UPTODATE

Frontotemporal dementia: Clinical features and diagnosis

Author Christopher Randolph, PhD, ABPP-CN

Section Editor Steven T DeKosky, MD, FAAN, FACP
Deputy EditorApril F Eichler, MD, MPH
All topics are updated as new evidence becomes available and our peer review
process is complete.
Literature review current through: Jun 2014. | This topic last updated: Jun 27,
2013.
INTRODUCTION AND NOMENCLATURE Frontotemporal dementia (FTD) is a
neuropathologically and clinically heterogeneous disorder characterized by focal
degeneration of the frontal and/or temporal lobes [1]. Age of onset is typically in the
late 50s or early 60s, and the primary initial clinical manifestations are changes in
personality and social behavior or language, progressing over time to a more global
dementia. A subset of patients may also exhibit symptoms of extrapyramidal or motor
neuron involvement at some point in the disease process.
For many years the clinical syndrome was known as Picks disease after Arnold Pick,
who originally described the behavioral variant of this syndrome in 1892 [2]. A variety
of terms to describe the clinical syndrome have ensued, including:
Frontal lobe dementia
Frontal lobe degeneration
Frontotemporal lobar degeneration
Pick complex
The preferred terminology for the clinical syndrome is frontotemporal dementia [3,4].
However, the term remains somewhat unclear. While frontotemporal dementia is often
meant to encompass the entire syndrome complex that is associated with
degeneration of the frontal and temporal lobes, at other times frontotemporal
dementia is used to describe the behavioral variant specifically. In this topic,
frontotemporal dementia will be used to describe the entire syndrome as a whole, and
the behavioral variant will be specified when that subgroup is specifically described.
FTD subtypes are also subject to the use of evolving and inconsistent nomenclature.
As an example, the terms frontal variant and temporal variant are often applied to
behavioral variant FTD and to semantic dementia respectively. Because the implied
neuroanatomic associations are not perfect, we prefer using the clinical rather than
the anatomic terms to describe the clinical syndrome [5,6]. Finally, the term primary
progressive aphasia originally described a series of patients with a progressive
nonfluent aphasia [7]. As another syndrome characterized by a progressive fluent
aphasia was increasingly recognized, some authors categorized this as a subset of
primary progressive aphasia, while others, recognizing the frequent (but not

invariable) presence of more extensive cognitive deficits in these patients, coined the
term semantic dementia. We will use the terms progressive nonfluent aphasia and
semantic dementia to describe these syndromes, recognizing that some patients may
fall into an intermediate category.
This topic describes the clinical features and diagnosis of frontotemporal dementia.
Other dementia syndromes are described separately. The epidemiology, pathology,
pathogenesis, and treatment of frontotemporal dementia are also discussed
separately. (See "Clinical manifestations and diagnosis of Alzheimer disease" and
"Etiology, clinical manifestations, and diagnosis of vascular dementia" and "Clinical
features and diagnosis of dementia with Lewy bodies" and "Cholinesterase inhibitors
in the treatment of dementia" and "Treatment of behavioral symptoms related to
dementia"
and
"Frontotemporal
dementia:
Epidemiology,
pathology,
and
pathogenesis" and "Frontotemporal dementia: Treatment".)
CLINICAL FEATURES Frontotemporal dementia (FTD) typically presents as either a
progressive change in personality and social behavior or as a progressive form of
aphasia; in both cases continuing ultimately to a global dementia. In a minority of
patients, motor symptoms may follow the initial manifestation of the disorder. While a
distinctive clinical syndrome may be identifiable in most patients as the disease
evolves, in some series, clinicians have noted that symptoms overlap considerably
and that some patients do not clearly fall into one category over another, particularly
in the early stages [8].
FTD appears to progress more rapidly than Alzheimer disease [9-11]. The duration of
disease from diagnosis to death is not well established, but between four to eight
years appears to be usual [12-17]. Survival time appears to be shorter for individuals
with the motor neuron variant of FTD [13].
Behavioral variant The most common presentation of FTD is progressive change
in personality and behavior; this is the so-called behavioral or frontal variant of FTD
(bvFTD) [6,8]. Common behavioral features include [5,16,18-32]:
Personality change. This can manifest as apathy with social withdrawal, loss of
spontaneity, and abulia that can be mistaken for depression. In contrast, some
patients develop social disinhibition and impulsivity, leading them to act out in various
ways, ranging from expressions of excessive sentimentality to violent acts of
aggression. Some patients alternate between passive behavior and disinhibited
outbursts.
Lack of insight. Most patients with bvFTD seem unaware or incompletely aware of
their deficits at presentation, and virtually all exhibit impaired insight within two years
of symptom onset. On probing, this is often a lack of concern rather than a lack of
knowledge of their impairments [33].

