Cognition and cognitive syndromes cme

Cognition
& cognitive
syndromes
Dr. Doha Rasheedy
Assistant professor of Geriatric Medicine
Ain Shams University

Cognition
• From Latin word “ Cognitio” which means
know together.
• Cognition: the collection of mental processes
and activities used in perceiving, learning,
remembering, thinking, understanding and
the act of using these processes

COGNITIVE DOMAINS AND
RELATED SYNDROMES

• Much cross over occur between different
domains (many mental functions are
processed under different domains, so difficult
to separate completely during
neuropsychoogical assessment.
– Working memory (attention, executive, memory)
– Behavior (social cognition, executive functions)

EXECUTIVE FUNCTIONS
Executive function refers to components of cognition that
allow humans to form relevant goals, plan how to achieve
them, and successfully carry out the intended actions.

Executive functions
known as cognitive control and supervisory attentional system
Includes a set of skills or cognitive processes including:
1. Attentional control: an individual's capacity to choose what they pay attention to and
what they ignore.
2. Inhibitory control (overriding habits): choose the more complex, effortful true
solution
3. Working memory: holding information for a brief time while manipulating information
4. Mental flexibility: ability to shift between two tasks
5. Reasoning: process of looking for reasons for beliefs, conclusions, actions or feelings
6. Problem-solving (the three phases of strategy selection, application of operations
and evaluation of outcomes)
7. Planning: The identification and organization of the steps and elements (e.g.,
skills, material, other persons) needed to carry out an intention or achieve a goal
8. Feedback utilization: awareness of own mistakes

Dysexecutive syndrome (Frontal lobe
syndrome)
• Dysexecutive syndrome encompasses cognitive,
emotional, and behavioral symptoms.
• Dysexecutive syndrome can result from many
causes:
– head trauma
– tumors
– degenerative diseases
– cerebrovascular disease
– psychiatric conditions

Supervisory attentional system
• For example, making a cup of coffee can become
relatively automatic, but sometimes when a
person is tired or distracted, he or she may
unintentionally pour milk into the coffee pot
instead of into the cup.
• In contrast, the supervisory attentional system
is activated when conscious effort is required:
for example, in situations of novelty or crisis or
when new skills are learned.

Cognitive symptoms
Mild:
1. Increased effort required to complete multistage projects.
2. Has increased difficulty multitasking or difficulty resuming
a task interrupted by a visitor or phone call.
3. May complain of increased fatigue from the extra effort
required to organize, plan, and make decisions.
4. May report that large social gatherings are more taxing or
less enjoyable because of increased effort required to
follow shifting conversations.
Major:
1. Abandons complex projects.
2. Needs to focus on one task at a time.
3. Needs to rely on others to plan instrumental activities of
daily living or make decisions.

Emotional symptoms
• They may have difficulty inhibiting many
types of emotions such as anger, excitement,
sadness, or frustration.
• may have higher levels of aggression or anger
because they lack abilities that are related to
behavioural control

Behavioural symptoms
• People with DES often lose their social skills
because their judgments and insights into what
others may be thinking are impaired. Interrelated
with social cognition domain.
• They may have trouble knowing how to behave in
group situations and may not know how to follow
social norms. They appear self-centered and
stubborn
• Examples of DES behaviour: (Utilization
behaviour and preservation)

Utilization behaviour
• Utilization behaviour is when a patient automatically
uses an object in the appropriate manner, but at an
inappropriate time.
• For example, if a pen and paper are placed in front of an
individual with DES they will start to write or if there is a
deck of cards they will deal them out.
• Patients showing this symptom will begin the behaviour
in the middle of conversations or during auditory tests.
• Utilization behaviour is thought to occur because an
action is initiated when an object is seen, but patients
with DES lack the central executive control to inhibit
acting it out at inappropriate times

Perseveration
• Perseveration is the repetition of thoughts, behaviours,
or actions after they have already been completed.
• For instance, continually blowing out a match, after it is
no longer lit is an example of perseveration behaviour.
• Stuck-in-set perseveration is most often seen in
dysexecutive syndrome. This type of perseveration refers
to when a patient cannot get out of a specific frame of
mind, such as when asked to name animals they can only
name one. If you ask them to then name colours, they
may still give you animals.
• E.g. graphomotor luria’s task

Is it always frontal lobe lesion???

orbitofrontal circuit (inhibition)
• affect the behavioral response to environmental and
social demands
• Affected patients show:
– Impulsivity
– Inappropriate behavior
– liable to increased distractibility
– Utilization behaviour

Anterior Cingulate Circuit (Volition)
Affected cases show:
• apathy, the most extreme form being of akinetic mutism: a
profound indifference to the environment in which patients
lack any internally generated activity or behavior.
• Such persons may be fully capable of performing complex
activities and yet not carry them out unless instructed to do
so.
• For instance, although able to use eating utensils properly,
some will not eat what is set before them without ongoing
explicit instructions. Less impaired persons may eat or drink
what is set before them, but will not seek nourishment
spontaneously, even when hungry

Dorsolateral Prefrontal Circuit
(cognition)
• Responsible for: cognitive flexibility or
attentional switching and the formulation of
novel ideas and responses.
• Affected cases show:
– Impaired planning
– maintenance of cognitive set and set switching
– impaired working memory information
– impaired mental flexibility and
– poor reasoning.

