioral variant is characterized by diverse
symptoms including loss of social skills, apathy,
disinhibition, repetitive and compulsive behaviors,
progressive inability to represent the self and oth-
ers, loss of word meaning, and inability to express
oneself. Changes in diet and a tendency to put
nonedible things in one’s mouth (hyperorality) also
can be present. Decline in self-care, rigidity in think-
ing, and a tendency toward perseveration are also
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common.
The three language variants of FTD—progressive
nonfluent aphasia (PNFA), logopenic, and
semantic—have some distinct features as well as
some overlap between them. Therefore, they should
be viewed as a continuum rather than as distinct cat-
egories that are always easily distinguishable.
PNFA includes agrammatism (decline in the
ability to use articles, adverbs, and prepositions),
phonemic paraphasias (omission or addition of
sounds in a word), or anomia (impairment of abil-
ity to name objects; Gorno-Tempini et al., 2011;
Mesulam et al., 2009). Other possible symptoms are
stuttering, repetition difficulty, and acquired inabil-
ity to read (alexia), or an acquired inability to write
(agraphia).
In logopenic aphasia, word retrieval, difficulty
with repetition, and phonemic paraphasias are most
evident (Mesulam et al., 2009). Semantic dementia
(SD) is the loss of understanding of the meaning of
words and difficulty in identifying objects (Neary
et al., 1998). Speech may be fluent but is usually
empty; semantic paraphasias and a loss of the mean-
ing of words are common. Poor comprehension of
single words is often one of the first symptoms of
the disease. Impaired object knowledge and dif-
ficulty reading irregular words is frequent, while
speech production and repetition speech are usually
normal.
Incidence and Prevalence
The prevalence of FTD has ranged from 2.7/100,000
(Rosso et al., 2003) in a study of 60- to 69-year-
olds in the Netherlands to 15.1/100,000 (Ratnavalli
Neurocognitive Disorders
et al., 2002) in a study of adults under 65 years of
age in the United Kingdom. FTD is believed to be
the third most common cause of dementia overall;
in patient’s under the age of 65 years, it is thought
to be the second most common cause (Arvanita-
kis, 2010). A convenience study of patients with
dementia whose brains were donated to the Florida
Brain Bank at time of autopsy (n = 382) revealed
that 5% had FTD. Of FTD subtypes, the behavioral
variant is the most common (~55%) followed by
PNFA (~25%) and semantic dementia (~20%; John-
son et al., 2005; Kertesz et al., 2007; Neary et al.,
1998). Age of onset for FTD is typically between the
ages of 45 and 64 years, although there are reports
that as many as 20%–30% of cases occur outside of
this range (Hodges et al., 2010; Ikeda, Ishikawa, &
Tanabe, 2004; Johnson et al., 2005; Ratnavalli et al.,
2002; Weder et al., 2007). One study found that the
median time between symptom onset and institu-
tionalization for FTD patients was 5 years; however,
because it usually takes several years to obtain a cor-
rect diagnosis, institutionalization occurred approx-
imately 1 year after diagnosis (Hodges et al., 2003).
FTD is often misdiagnosed as drug use, alcoholism,
or primary psychiatry disorder, and this may con-
tribute to the later diagnosis. Because patients are
further along in their disease at the time of diagno-
sis, institutionalization may occur seemingly closer
to diagnosis than AD.
Pathology
Atrophy of the frontal or anterior temporal lobes
is characteristic of FTD. (Cairns et al., 2007).
Changes also may be occurring at the microscopic
level; these are found at autopsy. So far, seven genes
have been identified in which, if mutations occur,
will result in FTD. These include tau (MAPT),
progranulin (GRN), chromosome 9 open reading
frame 72 (C9ORF72), and four other less common
genes: valosin-containing protein (VCP), transac-
tive response-DNA binding protein-43 (TARDBP),
fused in sarcoma (FUS), and charged multivesicular
body protein 2B (BHMP2B; Goedert, Ghetti, & Spill-
antini, 2012). The most common of these generally
involve protein abnormalities to tau, TDP-43, and
progranulin. Nearly half of patients with FTD have
a family history of NCD, although most cases are
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 Guerriero Austrom et al.
sporadic (Binetti et al., 2003; Bird et al., 2003; Chow
et al., 1999).