Loss of social awareness. Individuals with bvFTD may infringe upon social norms in a
manner that is incongruent with their premorbid behavior. Their sense of decorum
appears altered; they may make offensive remarks and behave inappropriately.
Personal hygiene may be affected. Patients may be incontinent voiding urine or
feces in inappropriate places without apparent concern. They may commit antisocial,
even criminal acts. While inappropriate sexual comments are somewhat common in
patients with bvFTD, hypersexual behavior is not; most patients with FTD have
diminished libido.
Stereotyped or ritual behaviors. These can include insisting on the same foods or
employing a repetitive "catch-phrase." Hoarding, counting, and pacing are among the
described behaviors.
Change in eating patterns. Patients with bvFTD frequently overeat and may binge or
develop food fads. Alcohol consumption may be excessive. As the disease progresses,
there may be oral exploration of nonfood objects.
Emotional blunting and loss of empathy. Patients may be described as more selfcentered, unconcerned about family and friends, and "cold." They may have difficulty
recognizing emotional expression in others [34].
Mental rigidity. Patients with bvFTD often appear inflexible in their adherence to
routines, as well as unable to adapt to new situations and see another's point of view.
Deficits in modulating attention. This can manifest as distractibility and
impersistence, or as perseverative behavior. In addition, some patients, 14 of 16
patients in one series, demonstrate utilization behaviors [35]. These are stimulusbound actions in which the individual repeatedly uses and reuses objects within their
sight, despite irrelevance to situation (eg, they might repeatedly pick up a comb in
front of them and use it while participating in a conversation).
These behavioral changes are presumed to reflect early involvement of orbitofrontal
systems [5,21,22,36]. While sometimes referred to as frontal variant FTD, significant
temporal lobe pathology is also evident in some of the behaviors, as well as on
neuroimaging and neuropathologic examination of patients with bvFTD [5,15,16].
Socially aberrant behavioral changes have been associated with greater involvement
of the right hemisphere [21,37,38]. These behavior changes are useful in
distinguishing FTD from other forms of degenerative dementia [21,25,39-41]. (See
'Differential diagnosis' below.)
Cognitive functioning may be relatively intact early in the course of the disease for

patients with bvFTD [42]. Such patients may have normal mini-mental state
examination scores. Even tests of executive function may be normal, since success on
these tests is largely mediated by dorsolateral prefrontal systems, which are relatively
spared in early bvFTD [5,21,22,36,43-45]. As the disease progresses, however,
neurocognitive deficits emerge, particularly impairment of executive functions,
problem-solving, judgment, attention, organization, and planning. In contrast, memory
and visual perceptual and spatial skills are better preserved, although rarely normal
[16,40,46,47].
A less commonly-recognized form of bvFTD involves a progressive difficulty with
organizational and executive skills, with behavioral changes that are more subtle, at
least initially [18]. This is presumed to reflect greater early involvement of dorsolateral
prefrontal systems (particularly on the left), and can be particularly difficult to
diagnosis in the early stages.
Altered speech patterns, while not as prominent as in the semantic or nonfluent
aphasia subtypes of FTD, are also present and can manifest either by aspontaneity
and paucity of speech output or as an increased, often pressured, speech pattern [48].
Other abnormal language features may include stereotypy (single words or phrases
used repeatedly), echolalia, perseveration, and in late stages, mutism.
Frontal release signs may be seen (grasp, snout, and sucking reflexes, incontinence) at
a somewhat earlier disease stage than is typical for patients with Alzheimer disease.
Progressive aphasias Three distinct forms of progressive aphasia are recognized;
although not all patients fall clearly into one category or another [49].
Progressive nonfluent aphasia Somewhat less common than bvFTD is a
presentation with a progressive nonfluent agrammatic aphasia (PNFA). Anomia is the
first deficit to appear [6,14,50-53]. This manifests as word-finding difficulty and
impaired object naming. Spontaneous speech becomes increasingly dysfluent. Speech
errors, including simplification, circumlocution, and phonemic paraphasic errors, may
become evident. While grammar, repetition, and comprehension are spared in early
stages, agrammatism with a progressive telegraphic-type speech pattern develops
and comprehension for grammatically complex language becomes impaired.
Ultimately, patients have increasing difficulties with comprehension and may become
mute. (See "Approach to the patient with aphasia".)
Social comportment, memory, visual spatial skills, and other cognitive abilities are
typically preserved at the time of presentation [14,19,30,54]. While patients usually
have retained insight, they may seem unconcerned [29]. Deficits may be restricted to
expressive language function for several years before a more global dementia
supervenes [54,55]. Some patients go on to develop behavioral alterations or
symptoms of motor neuron disease or corticobasal degeneration [39,54-56]. (See
'Motor syndromes' below.)
Semantic dementia Semantic dementia (SD) is sometimes referred to as the

