A brief review of dementia

A brief review of
dementia
Dr. M. Atazadeh
Neurologist
2019

Major cognitive domain and their anatomical
location:

Mild cognitive decline (MCD) or mild cognitive impairment
(MCI):
- a transitional state between the cognition of normal aging
and mild dementia.
- a clinical construct that describes individuals with mildly
impaired performance on objective neuropsychological tests
but relatively intact global cognition and daily functioning.
- qualitatively different from both normal aging and
dementia
- The overall prevalence of MCI in the general elderly
population has a range of 2-20% and is lower in the amnestic
subtype, i.e., 2-4% as compared to the broader group.
- In one Indian study, the prevalence of MCI was found to be
14.89%.

A simple approach to memory complaints:

The Mayo Clinic Study of Aging:
-- prevalence of MCI increases with age .
-- may be more common in men, in those who
were never married, and in those individuals with
APOE 4 genotype.
-- less prevalent in individuals with a greater
number of years of education.
-- aMCI is more prevalent than non amnestic MCI(
na MCI)

Neurobiology of MCI
-Neuropathologic changes: intermediate between normal
aging and early dementia.
--so: it is a transitional state that is evolving to dementia.
- Common pathologic findings:
Argyrophilic grain disease
hippocampal sclerosis
vascular lesions.

imaging in MCI:
-areas of atrophy in MRI:
-1. medial temporal lobe
-2. entorhinal cortex
-3. hippocampus
-4. posterior cingulate gyrus.
-amyloid-PET scans have shown increased beta amyloid in several areas:
-1.the lateral frontal cortex
-2. posterior cingulate cortex
-3. medial and lateral parietal lobes
-4.the lateral temporal lobe.

Consequences of aMCI:
-Progress to Alzheimer’s disease at a rate of 10% to 15% per year.
-- significantly greater than the population incidence rates for Alzheimer’s
disease, which is 1% to 2% per year.
-- Risk factors for progression of MCI to dementias include:
-1. the degree of cognitive impairment at initial evaluation
-2. apolipoprotein E-ε4(ApoE4) carrier status
-3. neuropathological changes,
-4.functional changes in the brain (functional imaging)
-5.changes in the cerebrospinal fluid status (CSF biomarkers).
-Note: multiple domain MCI is at greater risk of dementia, in comparison to
single domain.

CSF study to predict AD:
- CSF concentrations of T-tau and Abeta42 at baseline yield a
sensitivity of 95% and a specificity of 83% for the detection
of incipient AD in individuals with MCI.
- the combination of T-tau and Abeta42/Ptau181 ratio
yielded a sensitivity of 95% and specificity of 87% with a
hazard ratio (HR) of 19.8

United States: 14% of people 71 years and older have
dementia and AD accounts for 70% of them.

Main presentations of AD:
-Cognitive symptoms of AD most commonly include
deficits in:
-- short-term memory
-- executive function
-- visuospatial function
-- praxis

Risk factors for AD:
-- age (the greatest risk factor)
- apolipoprotein gene E4 alleles (APOE4),
- low educational and occupational attainment,
-- family history of AD
-- moderate or severe traumatic brain injuries
-- cardiovascular risk factors
-- female to male ratio = 2
--

Cognitive decline in AD: memory
-- Early in the disease course, recent episodic memories
are most affected, while memories from the distant past
are usually spared.
-- As the disease progresses, all aspects of episodic
memory become affected.
- In contrast to episodic memory, working memory and
semantic memory are preserved until later in the disease
course.

Cognitive decline in AD: Lang ,VsF. and EF.
- Language disturbance, especially word-finding
difficulties, is a common early symptom in AD but is
generally mild.
- Subtle decline in visuospatial skills likewise occurs
in the mild dementia stages.
- Executive dysfunction, begins even earlier in the
predementia stages.

Neuropsychiatric Symptoms
The earliest: :
apathy,
anxiety,
irritability.