Diagnosis
One of the main considerations in the differential
diagnosis of FTD is ruling out other NCDS because
there can be symptom overlap. For example,
patients with AD may have some changes in behav-
ior or language, but the first noted symptom is usu-
ally memory impairment. Lewy body dementia also
should be considered, although this form of demen-
tia often includes a REM sleep disorder, significant
cognitive fluctuations, and well-formed visual hal-
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lucinations. Vascular dementia also should be con-
sidered, although the timeline for decline usually
begins more abruptly and may include periods of
improvement.
Neuropsychological testing can be beneficial
toward gaining a diagnosis. Results may reveal
impaired executive functions such as difficulty with
conceptual reasoning, set shifting, problems solv-
ing, or inhibition. Language impairment may be
present.
Magnetic resonance imaging (MRI) may reveal
bilateral or asymmetric atrophy of the frontal or
anterior temporal lobes; however, this finding is not
necessary for diagnosis because it is not always pres-
ent until later in the disease. A fluorodeoxyglucose
positron emission tomography (FDG-PET) or single
photon emission computed tomography (SPECT)
image may reveal hypometabolism or reduced cere-
bral blood flow in the frontal or temporal lobes;
however, the SPECT scan is less sensitive to FTD
than the FDG-PET. Cerebrospinal fluid has shown
some promise as a measure of biochemical markers.
Current biomarkers being studied for evaluating
neurodegenerative diseases include amyloid B-42,
phosphorylated tau-181, and total tau protein.
Treatment
Currently, there are no disease-modifying treatments
for FTDs. Treatment is focused on managing symp-
toms and distressing behaviors. Antidepressants
commonly are prescribed to treat social disinhibition
and impulsive behavior. Patients with aggression
or delusions sometimes are prescribed low doses
of antipsychotic medications. Anticholinesterase
258
inhibitors are currently being researched as a way
to improve behavioral and cognitive symptoms in
people with behavior variant FTD, but so far, results
have been mixed at best, including occasional evi-
dence of symptom worsening (National Institute
on Aging, 2010). Until a disease-modifying therapy
becomes available, people with FTD likely will
benefit from a team-based approach to care that
includes several disciplines—usually neurology,
neuropsychology, social work, and psychiatry—and
also should include rehabilitative therapies such
as speech-language pathology (SLP), occupational
therapy (OT), and perhaps rehabilitation psychol-
ogy. Because of the progressive nature of FTD and
NCDs in general, patients with these disorders have
not been considered for rehabilitation, but the bias
is changing as the field begins to apply the concepts
and models of rehab to people with NCDs as way
to keep them engaged in life activities and possibly
stave off the decline as long as possible (Buchanan
et al., 2011; Kortte & Rogalski, 2013). Therapies
and interventions must be tailored to meet the spe-
cific and current needs of patients with FTD so it is
difficult to offer a one-size-fits-all recommendation
for interventions. General compensatory strategies
might include using technology to help patients
remember appointments or to take medications; SLP
services can help them focus on maximizing their
communication skills for as long as possible and
introducing augmentative communication devices
or communication boards when necessary. OT
can assist by tailoring and breaking down self-care
tasks, such as dressing, bathing, and feeding, into
manageable steps so that the patient can complete
them for as long as possible. These strategies for
maximizing the patient’s participation in his or her
daily life are also helpful in reducing burden for the
family caregiver (Kortte & Rogalski, 2013). Care-
givers also benefit from psychoeducational support
groups designed to educate them about the nature
of FTD, teach coping strategies, and provide emo-
tional support. The Association for Frontotemporal
Degeneration (http://www.AFTD.org) is the lead
voluntary health organization committed to address-
ing the needs of people with FTD and their family
caregivers, in addition to advocating and supporting
research.
 Neurocognitive Disorders

MAJOR NCD DUE TO LEWY BODY DEMENTIA protein—inside the nuclei of neurons in areas of
the brain that control particular aspects of memory
There are two presentations of Lewy body disease. The
and motor control. Researchers do not know exactly
first is dementia with Lewy bodies (DLB), one of the
why alpha-synuclein accumulates into Lewy bodies
most common types of progressive dementia. The cen-
or how Lewy bodies cause the symptoms of DLB,
tral feature of DLB is progressive cognitive decline (the
but they do know that alpha-synuclein accumula-
cognitive symptoms appear within a year of movement
tion is also linked to PD, multiple system atrophy,
problems), combined with three additional defining
and several other disorders, which are referred to as
features: (a) pronounced fluctuations in alertness
the synucleinopathies.
and attention, such as frequent drowsiness, lethargy,
lengthy periods of time spent staring into space, or dis-
Diagnosis
organized speech; (b) recurrent visual hallucinations;
The similarity of symptoms between DLB and
and (c) Parkinsonian motor symptoms, such as rigid-
PD, and between DLB and AD, makes differential
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ity and the loss of spontaneous movement. The second

diagnosis difficult. In addition, Lewy bodies often
type of Lewy body dementia is Parkinson’s disease
are found in the brains of people with PD and AD.