temporal variant of FTD. This usually manifests initially as a progressive speech

disturbance with normal fluency, but impaired comprehension, anomia, and semantic
paraphasias [6,14,19,22,30,57-59]. Repetition is generally normal. In many respects,
the appearance is similar to a transcortical sensory aphasia (see "Approach to the
patient with aphasia").
Patients with SD appear to have incomplete awareness of their deficit; they are more
apt to acknowledge the word-finding difficulty than impaired comprehension. Many
patients with SD also manifest a so-called surface dyslexia and dysgraphia; they can
read and spell common, but not irregular, words, make regularization errors, and read
and write phonetically (eg, when asked to read or write the word "shoe" they may
write "shoo" and read "show") [19]. On verbal fluency tests (generation of word lists in
one minute's time), patients with SD characteristically have more difficulty with
semantic/category fluency (eg, lists of animals) than on a letter fluency test (eg, lists
of words beginning with F), while patients with PNFA and bvFTD have equal
impairments on both tests [60]. Normal performance is age related, with at least 15
items expected at age 65 [61].
In many patients, it is apparent that the language deficit is a manifestation of a
broader impairment, and that visual object recognition, as well as anomia, is present
[19,30,57,59]. This suggests a primary deficit in semantic memory, which is that
aspect of long-term memory that contains knowledge of objects, facts, and concepts,
as well as of words and their meaning. At the same time, episodic memory (that of
autobiographical events) is relatively preserved and patients may perform surprisingly
well, at least initially, in their home life [19].
Right temporal lobe deficits are often less obvious than language deficits, but these
can include difficulty recognizing faces, as well as difficulty recognizing voices of
people familiar to them [22,59,62].
While social comportmental behavior is usually normal at presentation, behavioral
problems typical of bvFTD are more common and present earlier in SD than in PNFA,
usually within a few years of presentation [20,30,39,57,59,63,64].
Logopenic phonological aphasia A third category of progressive aphasia has
been more recently defined. In the logopenic presentation, there is an overall paucity
and slowness of speech output with deficits in naming and word retrieval, along with
phonemic paraphasias [65-69]. Grammar and syntax are relatively normal early on,
but repetition and comprehension, even for simple sentences, are often found to be
impaired. (See "Approach to the patient with aphasia".)
Patients with logopenic aphasia also tend to have more difficulty with episodic
memory and with calculations compared to those with other forms of aphasia [65-68].
As with PNFA, apathy is typically prominent, but some patients are notably irritable,
anxious, and/or agitated.
Motor syndromes Some patients with FTD will develop a syndrome of motor

impairment during the course of their disease. Such syndromes include:

Motor neuron disease Motor neuron disease (MND) may precede or follow the
development of dementia, which is usually of the bvFTD type [6,70-74]. Some studies
suggest that as many as 50 percent of patients with MND have or develop dementia;
the incidence of subsequent MND in patients who present initially with FTD is less
certain. In one study of 36 consecutively diagnosed patients with FTD, five had
definite MND, and two additional patients had abnormal electromyography that was
consistent with MND [75]. In patients with MND and FTD, the motor presentation is
usually that of progressive muscular atrophy with flaccidity and fasciculations
affecting the bulbar muscles and upper extremities primarily. Upper motor neuron
signs may be less prominent. In patients who develop FTD-MND, clinical features of
both syndromes are usually apparent within two years of initial presentation;
progression to death is more rapid in these patients. (See 'Clinical features' above.)
FTD-MND is discussed separately. (See "Clinical features of amyotrophic lateral
sclerosis and other forms of motor neuron disease", section on 'Cognitive symptoms'
and "Familial amyotrophic lateral sclerosis", section on 'C9ORF72 gene'.)
Corticobasal degeneration Corticobasal degeneration is a syndrome of
asymmetric rigidity and apraxia [76]. A characteristic feature is the alien limb
syndrome in which the affected limb makes uncontrolled spontaneous movements
that sometimes resemble choreoathetosis. Patients often try to restrain the limb with
the contralateral arm. This is a self-limited phenomenon that becomes superseded by
rigidity. Other clinical features can include dystonia, reflex myoclonus, impaired
cortical sensory loss (ie, impaired joint position, 2-point discrimination, agraphesthesia
in the setting of intact primary sensory modalities), and mirror movements. Gait
impairment and supranuclear gaze palsy spread to the less affected side as the
disease progresses.
Cognitive impairment develops in most patients with CBD, and CBD may subsequently
develop in some patients with FTD, particularly those with PNFA [30,55,74,77,78].
Progressive supranuclear palsy Progressive supranuclear palsy (PSP) is a
clinical syndrome of supranuclear vertical gaze palsy, axial dystonia, bradykinesia,
rigidity, and falls [79,80]. Motor findings are usually symmetric in onset and most
patients have a dementia syndrome with features that overlap with bvFTD [81]. (See
"Diagnosis of Parkinson disease", section on 'Progressive supranuclear palsy'.)
Conversely, patients who meet standard clinical criteria for the diagnosis of motor
neuron disease (MND), corticobasal degeneration (CBD), or progressive supranuclear
palsy (PSP) may at some time during the course of their illness exhibit cognitive or
behavioral
features
suggestive
of
a
frontal
lobe
dementia
[5,21,22,36,43,44,70,78,82,83].
Others Case series have described other patients with some clinical features of
FTD along with other neurologic features that correspond to areas of regional

involvement on neuroimaging. Not all of these have neuropathologic correlation and

therefore the relationship of these syndromes to FTD is often unproven.
As an example, one report describes 20 patients with right temporal lobe atrophy
(similar to the pattern described in the left temporal lobe in patients with semantic
dementia) who had on presentation complaints of getting lost and marked
prosopagnosia along with the more typical impaired episodic memory and
characteristic behavioral disturbances associated with bvFTD [84].
DIFFERENTIAL DIAGNOSIS Any of the neurodegenerative dementias may be
considered in the differential diagnosis of FTD. Considered diagnoses will vary
according to the clinical syndrome.
For bvFTD, psychiatric disorders (depression, obsessive compulsive disorder, bipolar
disorders) are commonly considered, as well as Alzheimer disease (AD) and other
neurodegenerative dementias [19,85,86]. The typical age of onset for bvFTD is
somewhat younger than most patients with AD but somewhat old for a first
presentation of major psychiatric disease. However, there is substantive overlap in the
age range at presentation among these disorders. Major psychiatric symptoms of
psychosis (frank delusions, hallucinations) are uncommon in bvFTD; however these
are not invariable symptoms in psychiatric disease [8,32,87]. In some patients,
treatment trials and clinical follow-up are required to establish the diagnosis of bvFTD
versus psychiatric disease. Because it is better known, the diagnosis of AD is not
infrequently given to patients with FTD. However, the younger age at onset, the
prominent neurobehavioral features, and the absence of significant amnesia on the
initial examination are typical for bvFTD and atypical for AD and for other causes of
dementia [26,27,32,40,41,43,88,89]. However, there is some clinical overlap between
the conditions, and occasionally at postmortem, a patient who appeared to meet
clinical criteria for AD will be found to have FTD and vice versa [8,90,91]. While
patients with dementia with Lewy bodies can have prominent neuropsychiatric
symptoms, these usually take on a different form, in particular the early presentation
of hallucinations (usually visual), a feature that is uncommon in patients with early
bvFTD. (See "Clinical features and diagnosis of dementia with Lewy bodies".)
Structural disease of the frontal lobes (infarction, tumors, abscess, trauma) can also
produce a clinical picture that is similar to bvFTD, however, the onset and course of
these diseases are quite different. In general, neuroimaging can reliably exclude these
entities.
The differential diagnosis of progressive aphasia includes AD, vascular dementia, and
other neurodegenerative dementias, as well as slowly growing mass lesions such as a
primary brain tumor [14,53,55,92-94]. One clinical neuropathologic study found that
one-third of patients with either PNFA or SD had AD rather than FTD pathology at post
mortem [14,91]. The neuroanatomic distribution of the AD pathology in these patients
was atypical. (See "Clinical manifestations and diagnosis of Alzheimer disease",
section on 'Primary progressive aphasia'.) Another case series reported that 14
percent of their patients with progressive aphasia ultimately met clinical criteria for