Anxiety and irritability:
often provoked in situations that the patient finds challenging

Other psychiatric symptoms:
-- mild to moderate depressive symptoms are also
frequently present early on.
-- Disturbances of appetite and sleep
-- disinhibition
-- alterations in perception (hallucinations) or thought
(delusions) commonly occur in the
later stages of dementia.
- anosognosia (i.e., lack of insight) often manifests early on
and poses another difficult management problem.

the most pressing psychiatric symptoms:
- irritability
- agitation
- sundowning
- Psychosis
- diminished insight

Neurologic Examination:
-- N/E is normal except for mental status exam.
-- Parkinsonian symptoms can emerge in the later stages.
-- later in the disease course, pathologic reflexes such as
grasp, root, and suck reflexes may be found
Note:
-If parkinsonism is present early in the course of the
disease (e.g., within 1 year of onset of cognitive
problems) and especially when accompanied by cognitive
fluctuations and early-onset psychosis, a diagnosis of DLB
shuld be considere.

ATYPICAL ALZHEIMER DISEASE VARIANTS:
- Frontal variant
- Posterior cortical atrophy
- Logopenic variant

FRONTAL VARIANT:
-- substantial behavioral or personality changes
that are out of proportion to the observed short-
term memory loss.
-- These patients are often profoundly impatient,
irritable, impulsive,
and disinhibited.
- On formal testing, they invariably show significant
executive disturbances

Posterior cortical atrophy:
-Presents with visuospatial dysfunction often in the form of
partial or full :
-- Balint syndrome (simultanagnosia, ocular apraxia, and ocular ataxia),
-- Gerstmann syndrome (acalculia,agraphia, right/left disorientation,
and finger agnosia),
-- apperceptive visual agnosia
-- environmental disorientation.
- Patients often develop constructional, dressing, and
ideomotor apraxia
early on in the presence of relatively preserved memory and
insight.

Logopenic variant:
- early progressive language involvement,
- most often in the form of Logopenic aphasia
with pronounced anomic deficits and
impaired repetition but preserved
grammar and syntax.

NIA-AA criteria for dementia:
-- no longer explicitly require memory impairment
to be present.
-- two cognitive domains or one cognitive and one
behavioral domain in addition to significant
decline in day-to-day functioning is required.
--for probable AD: the diagnostic utility of disease
biomarkers.

2 types of biomarkers…
1 - Two neurodegenerative biomarkers
- mesial temporal lobe atrophy on structural imaging
- posterior predominant hypometabolism with involvement of the posterior
cingulate gyrus on FDG-PET
Note:
Neither of these are exclusively seen in AD dementia. Hippocampal
atrophy occurs in normal aging, and both hippocampal atrophy and FDG-
PET abnormalities occur in other dementing conditions.

Structural imaging: Mesial temporal atrophy

FDG- PET: posterior hypometabolism

2 types of biomarkers…
2 - amyloid protein based biomarkers such as:
- a low CSF level of beta-amyloid
- positive amyloid PET scan
Note:
These are highly sensitive and specific in their ability to detect amyloid
pathology in the brain.

Current Role of Biomarkers in AD Diagnosis
-The American Academy of Neurology (AAN) guidelines :
-- a structural imaging scan in every patient with objective cognitive
decline is indicated.
-- Currently, FDG-PET and SPECT use is only covered by Medicare for
-differentiating AD from frontotemporal dementia.

Gradient echo MRI: blooming infavor of cortico
subcortical hemorrhages, infavor of CAA.

amyloid PET imaging:
-- detection of moderate to severe amyloid deposition in
the brain.
-- well documented sensitivity and specificity,
- but not yet entered routine clinical practice,
(because insurance companies do not provide This type of imaging coverage).

Appropriate Use Criteria for amyloid PET
imaging :
(1) patients with amnestic mild cognitive impairment (i.e.,
mild neurocognitive disorder),
(2) Patients in the dementia stages with suspected atypical
AD or etiologically mixed presentation,
(3) those with early disease onset (younger than 65 years of
age).