(PD) in which cognitive symptoms develop more than
These findings suggest that either DLB is related to
a year after the onset of movement problems. As the
these other causes of dementia or that an individual
Lewy body dementia progresses, symptoms of both
can have both diseases at the same time. DLB usu-
types are similar. Patients with either of these diag-
ally occurs sporadically, in people with no known
noses can also suffer from depression, hallucinations,
family history of the disease. Rare familial cases,
and delusions, as concomitant psychiatric symptoms
however, occasionally have been reported.
are common (National Institute on Aging, 2013). Of
note is that PD, PD dementia, and DLB dementia all
Treatment
have the same proteinapthy—abnormality of alpha
Like PD and AD, DLB disease gets worse over time.
synuclein that leads to loss of neurons. The clinical
Currently, there are no disease modifying treat-
presentation differs based on where the alpha synu-
ments, and pharmacologic treatments are aimed at
clein accumulates. If it is restricted to the brainstem
controlling the cognitive, behavioral, and motor
(i.e., substantia nigra), then it’s PD. If it is diffusely
symptoms of the disorder. Acetylcholinesterase
distributed cortically, then it’s Lewy body dementia.
inhibitors, such as donepezil and rivastigmine, pri-
The distinction between PD dementia and Lewy body
marily are used to treat the cognitive symptoms of
dementia is somewhat arbitrary. If motor symptoms
DLB, but they also may be of some benefit in reduc-
precede the dementia, then it’s PD dementia; if the
ing the behavioral and motor symptoms. Physi-
dementia precedes the motor symptoms, then it’s
cians tend to avoid prescribing antipsychotics for
Lewy body dementia.
hallucinatory symptoms of DLB because of the risk
that neuroleptic sensitivity could worsen the motor
Incidence and Prevalence symptoms. Some individuals with DLB may benefit
Lewy body dementia can begin any time between from the use of levodopa for their rigidity and loss
the ages of 50 and 85. It is estimated that 1.3 million of spontaneous movement (National Institute on
individuals in the United States and approximately Aging, 2013).
20% of people with dementia worldwide are affected.
It appears that Lewy body dementia affects slightly
more men than women (National Institute on Aging, MAJOR VASCULAR NCD
2013). Major Vascular NCD also may be referred to as
multi-infarct or poststroke dementia. Individuals
Pathology with major vascular NCD demonstrate cognitive
The symptoms of DLB are caused by the build-up of impairment secondary to infarctions, ischemic
Lewy bodies—accumulated bits of alpha-synuclein injury, or hemorrhagic lesions. The clinical and

259
 Guerriero Austrom et al.
neuropathologic features of this condition vary
widely across individuals.
The clinical features of classic major vascular
NCD generally follow a pattern of abrupt decline in
functioning with gradual improvement that is often
described as a stairstep pattern. Individuals may be
diagnosed with major vascular NCD because of sub-
cortical ischemic pathology that accumulates over
time; this presentation is thought to be most common
(Rocca & Knopman, 2003; Sjogren, Blennow, & Wal-
lin, 2003). For these individuals, they are most likely
to present with a slowly progressive dementia that
often is difficult to distinguish from AD. Problems
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with executive functions (e.g., multitasking, planning,
and organization) are likely to be more pronounced
than memory problems This is not to say that these
individuals do not have problems with new learning
and memory, they do, but these difficulties are more
related to inefficiency as opposed to an amnestic pro-
cess. Finally, the least common presentation emerges
because of a single stroke that affects a specific brain
region that causes significant impairment resulting
from the location of the stroke being in an area critical
for cognitive abilities (e.g., angular gyrus, thalamus,
caudate, heteromodal areas of the cortex, frontal
lobe). Individuals may demonstrate apathy or anxiety,
inattention, memory problems, and general slowing
of thinking, speech, and behavior.