dementia with Lewy bodies [55].A neuroimaging study, usually brain magnetic
resonance imaging study, is important to exclude mass lesions and other structural
pathology in patients with primary aphasia. (See "Approach to the patient with
aphasia".)
DIAGNOSIS The diagnosis of frontotemporal dementia (FTD) is made primarily by
clinical assessment. Neuroimaging studies are required to exclude alternative
pathologies and may provide supporting findings. Neuropsychological testing can be
helpful in patient management but is not diagnostic. Other laboratory tests are
typically performed to exclude potentially reversible contributors or causes of the
cognitive impairment. (See "Evaluation of cognitive impairment and dementia".)
Clinical assessment Consensus clinical criteria specific to the three major clinical
syndromes (table 1 and table 2 and table 3) were published in 1998 [48].
These clinical criteria have subsequently been shown to have substantive limitations,
particularly for the behavioral variant syndrome [95-98]. As an example, the
requirement for all five of the core diagnostic features is likely too restrictive, limiting
the sensitivity for diagnosis, especially at clinical presentation. Also, the supportive
features, while viewed as useful in clinical practice, have no formal role in the
diagnosis. Further research on the prevalence and reliability of clinical characteristics
individually and in combination is needed.
Nonetheless, clinical-pathologic studies suggest that bvFTD can be fairly reliably
diagnosed in life. A report of abnormal behaviors is more helpful in making the
diagnosis, particularly at early stages, than are cognitive assessments. These are also
helpful in distinguishing bvFTD from AD [27,43]. Patients are not reliable informants of
these behavioral problems and interviewing family members is important [41]. Studies
vary somewhat in their conclusions about which behavioral features are most helpful
diagnostically. However, in general, the presence of abnormal social conduct, eating
disorders, stereotyped behaviors, and akinesia/apathy along with the absence of
significant memory or visual spatial deficits are very specific (close to 99 percent) and
moderately sensitive (80 to 85 percent) for the diagnosis of bvFTD
[1,24,25,40,74,89,99-101].
Most patients who present with a syndrome of progressive aphasia consistent with
either PNFA or SD are found to have FTD at post mortem. However, a significant
minority, as many as one-third in one series, will have AD pathology instead
[14,92,93]. (See "Clinical manifestations and diagnosis of Alzheimer disease", section
on 'Atypical presentations'.)
Neuroimaging Findings on neuroimaging studies may support the diagnosis of
FTD, but are not in themselves diagnostic [6,102,103]. A neuroimaging study, usually
brain magnetic resonance imaging (MRI), is nonetheless required in patients with
bvFTD, PNFA, and SD to exclude structural disease.

MRI may be normal early in the course of the disease [6,32]. With clinical progression
there is usually evidence of focal atrophy of the frontal and/or temporal lobes that
worsen over time [104]. Specific patterns of atrophy have been associated with the
clinical syndrome in about 50 to 70 percent of patients [6,18,53,54,67,69,76,105-108]:
In bvFTD, bifrontal atrophy is the most common finding, in later stages, this usually
progresses to include bitemporal atrophy as well. Right frontal atrophy may correlate
most specifically with more prominent behavioral manifestations.
In PNFA, prominent left perisylvian atrophy can be noted
In SD, there is temporal lobe atrophy, which may be more marked on the left. The
atrophy is more marked anteriorly. This contrasts with AD in which temporal lobe
atrophy is more universally symmetric and not associated with an anterior-posterior
gradient [58,109,110].
In CBD, asymmetric atrophy involving the frontoparietal regions, basal ganglia and/or
cerebral peduncles may be seen.
In PSP, symmetric atrophy of the superior cerebellar peduncle and midbrain is
reported.
In FTD-MND, bifrontal atrophy is variably seen.
Diffusion tensor imaging shows abnormal diffusivity of gray matter in these regions
[111]. Functional neuroimaging studies (single photon emission computed tomography
[SPECT], perfusion MRI, or fluorodeoxyglucose positron emission tomography [FDGPET]) may be more sensitive than MRI in the early stages of disease
[15,19,21,54,76,97,106,112-121]. Similar associations between neuroanatomic
patterns of hypometabolism or hypoperfusion and clinical syndromes that have been
reported for MRI have been reported in these studies as well.
Neuropsychological testing Formal neuropsychological assessment can be a
valuable tool for quantifying and profiling neurocognitive impairment. However, its
role in the diagnosis of FTD is somewhat limited. In very early stages of FTD
characterized only by behavioral changes, neurocognitive testing may be relatively
normal [42].
While there may be preferential involvement of certain cognitive domains in FTD that
are distinct from AD and other forms of neurodegenerative dementia, efforts to define
neuropsychological test batteries and specific profiles of results that reliably

distinguish among disorders have not produced consistent results [22,45,122-126].