Rational for the use of amyloid PET
(1) Helping the practitioner to select appropriate treatments and avoid
unnecessary interventions and to aid in accurate diagnosis,
(2) improving diagnostic accuracy,
(3) advising patients and families on the clinical course and prognosis,
(4) educating patients and families on community services and resources
for medical, financial, and legal planning.

inappropriate uses of amyloid
PET imaging.
Patients who should not be scanned include:
-those lacking objective cognitive decline.
-
-Scanning solely on the basis of family history or APOE4
status was likewise determined to be inappropriate.
-
- amyloid PET scans cannot be used for determining
dementia severity, as this is not feasible with this technology.

The most established AD CSF biomarkers:
Amyloid beta:
- decline early in the disease course.
-- highly correlated with the presence of amyloid deposition in the cortex
-- a proxy measure of the brain amyloidosis instead of amyloid PET scan
tau and phosphorylated tau:
- rise later, associated with cognitive decline.
- Not routinely used due to relative invasiveness of the lumbar puncture.
- quite useful in diagnostically challenging cases as well as in those with
early disease onset or an unusual clinical course.

APOE4 gene :
- the most established genetic risk factor for sporadic AD.
-- screening for APOE4 is not recommended on a routine
basis.
- a large multicenter study :
the presence of the APOE4 allele increased the positive
predictive value of diagnosing AD by only 4% over diagnoses
made on clinical grounds alone (from 90% to 94%).
- The APOE4 genotype :
a risk factor that is neither sufficient nor necessary for
disease development.

Family history of sporadic AD:
-A well-established
-risk factor.
-Genetic makeup
-is not the only risk;
-shared environmental
-and
-lifestyle factors
-likely also play a role.

autosomal dominant variants of AD
-- rare (less than 2% of cases)
-- The first symptoms usually affect individuals in their 30s
and 40s.
-- These families show multiple affected individuals across
generations.
-- genetic mutations in:
-amyloid precursor protein (APP),
-presenilin 1 (PSEN1),
-presenilin 2 (PSEN2) genes.
-- all of them increase the level of beta amyloid.

ALZHEIMER DISEASE PATHOLOGY
-- due to : overproduction and impaired clearance of
beta amyloid.
-- Downstream events: tau hyper phosphorylation and
neuronal toxicity.
- The primary pathologic features of AD:
brain atrophy from regional neuronal and synaptic
loss,
extracellular beta amyloid deposition in the form of
neuritic plaques
intraneuronal tau protein deposition in the form of
intraneuronal NFT.

Cerebral amyloid angiopathy (CAA):
Beta Amyloid also deposits in the cerebral blood vessels.
CAA ranges in severity:
- small amounts of amyloid
- major deposits that distort the artery architecture and
cause:
cortical micro infarcts,
micro aneurysms,
cerebral micro hemorrhages or macro hemorrhages.

CAA consequences:
- Therefore, the hallmark of CAA on imaging is:
the presence of microhaemorrhages.
- These microhaemorrhages are not visible on CT, and are
also invisible on most of the standard MR sequences ( T1-
weighted, T2-weighted and FLAIR).
but visible on a sequence known as susceptibility weighted
imaging

NFT: specific?
-- not exclusive to AD
-- can be found in:
-dementia pugilistica
-chronic traumatic encephalopathy,
prion disease
normal aging.
NFT burden and neuronal loss show a robust association With global
cognitive impairment.

MTA:
score 0: no atrophy
score 1: only widening of choroid fissure
score 2: also widening of temporal horn of lateral ventricle
score 3: moderate loss of hippocampal volume (decrease in
height)
score 4: severe volume loss of hippocampus
< 75 years: score 2 or more is abnormal.
> > 75 years: score 3 or more is abnormal.