Incidence and Prevalence
Major vascular NCD is the second most common
form of dementia (estimated to account for 15%–20%
of all cases; Rocca & Knopman, 2003). Autopsy find-
ings were consistent with this estimate (reported
18% of cases to have vascular pathology). Mixed
pathology, in which autopsy findings show evidence
of both AD and vascular pathology, is somewhat
common (77% from this sample; Barker et al.,
2002). Risk factors for major vascular NCD include
hypertension, hyperlipidemia, diabetes mellitus, and
smoking (Kuller et al., 2005).
Pathology
For major vascular NCD, pathology does not vary
widely, the underlying pathology will be due to
either ischemic or hemorrhagic causes that involve
the vessel walls.
260
Diagnosis
Diagnosis requires cognitive decline that is related
temporally to cerebrovascular pathology based on
history, examination, and neuroimaging.
Treatment
Treatment for major vascular NCD focuses on opti-
mizing medical management of vascular risk factors
(e.g., hypertension, hyperlipidemia, diabetes) and
promoting a healthy lifestyle. Individuals may be
directed toward programs to stop tobacco use, to
learn more about making nutritious meal choices,
and to engage in a healthy exercise routine.
MAJOR NCD DUE TO TRAUMATIC
BRAIN INJURY
According to the DSM–5 (American Psychiatric
Association, 2013), an individual meets criteria for
major NCD due to TBI when they meet criteria for
a major NCD and there is evidence of a brain injury
with demonstration of at least one of the follow-
ing: loss of consciousness, posttraumatic amnesia,
disorientation or confusion, and neurological signs.
Symptoms must persist beyond the acute period
postinjury and must present directly after the injury
or directly after regained consciousness (American
Psychiatric Association, 2013).
Pathology
TBI has been defined as “an alteration in brain func-
tion, or other evidence of brain pathology, caused
by an external force” (Menon et al., 2010). There
are a few potential mechanisms for a TBI: a blow to
the outside of the head, a penetrating wound (e.g.,
shrapnel, gunshot, or some other object), and shak-
ing or sudden deceleration of movement causing the
brain to have an impact with the skull. In addition,
damage may be focal or diffuse. Depending on the
mechanism and location of the injury, cognition,
behavior, and emotional regulation may be affected
in many ways.
Incidence and Prevalence
TBI is considered a universal public health issue.
In the United States, it has been estimated that the
incidence of TBI is 100 per 100,000; prevalence

is estimated at 2.5–6.5 million individuals. Other
estimates include about 1.4 million injuries per
year, with 50,000 deaths and 235,000 hospitaliza-
tions (Langlois, Rutland-Brown, & Wald, 2006). In
regards to people admitted to hospitals with TBI in
the United States, several studies reveal that approx-
imately 80% are classified as mild, 10% as moder-
ate, and 10% as severe (Kraus & McArthur, 1996).
Concerns have been raised about the relationship
of TBI to dementia. Many individuals with TBI
who have significant impairments in memory and
executive function meet the criteria for dementia.
The larger issue, however, is whether exposure to
Copyright American Psychological Association. Not for further distribution.
a TBI increases the risk of a progressive dementing
disorder such as AD later on. At this time, it is not
possible to say definitively whether TBI, particularly
mild TBI, is a risk factor for AD (McAllister, 2008).
A review by Jellinger (2004), concluded that both
AD and TBI are associated with abnormalities in
amyloid and tau protein deposition and that several
epidemiological studies have suggested either that
AD occurs with increased frequency in individuals
with TBI or that the age of onset of the disease is
reduced after TBI when compared with noninjured
controls. The reduced cognitive reserve associated
with TBI may facilitate earlier symptom manifesta-
tion of dementia in individuals likely to develop AD
(Starkstein & Jorge, 2005). Plassman et al. (2000)
found an increased risk for dementia associated with
both moderate and severe head injury.