Part of the challenge results from the fact that neuropsychologic test performance
varies over the course of the disease, and it is difficult to define or control for disease
severity between diseases [124]. Test administrators also need to be sensitive to the
fact that deficits in one domain (eg, apathy, poor attention, impaired comprehension)
may lead to the appearance of deficits in another and should not rely on test scores
alone [54].
Despite these limitations, certain patterns of findings on neuropsychological testing
may support the diagnosis of FTD over AD. These include a relatively worse
performance on tasks of frontal executive function and social cognition and relatively
better performance on tests of memory and visual spatial function [19,22,123,125129]. There are reported exceptions, however, with patients with FTD occasionally
presenting with prominent amnesia [8,130,131].
Genetic testing Genetic testing is available for several of the mutations that are
known to cause familial FTD syndromes. (See "Frontotemporal dementia:
Epidemiology, pathology, and pathogenesis", section on 'Genetic factors'.)
In the absence of a clinical imperative for diagnosis, such testing should be done
selectively. The family history, the results of pathologic studies (if available) in the
patient or family member, and the clinical syndrome can guide appropriate testing
[132]. Because not all mutations underlying familial FTD syndromes have been
identified, negative test results are less helpful than positive results. A role for
presymptomatic testing is not as yet defined.
Other laboratory investigations Other laboratory studies may be useful to rule
out alternative dementia etiologies [6]. (See "Evaluation of cognitive impairment and
dementia".)
Cerebrospinal fluid (CSF) studies of tau levels have been inconsistent, possibly in part
because many, if not most, cases of FTD do not involve tauopathies [106,133-136].
Among the clinical syndromes, progressive supranuclear palsy is overwhelmingly likely
to be associated with tau-positive pathology. In one case series, all 21 patients with
PSP were found to have truncated tau production on CSF analysis, while none of the
age-matched controls with no or alternative neurodegenerative disorders had this
finding [137]. CSF tau levels are not recommended in the diagnosis of FTD at present.
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SUMMARY AND RECOMMENDATIONS Frontotemporal dementia (FTD) is a
neuropathologically and clinically heterogeneous disorder characterized by focal
degeneration of the frontal and/or temporal lobes.
Behavioral variant FTD (bvFTD) is the most common clinical subtype. Patients
present with progressive change in behavior and personality. Features can include
abnormal social comportment, unusual eating patterns, and ritualized behaviors,
among others. Cognitive impairments appear later and are most prominent in tasks of
executive function, judgment, and problem-solving. (See 'Behavioral variant' above.)
Three aphasia syndromes are recognized:
Progressive nonfluent aphasia (PNFA) is a syndrome characterized by increasing
anomia, dysfluency, and agrammatism progressing to mutism. (See 'Progressive
nonfluent aphasia' above.)
Semantic dementia (SD) patients have a fluent aphasia characterized by anomia,
impaired comprehension, and semantic paraphasic errors. Patients often have a
broader loss of semantic memory that affects visual perceptual skills; episodic
memory is relatively spared. Behavioral features often develop in patients with SD.
(See 'Semantic dementia' above.)
Logopenic phonological aphasia is characterized by an overall paucity and slowness
of speech output with deficits in naming and word retrieval, along with phonemic
paraphasias. (See 'Logopenic phonological aphasia' above.)
Motor syndromes associated with FTD include progressive supranuclear palsy,
corticobasal degeneration, and FTD with motor neuron disease. (See 'Motor
syndromes' above.)
bvFTD must be distinguished from other neurodegenerative dementias, structural
disease of the frontal lobes, and psychiatric disease. The differential diagnosis of PNFA
and SD includes slowly growing mass lesions and atypical Alzheimer disease (AD).
(See 'Differential diagnosis' above.)

The diagnosis of FTD is made primarily on the basis of clinical features. Neuroimaging
studies (usually magnetic resonance imaging) are required to rule out other entities
and may provide supportive evidence of FTD. (See 'Diagnosis' above.)