Management of AD :
- Diagnostic and prognostic counseling
- Management of coexisting behavioral and non-neurologic
conditions.
- safety precautions
- social activities -
- exercise programs
- support groups
-- medications:
-Cholinesterase inhibitors
-Glutamate receptor modulators
-

Acetylcholinesterase Inhibitors
- donepezil,
- rivastigmin,
- galantamine.
- should be started in patients with mild to moderate AD.
- showing some benefit on cognitive measures including memory
and
concentration as well as global and functional outcome
measures.
- their therapeutic cognitive and functional effects seem to be
modest in size
and purely symptomatic-

Ache Inhibitors, side effects:
-- GI : all three agents
-- Bradycardia and heart block may occur, especially in
patients with underlying cardiac conduction deficits or in
those individuals taking medications that cause PR
interval prolongation such as beta-blockers.
- If one agent causes intolerable side effects, another Ache I
should be tried.

Glutamate Receptor Modulators
- Memantine : as an add-on to ongoing Ache I.
-- beneficial effect on cognition, behavior, ADL, and
-global function.
- FDA : for the moderate to severe AD stages
( MMSE = 5 to 15)
- The main side effects : confusion and dizziness
rare))

Off – labele:
-- AChEI and memantine are frequently prescribed off-label
for MCI in the United States.
-Recent meta-analyses: no benefit of AChEI in MCI
although a benefit for subgroups of patients remain
undetermined.
- 2011 NIH guidelines : memantine as an option for
managing moderate AD for people who cannot take AChEI
and as an option for managing severe AD.

Medications for behavioral symptoms
- The first line : non pharmacologic techniques:
-- A quiet, familiar environment with labels on doors and
-sufficient lighting in all rooms is important to reduce
-disorientation.
-- Aggressive behavior should always be addressed with
-positive and clear language to reassure and distract the
-patient.
-- Depressive symptoms : SSRIs
-* may also ease anxiety and irritability.
*citalopram may be useful for agitation.

Antipsychotics
-- agitation or disruptive behavior may require a neuroleptic
-for optimal therapeutic response.
-- The newer ‘atypical’’ antipsychotic medications
(quetiapine, risperidone, olanzapine) are often used in low
doses with careful titration.
-- a black box warning for all antipsychotics in elderly.
- thus, judicious use and frequent reassessment of the
-therapeutic need.

Lewy body? synuclein?
- Lewy bodies (LB) :
the main pathological hallmark of Parkinson’s
Disease (PD) and Dementia with Lewy Bodies (DLB).
- α- synuclein (aSyn):
major component of Lewy bodies.

Lewy body dementias:
Lewy body related pathology is observed in:
- dementia with Lewy bodies (DLB)
- idiopathic PD
- multiple system atrophy (MSA).
DLB and the PDD together comprise the Lewy body
dementias.

Common features of LBDs:
- hallucinations,
- cognitive fluctuations
- dementia
- parkinsonism.
The involved cognitive domains :
- prominent executive dysfunction
- visuospatial abnormalities
- variable impairment in memory capacities.

1year cut off point
In DLB, dementia often heralds the onset of parkinsonian
motor signs, but by consensus may follow their development
up to 1 year from their onset.
In contrast, a diagnosis of PDD is made when cognitive
impairments develop in the setting of well-established PD.

LBDs Common neuropathology:
- Widespread Lewy bodies: brainstem - limbic - cortex
- loss of midbrain dopamine cells
- loss of cholinergic neurons in ventral forebrain nuclei

basal forebrain:
The substantia innominate (si) and the amygdaloid complex (a) are located
on the surface of the brain. c=head of the caudate nucleus; cg=cingulate
gyrus; g=globus pallidus

Lewy body disease?
It is equal to widespread deposition of Lewy bodies
in cortex and limbic and brainstem.
But not equal to Lewy body dementias!
- Lewy body disease at autopsy does not
successfully predict whether patients had DLB or
PDD syndromes in life

LBDs: neuritic plaque?
Neuritic plaques that contain amyloid and NFT are found in
the majority of cases of DLB and are common in PD.
Current neuropathologic criteria of Lewy body disease weigh
a-synuclein pathology against AD neurofibrillary tangle
pathology to estimate the probability that Lewy
body disease caused the clinical syndrome in life.