Treatment
Anyone with signs of moderate or severe TBI
should receive medical attention as soon as pos-
sible. Because little can be done to reverse the initial
brain damage caused by trauma, medical personnel
try to stabilize an individual with TBI and focus on
preventing further injury. Primary concerns include
ensuring proper oxygen supply to the brain and the
rest of the body, maintaining adequate blood flow,
and controlling blood pressure. Imaging tests help
to determine the diagnosis and prognosis of a TBI
patient. Moderately to severely injured patients may
receive rehabilitation that involves individually tai-
lored treatment programs in the areas of physical
therapy, occupational therapy, speech and language
therapy, physiatry (physical medicine), psychology
Neurocognitive Disorders
and psychiatry, and social support. In cases in which
dementia is present along with behavioral chal-
lenges, psychiatry and psychology can be most help-
ful by conducting neuropsychiatric, environmental,
and behavioral assessments with both the patient
and the family caregiver. To ignore the environ-
ment and factors that may be provoking challeng-
ing behaviors will greatly reduce the efficacy of any
prescribed medication, even if it is the proper one.
Conversely, without the properly prescribed medi-
cation, even extensive efforts at applying behavioral
analysis and environmental modifications may prove
ineffective. As McAllister (2008) aptly suggests the
therapeutic question should not be, “Do we pre-
scribe a drug, or write a behavioral plan?” The ques-
tion is better framed as, “Which is the best medicine
prescribed in the context of what changes in envi-
ronment and strategies for shaping behavior has the
best potential for success?”
Psychological Treatments
More than 90% of patients with NCD will experience
behavioral and psychological symptoms at some point
during the course of their illness (Haupt, Kurz, &
Janner, 2000). These symptoms, which include
a broad range of distressing behaviors—agitation
and aggression, disinhibition—and psychologi-
cal reactions—depression, apathy, paranoia, and
hallucinations—affect the health and quality of life of
both the patient and the caregiver. Leaving patients’
behavioral and psychological symptoms of NCD or
dementia untreated has been associated with care-
giver burnout, earlier nursing home placement, poor
management of comorbid conditions, and excess
health care costs (Callahan et al., 2006; Mittelman
et al., 1996).
Psychosocial or nonpharmacological interven-
tions have received much attention in the literature,
with evidence suggesting that many of these are
effective in reducing both caregiver distress and
behavioral distress, particularly in patients with
NCD resulting from AD. Some examples of these
interventions include hand massage with lemon
balm or spraying a 2% solution of lavender oil in a
special care unit for patients with dementia, which
reduced agitated behaviors (Ballard et al., 2002;
Holmes et al., 2002). Another study found that a
261
 Guerriero Austrom et al.
15-min audiotape made by a family member reduced
agitated behaviors when played through headphones
(Garland et al., 2007). Physical exercise is beneficial
to patients with dementia. A daily, 30-min active
exercise program demonstrated a bigger improve-
ment in mood than participation in either a gentle
walking group or a conversation group (Williams &
Tappen, 2007). Reducing or eliminating distract-
ing stimuli—harsh lighting, noisy televisions, and
radios—in the physical environment is also helpful
in keeping the patient with AD and dementia calm.
Tailoring activities and interventions to the individ-
ual patient is critical, as many distressing behaviors
Copyright American Psychological Association. Not for further distribution.
change or even disappear over time and not all inter-
ventions work for all patients.
Less research is available on psychosocial inter-
ventions in patients with other forms of NCDs;
however, the authors have found that working with
patients and families affected by several forms of
NCDs, psychosocial interventions have been effec-
tive. In a clinical trial of collaborative care (Callahan
et al., 2006) in which psychosocial and pharmaco-
logical treatment were integrated within the con-
text of primary care, it was demonstrated that the
comprehensive care approach resulted in clinically
significant improvements in behavioral symptoms of
dementia and NCD. The improvements were accom-
panied by a reduction in caregiver stress. Examples
of interventions for common behavioral and psychi-
atric symptoms of dementia and NCD are presented
in Table 9.2 (Callahan et al., 2006; Guerriero Aus-
trom et al., 2004, 2005, 2006).
When caring for patients with NCDs, psycholo-
gists must include the family caregiver in the treat-
ment plan. Indeed, partnering with the family
caregiver is key to the successful diagnosis, treat-
ment, and management of all NCDs. Most important
for family caregivers is that early diagnosis allows
the patient and family time to plan for future needs,
such as executing a power of attorney, appointing
a health care representative, ensuring advanced
care planning, and making important legal and
financial decisions because as the NCDs progress,
the patient’s decision-making abilities decrease.