DLB: Cognitive symptoms
-The typical patient with DLB presents with early
dementia, often in association with visual hallucinations.
- Extrapyramidal motor symptoms and signs
characteristic of PD often develop simultaneously or
soon thereafter.
- Progressive cognitive decline begins early, typically after age
55.

First domains:
- Although short-term memory may be involved,
- cognitive domains other than memory are frequently affected
as well:
attention
executive function
visual-spatial skill.
- Patients may therefore report early difficulty multitasking at
work or home and may start to lose the thread of
conversations.

DLB: cognitive symptoms
- may occasionally get lost while driving or grow increasingly
dependent on GPS devices.
- Short-term memory loss can be significant : retrieval
deficit
- which can be improved with cues.
- impairments progress and spread to involve other
cognitive domains.
- When they are sufficiently severe to impair ADL and iADL
,they reach the criteria of DLB.-

DLB: Neuropsychiatric Symptoms
- Recurrent, complex visual hallucinations
usually well formed and animate
commonly include adults or small children, deceased
family members, and small animals.
- Delusions:
typically later in the course,
usually a paranoid quality.
also : infidelity, house intruders, theft.

Capgras syndrome in DLB:
A delusion that their spouse or other caregiver has
been replaced by an imposter.
Due to loss of emotional associations for a memory,
such that a familiar face loses its ability to retrieve
emotional associations.

DLB: Fluctuations of Attention and Arousal
- episodes of staring and perturbed flow of ideas
- frequent daytime drowsiness and naps during the
day.
- note: R/O toxic metabolic processes such as
medication side effects or infections.
Fluctuation can be confirmed by dementia cognitive
Fluctuation Scale:
3 positive response out of 4 is required.

dementia cognitive fluctuation Scale:
(1) Does the patient’s inability to organize thoughts in a coherent
way vary significantly over the course of the day?
(2) Does the patient spend more than 1 hour sleeping during the
waking day?
(3) Is the patient drowsy and lethargic for more than 1 hour
during the day, despite getting the usual amount of sleep the night
before?
(4) Is the patient difficult to arouse on a usual day?
Note: This approach had a sensitivity of 80% and a specificity of
76% in differentiating clinical syndromes of DLB and PDD from AD
and vascular dementia, but has yet to be neuropathologically
validated.

DLB motor features:
-often develop concurrently with or subsequent to cognitive problems
- often symmetric, and bradykinesia and gait impairment are more
common than rest tremor.
- Some may present with a classic asymmetric pill-rolling tremor of PD
- Some may have no motor concerns yet will display clear extrapyramidal
dysfunction on examination

DLB motor features…
- limited response to levodopa.
- reduced dopamine transporter (DAT) activity on SPECT or
PET.
- Generalized myoclonus can also occur.

Neuroleptic Sensitivity
- Due to dopamine cell loss in DLB.
- neuroleptics can trigger or exacerbate parkinsonism, this
may be irreversible.
- increased mortality
- neuroleptic malignant syndrome.
- affect cognition and impair attention and alertness.

Other features of DLB:
RBD :
may precede other features of DLB , in this condition
normal sleep paralysis impairs , leading to acting out
movements in sleep.
-autonomic impairment:
constipation, orthostatic hypotension, syncope and falls.
More common in later stages of DLB.
-

1. Core Features (2 feature = probable DLB, one = possible DLB)
-Dementia with progressive cognitive decline of sufficient
magnitude to interfere with social or occupational function.
- Memory impairment may not necessarily occur early but
usually develops with progression. Deficits on tests of
attention, executive function, and visual-spatial ability may be
prominent.