Patients and family caregivers often fear that after
the diagnosis, the clinician will abandon them
because of the stigma associated with dementia and
262
NCDs (Boustani et al., 2011; Fowler et al., 2012). It
is essential that clinicians convey to the family that
they will continue to be involved with the patient
and family and that management issues will be
reviewed as they arise (Callahan et al., 2011; Guer-
riero Austrom & Lu, 2009; McKhann et al., 2011).
Because of the changing nature of the NCDs over
time, clinicians must plan for fluctuations in patient
behavior, patient function, and the caregiver’s ability
to cope, and therefore, they must continue to sup-
port the family over time. Interdisciplinary health
care providers, working together, should educate
the patient and the family regarding disease progres-
sion and prognosis, provide support and sources of
information (a resource list is provided in Appendix
9.1), and monitor judgment and safety issues so that
the patient can remain independent or community
dwelling as long as possible (Boustani et al., 2011;
Callahan et al., 2012; Guerriero Austrom, Lu, &
Hendrie, 2013).
MAJOR ACCOMPLISHMENTS
Although the initial descriptions of AD and other
related disorders was published more than 100
years ago, the major accomplishments in the under-
standing, assessment, diagnosis, treatment, and
possible prevention of NCD have occurred within
the past 20 years or so. There has been a tremen-
dous increase in the amount of research on under-
standing the causes of the NCD, the use of various
biological markers or biomarkers to diagnose the
disorders earlier, and the search for potential treat-
ments and prevention strategies. The National
Institute of Aging together with the Alzheimer’s
Association convened expert workgroups to update
the diagnostic criteria and guidelines for AD
(National Institutes of Health, 2007). This was
important an advancement as the guidelines had not
been updated since 1984.
Currently, diagnosis relies on documenting cog-
nitive decline. Researchers now believe that by the
time patients get a diagnosis, the neuropathologi-
cal changes have been occurring for years, if not
decades. Researchers hope to find an easy and accu-
rate way to detect such changes before these devas-
tating symptoms begin. Several potential biomarkers
 Neurocognitive Disorders

TABLE 9.2

Recommended Psychosocial Interventions for Common Behavioral Challenges Associated With NCDs

Behavioral symptoms and

common challenges Intervention
Delirium Prevention of cognitive impairment
Orient to person, place, and time; daily schedule
Offer stimulating activities multiple times per day
Ambulation
Avoid leaving patient alone
Prevention of sleep deprivation
Soothing music
Massage
Copyright American Psychological Association. Not for further distribution.

Warm milk, herbal tea

Reduce noise in room
Depression and anxiety Maintain a cheerful and bright environment
Encourage mild to moderate exercise
Encourage participation in enjoyable and meaningful activities
Promote positive social interactions
Redirect the patient when he/she expresses negative thoughts or feelings
Validate feelings and offer comfort when person is in distress
Aggression/agitation/catastrophic reactions Identify potential triggers of emotional outbursts to prevent future outbursts
Establish a calm and predictable environment
Use nonthreatening, calm, and gentle approach
Offer reassurance
Avoid arguing or direct confrontation
Do not try to reason or use logic
Try to distract and redirect the patient
Move them to a quiet and safe place
Delusions/hallucinations/paranoia Give noncommittal responses
Avoid arguing or direct confrontation
Avoid reasoning or rationalizing with the patient
Try to distract and redirect the patient
Repetitive behaviors Reassure and comfort the patient
Ignore repetitive questions or answer them calmly
Recognize that the patient no longer remembers how to gain attention
Redirect attention if and when possible
Recognize their nonverbal cues
Find enjoyable repetitive tasks based on patient’s current
abilities and social history (e.g., sorting/folding laundry; hitting golf balls at the
driving range; meaningful crafts and chores)
Apathy Understand it is a common effect of NCDs
Encourage exercise especially outdoors in nice weather
Identify meaningful and enjoyable activities
Encourage social engagement (e.g., family and friend visits)
Sleep problems Develop a good sleep hygiene routine
Encourage appropriate levels of activity and exercise during the day
Avoid caffeine late in the day
Day time naps should not be taken in the bedroom
Keep day time naps short
(Continued)

263
 Guerriero Austrom et al.
Recommended Psychosocial Interventions for Common Behavioral Challenges Associated With NCDs
Behavioral symptoms and
common challenges
Mobility
Copyright American Psychological Association. Not for further distribution.