2. Core Features (2 feature = probable DLB, one = possible DLB)
- Fluctuating cognition, pronounced variation in
attention and alertness
- Recurrent visual hallucinations, typically well
formed and detailed
- Spontaneous motor manifestations of parkinsonism

3. Suggestive Features:
- RBD
- Severe neuroleptic sensitivity
- SPECT or PET : low dopamine transporter concentration in the basal
ganglia
Note:
one core + one suggestive = probable
No core + at least one suggestive = possible.

4. Supportive Features
- Reduced MIBG myocardial scintigraphy
- SPECT perfusion with reduced occipital activity
- Relatively preserved medial temporal lobe structures on CT/MRI
- Repeated falls and syncope-
- Transient, unexplained loss of consciousness
- Autonomic dysfunction
- Other types of hallucinations
- Systematized delusions
- Depression
- Prominent slow-wave activity on EEG, with temporal lobe transient sharp waves

A Diagnosis of DLB Is Less Likely if:
- focal neurologic signs on examination or on brain
imaging
- presence of other physical illness or brain disorder
that can account in part or in full for the clinical
presentation
- If parkinsonism only manifests at a stage of
dementia.

Temporal Sequence of Symptoms
- DLB is diagnosed when dementia precedes or is concurrent
With parkinsonism.
- PDD should be used to describe dementia that occurs in the
context of well-established Parkinson disease.
Note:
a 1-year rule is recommended for a diagnosis of DLB, such that
dementia should begin no later than 1 year after onset of
parkinsonism.

.
Symptomatic Treatments in DLB and PDD

For Cognitive Impairment
1. Acetylcholinesterase inhibitors:
donepezil 5mg/d for 4weeks then 10mg/d.
rivastigmin: 1.5 mg 2 times a day, increase in 1.5 mg steps every 2- 4 weeks,
maximum 6 mg 2 times a day
Galantamine: 4.6 mg per 24 hours for 4 weeks, then increase to 9.5 mg per
24 hours.
2. NMDA receptor antagonist:
Memantine :
5 mg/d for 1 week, then 5 mg BID for 1 week, then 10 mg every morning, 5 mg
every evening for 1 week, then 10 mg 2 times a day

For Psychomotor slowing
1. Acetylcholinesterase inhibitors : as above
2.Levodopa/Carbidopa :
100 mg/25 mg - 2 times a day upon waking and at dinner to
ensure tolerability, then increase to 3 times a day (upon
waking, at lunch, and at dinner)

For Apathy:
1. Acetylcholinesterase inhibitors
2. SSRIs
3. coffee: 1- 2 cups before 2:00 PM

For psychosis (hallucination – delusion):
1. acetyl cholinesterase inhibitors
2. Quetiapine
3. clozapine

Depression:
Escitalopram
Venlafaxine
Citalopram
Fluoxetine
sertraline

Anxiety:
Escitlopram
Venlafaxine
Citalopram
Sertraline
buspirone

insomnia
Melatonin
Trazodone
Mirtazapine
quetiapine

Agitation:
Quetiapine
clozapine

Frontotemporal dementia
1. behavioral variant:

Behavioral variant FTD:
- early changes in behavior, personality, emotion, and
executive control.
- disinhibition,
- new compulsions,
- dietary changes,
- apathy and lack of empathy.

diagnosis of bvFTD
at least 3 symptoms
- disinhibition
- apathy
- lack of empathy
- compulsions
- hyperorality
- executive dysfunction.

Anatomy of bvFTD:
- dysfunction in the paralimbic areas, including medial frontal, orbital
frontal, anterior cingulate, and frontoinsular cortices.
- Right hemisphere atrophy is associated more with behavior
changes:

Disinhibition
- socially inappropriate behavior:
invading interpersonal space inappropriate touching.
overfamiliarity with strangers.
- Impulsive or careless actions:
new onset gambling stealing
poor decision-making
Anatomy: right orbital frontal cortex degeneration.