Activities of daily living
Disinhibition
Reducing disability
Caregiver support and education
264
TABLE 9.2 (Continued)
Intervention
Fall prevention
Provide a safe and secure environment
Remove throw rugs, secure electrical cords
Install grip bars in bathroom and kitchen
Encourage walking, use canes and walkers if necessary
Shadowing
Reassure the patient that he/she is safe
Encourage patience and tolerance of the behavior
Encourage caregiver to seek respite and a break
Wandering
Enroll the patient in the Alzheimer’s Association Medic Alert Safe Return program or
other tracking system
Offer appropriate levels of exercise
Provide the patient a safe place to wander (e.g., a fenced yard)
Install safety locks or alarms on windows and doors
Driving
Discuss driving cessation with patient and physician
Schedule a driving assessment by an objective third party
Develop a transportation plan if and when driving is no longer possible
Enlist the help of a trusted professional (e.g., lawyer, insurance agent)
Encourage independent functioning for as long as possible
Adapt activities to the patient’s current level of functioning (e.g., if using cutlery is
difficult, serve finger foods; reduce clothing options and simplify decision making)
Encourage a toileting schedule
Simplify bathing routines
Understand this is common in NCDs, especially behavioral variant FTD
Check environment for conditions that may aggravate the behavior (e.g., temperature
too high may encourage disrobing)
Do not overreact and try to distract and redirect
Approach the patient calmly and move them to a private and quiet room
Support family, especially younger children and teenagers
Based on functional assessments, collaborative care plans should involve speech,
occupational and physical therapy as needed and indicated
Can be particularly beneficial in mild cognitive impairment, TBI, and vascular dementia
Provide information on available community resources
Legal and financial decisions need to be addressed sooner rather than later
Encourage support group participation, in person or online
Provide education on issues as they arise; do not overwhelm with caregivers
Discuss NCDs and progression routinely so that changes do not take caregivers by
surprise
Stress how common the behaviors and symptoms are in NCDs thereby normalizing
them
Encourage caregivers to take care of themselves, physically and emotionally—take
medications, visit their primary care physicians, self-care, and stress-reduction
strategies
Suggest they seek and use respite care on a regular basis (e.g., adult day programs,
home health aids, friendly visitors)

are being studied for their ability to indicate early
stages of AD—for example, beta-amyloid and tau
levels in cerebrospinal fluid and brain changes that
are detectable by imaging. Recent research suggests
that these biomarkers may change at different stages
of disease (Jack et al., 2011).
Another accomplishment is a coordinated and
concerted effort to address the challenges of AD
as expressed in the National Alzheimer’s Proj-
ect Act in 2011 and the National Plan to Address
Alzheimer’s Disease by the President in 2012. The
Nation Plan calls for increased federal funding for
AD research, support for those affected by AD and
Copyright American Psychological Association. Not for further distribution.
their families, increased public awareness about
AD, and improved data collection and analysis to
understand the impact of AD on people with the
disease, families, and the health and long-term care
systems (U.S. Department of Health and Human
Services, 2013). These goals apply to AD-related
dementias, including, dementia with Lewy bodies,
frontotemporal, and vascular dementias. Having an
overall national plan has positively affected the pace
of research and the large-scale collaborative efforts
at answering the most important questions about
NCD.
FUTURE DIRECTIONS
NCDs are complex and challenging disorders to
work with because of the deterioration in cognition
over time. In addition, psychologists must work with
both the patient and a key family member or other
care partner as the diseases progresses. Most difficult
is the fact that no effective disease-alternating treat-
ments exist for these disorders.
Identifying effective models of care for patients
with NCD and their caregivers is an important area
of future development. Research into collabora-
tive care in primary care (such as medical homes),
specialty care clinics, and community settings has
demonstrated positive results, although large-scale
implementation of these models have yet to be
demonstrated. Several areas of promising research
include trials to determine the efficacy of drugs,
exercise, and neuroimaging. The results of these
efforts, if positive will certainly affect and help set
the direction for additional research.
Neurocognitive Disorders
Drugs
A number of agents that might slow the progres-
sion of AD and other disorders are in various stages
of testing. The Alzheimer’s Disease Cooperative
Study (ADCS) is currently testing drug and exercise
interventions in people in the early stages of the
disease, examining medication to reduce agitation
in people with AD (Prazosin for Treating Agitation
Trial), and testing a cutting-edge approach to speed
testing of drugs in clinical trials. The A4 trial (Anti-
amyloid Treatment in Asymptomatic Alzheimer’s
Disease) is testing a promising therapy in the early
stages of the disorder. This prevention trial will test
an amyloid-clearing drug in the symptom-free stage
of the disease in 1,000 cognitively healthy older
volunteers whose brain scans show abnormal levels
of amyloid accumulation (National Institutes of
Health, 2013).