Apathy
- Affective apathy: indifference or not caring.
- Motor apathy: decreased drive to move and less movement
overall,
- cognitive apathy: loss of desire to engage in goal-oriented
activities.
Symptoms:
- social withdrawal
- often need prompting to stay engaged in conversation, do chores,
or even to move.
- easily misinterpreted as depression.
- anatomy: medial prefrontal lobes and anterior cingulate.

severe frontal and temporal lobe atrophy:
60-year-old woman, with bvFTD.

PRIMARY PROGRESSIVE APHASIAS
- neurodegenerative syndromes, where language is
the primary impairment in the first 2 years of
symptom onset.3 types:

Semantic variant of FTD = svFTD
Left svPPA (Involvement of the left temporal) :
language symptoms predominate + slow loss of language
Right svPPA (Involvement of the right temporal) :
behavioral symptoms predominate.

As time progresses:
both temporal lobes become involved,
and symptoms begin to overlap,
By 5 to 7 years, patients get more frontal lobe structures
involved and develop symptoms of bvFTD with disinhibition,
change in food preference, and weight gain.
Note:
svPPA tend to have slow progression and can live a decade or more after
symptom onset.

Lt svPPA:
The initial features:
anomia and single-word comprehension deficits.
The earliest symptom :
poor comprehension of single low-frequency words,
such as “giraffe,” with initial preservation of higher frequency
words like “dog.”

Lt svPPA :
- As symptoms progress,
patients also lose semantic knowledge:
No improve with phonemic cues or semantic hints.
- Surface dyslexia: Yacht , gnat.
-- memory can be affected.
-- but executive function and visuospatial skills typically
are preserved.

Lt svPPA, previous case:
a 61-year-old man, whose first symptom was 6 years
before with the inability to name apples in a fruit
bowl.
His problems with semantics and naming
progressed, and 3 years before he had difficulty
recognizing faces of neighbors.
More recent symptoms include the urge to lick
people and things (hyperorality).

Rt svPPA: previous case
Bitemporal atrophy right greater than left:
-a 64-year-old woman, whose first symptom was 10 years
before, telling repetitive stories that slowly became less
appropriate and embarrassed her friends and family. Six
years before, she developed an obsession of taking
specific walking routes to collect cigarette butts. More
recently, she has shown lack of empathy toward her
family and dog.

Clinical diagnosis of svPPA
Both of these:
A. Impaired confrontation naming
B. Impaired single-word comprehension
At least 3 of these:
A. Impaired object knowledge, particularly for low-frequency or low-familiarity
items
B. Surface dyslexia or dysgraphia
C. Spared repetition
D. Spared speech production (grammar and motor speech).

Imaging- supported svPPA diagnosis:
Both of these:
A. Clinical diagnosis of svPPA.
B. Imaging must show one or more of the following results:
1. Predominant anterior temporal lobe atrophy
2. Predominant anterior temporal hypoperfusion or hypometabolism
on SPECT or PET

Nonfluent/agrammatic PPA:
- effortful speech and word-finding problems.
- Over time, the speech becomes labored, slow, slurred, and
choppy with disrupted prosody.
- The patient begins making inconsistent speech sound errors
without awareness of this deficit. There can be inconsistent
insertions, deletions,distortions,and substitutions in speech
sounds.

Clinical diagnosis of
nonfluent/agrammatic variant PPA:
At least one of these:
A. Agrammatism in language production
B. Effortful, halting speech with inconsistent speech sound errors and distortions
(apraxia of speech)
And at least 2 of these:
A. Impaired comprehension of syntactically complex sentences
B. Spared single-word comprehension
C. Spared object knowledge

nfvPPA:
MRI showing predominant left posterior
frontoinsular atrophy.

Treatment:
- no FDA approved treatments for FTD.
- treatments for AD have not shown benefit in FTD.
- FTD symptoms and behavior can improve with SSRIs.
- Atypical antipsychotics should be used with caution in
patients with FTLD.

A brief review of dementia

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