Exercise
Researchers are evaluating the effectiveness of a
supervised aerobic exercise program to enhance
general cognition in adults with age-related cogni-
tive decline. It is predicted that individuals with
greater fitness gains will make greater cognitive
gains. Another study will determine whether exer-
cise prevents memory loss from getting worse and
whether it improves daily functioning and attitudes
of those with probable AD.
Genetics
Many dementia-related disorders share genetic
and other characteristics of AD. Researchers are
studying the genome to identify genes that may be
responsible for the development of AD and related
dementias. Recent research on the role of ApoE in
the development of late-onset AD found that one of
the three forms of ApoE triggers an inflammatory
reaction and damages blood vessels that feed the
brain. Genetic research has identified a gene variant
for TREM2 that is involved with a form of FTD that
runs in families. Future research may identify novel
genes involved with FTD and other NCDs and pos-
sibly lead to therapeutic approaches in which the
delivery of normal genes might improve or restore
normal brain function (National Institutes of Health,
2013).
265
 Guerriero Austrom et al.
Neuroimaging
Clinical imaging is helping researchers understand
changes in brains of people with dementia as well
as helping diagnose these disorders. MRI may reveal
structural and functional differences in the brains
of people with AD and PD dementia. PET scanning
research uses ligands, radioactive molecules that bind
to proteins to show chemical functions of tissues
and organs in the body, to produce images of brain
activity. Researchers are testing new PET ligands that
bind to beta-amyloid for the early detection of AD-
type pathology and to tau to help in people with very
early AD (National Institutes of Health, 2013).
Copyright American Psychological Association. Not for further distribution.
Proteins
A common feature in the pathology of NCDs is
excess proteins or protein fragments thought to be
toxic to brain cells. Current research is trying to
better understand the toxic effects of the protein
buildup and how it is related to the development of
AD and related dementias. Some protein abnormali-
ties can be identified in cerebrospinal fluid. Abnor-
mally high accumulations of beta-amyloid protein
in the brain is a hallmark of AD; some research
is directed at finding which neural pathways are
affected by the beta-amyloid and contribute to the
development of AD pathology. The protein tau
accumulated in abnormal tangles that disrupt nerve
signaling cause cell death and affect cognition in
people with AD, FTD, and other NCDs.
How we prepare future professionals to care for
the increasing numbers of patients with NCD will
require the coordinated efforts among multiple dis-
ciplines. The Institute of Medicine has identified
interprofessional collaboration as an essential part
of improving the accessibility, quality, and value of
health care in the United States. The World Health
Organization (2010) described interprofessional
education (IPE) as “when students from two or
more professions learn about, from and with each
other to enable effective collaboration and improve
health outcomes” and stated that “interprofessional
education is a necessary step in preparing a ‘collab-
orative practice-ready’ health workforce that is bet-
ter prepared to respond to local health needs” (p. 7).
Interprofessional team-based care continues
to grow, with increasing recognition that health
266
outcomes improve when different professions work
together (e.g., Grumbach & Bodenheimer, 2004;
Interprofessional Education Collaborative, 2011;
Leape et al., 2009; Reeves et al., 2013). Consensus
is wide surrounding the need to create opportuni-
ties for health professions students to learn together
through IPE, to allow for new team care approaches
in patient care (Sullivan & Godfrey, 2012). As a
result, requirements for IPE have been integrated
into accreditation standards in many disciplines
(Zorek & Raehl, 2013), further increasing the
demand for IPE and effective assessments in profes-
sional training. Improved training in cognitive aging,
neuroanatomy, and neurodegenerative diseases
should occur at multiple levels, including gradu-
ate and internship preparation for psychologists as
well as medical school and residency training for
physicians. IPE training should include instruction
in terms of treatments and resources focused on the
psychology of aging, elder care, and elder law.
The future goal for the care for NCD is to find a
prevention or cure for these challenging disorders,
but in the meantime, care for those with NCD and
their caregivers must address their physical, cogni-
tive, and emotional well-being.
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