Test Batteries or Individual Tests for
Executive Function?
There are now several test batteries of executive
function, including
1. the Behavioural Assessment of the Dysexecutive
Syndrome (BADS)
2. the Behavioral Dyscontrol Scale
3. The Delis-Kaplan Executive Function Scale (D-
KEFS
4. the Frontal Assessment Battery

• The inhibition, overcoming habits (use stroop test).
• The ability to shift attention readily between
different cognitive tasks (set shifting-cognitive
flexibility) (use TMT a, b) not only b because we need
derived scores
• Planning: (Maze tracing), (Tower Tests: London,
Hanoi, and Toronto), (the Zoo Map test)
• Coordinating the Performance of Multiple Tasks/
divided attention (Dual Tasking):verbal performance
of digit span at the same time as a paper and pencil
tracking task; dual-task capacity is indexed by
comparing performance level in each single task with
performance of both tasks under dual-task
conditions.

• Motor perseveration may be evident in copying
drawings of repeating patterns (e.g., “+ 0 + + 0 + + +
0 . . .”),
• Cognitive inhibition (conflicting tapping test (“tap
once when I tap twice, and tap twice when I tap
once”) and the go–no-go test (“tap once when I tap
once, but don’t tap at all when I tap twice”), (The
Hayling Sentence Completion Test).
• Abstraction(using proverb interpretation??culture,
semantic), (word similarities/ differences).
• Judgment is sometimes assessed by asking patients
what they would do in a hypothetical situation, such
as if they found water flooding into their kitchen.

• Many traditional measures of executive function
(e.g., the Wisconsin Card Sorting Test [WCST])
are multifactorial (i.e., they assess a number of
different aspects of executive function and other
cognitive domains)
• sensitive but not specific
• Attention, working memory, flexibility, planning,
organized searching, using environmental
feedback to shift cognitive sets, and visual
processing.

Verbal fluency is it all executive
function???
• Phonemic fluency is an effortful task, requiring recruitment of executive
function, because retrieving words on the basis of orthographic criteria (spelling)
is unusual: People normally retrieve words on the basis of their meaning.
• In contrast, semantic fluency is considered less effortful, although patients with
early Alzheimer’s disease have been reported to demonstrate more difficulty
with semantic fluency than with phonemic fluency, presumably as a function of
impaired semantic memory caused by early involvement of the temporal
neocortex.
• Recently proved that both forms of fluency are equivalent in sensitivity to frontal
lesions, which suggests that both draw on resources of executive processes,
including initiation, efficient organization of verbal retrieval and recall, and self-
monitoring.
• However, semantic fluency is also sensitive to temporal lobe lesions, which
suggests that impaired semantic fluency may be a result of either executive or
temporal dysfunction.

VFT
• set-shifting ability contributes to verbal fluency by
allowing active strategic search of relevant retrieval
cues for generating words (e.g., “ship, sailor, sea . .
.”; “soap, shower, shampoo . . .”)
• the number of subcategory switches and the
cluster size of individual groups of words.
• Qualitative aspects, such as production of socially
inappropriate words or rule breaking by producing
proper nouns despite being able to state that these
are not allowed, are important additional
observations.
• Neuroimaging studies identify significant activation
of the left dorsolateral prefrontal cortex (or its
associated network) and the left thalamic nucleus
during verbal fluency tasks

Assessment of volitional capacity
by direct examination of motivational capacity:
1. should inquire into patients’ likes and dislikes, what they do for fun,
and what makes them angry, as many volitionally impaired patients
are apathetic with diminished or even absent capacity for emotional
response.
2. The patient’s behavior in the examination can also provide valuable
clues to volitional capacity. Volitionally competent persons make
spontaneous and appropriate conversation or ask questions; or they
participate actively in the examination proceedings by turning test
cards, handing back test material, or putting caps back on pens.
Patients whose volitional capacity is seriously impaired typically
volunteer little or nothing, even when responding to what the
examiner says or does.
• Examination techniques can require the patient to initiate activity.
Heilman and Watson (1991) scatter pennies on the table in front of
patients, then blindfold them and tell them to pick up as many pennies
as they can. The task thus requires exploratory behavior which may be
lacking in patients whose capacity to initiate responses is impaired

Classification of Memory Types
• Nondeclarative (implicit) memory
– Motor skill learning
– Priming???
– Classical conditioning
• Working memory vs. short term memory???
• Declarative (explicit) memory (conscious
recall)
– Episodic memory
– Semantic memory

Declarative/explicit memory
• Episodic memories are unique in that they are
recollections of an individual’s own past
experiences, and therefore each is specific in
time and space.
• In contrast, semantic memory refers to
knowledge of universal facts.
• (factual semantic knowledge (public events,
famous people), including personal semantic
facts (semantic autobiograohical memory):
(e.g., names of schools attended, name of
employers).???)

Autobiographic memory
• Both semantic and episodic AM
• Episodic AM refers to remembering past events that
are specific in time and place and it typically involves
the recollection of vivid sensory, perceptual, and
emotional details (e.g., my last birthday)
• Semantic AM, in contrast, refers to the recollection
of personal facts, traits, or general self-knowledge,
which are independent of time, place, and any sense
of re-experiencing a past event (e.g., I am 30 years
old, I went to medical school)

working memory,
– Not simply the short-term memory.(must be
manipulated not simply rehearsed) (not just holding a
telephone number till making a call)
– Working memory is a combination of the traditional
fields of attention, concentration, and short-term
memory.
– dorsolateral prefrontal cortex (executive component)
– 3 subcomponents under central executive control.

The three subcomponents of working
memory are:
• Phonological Loop: this allows for transient storage and
rehearsal of verbal information, including sub-vocal self-talk.
• Visuospatial Sketchpad: this allows for transient storage and
rehearsal of visual features and spatial representations, such as
those required for mental representation of objects and
mental navigation.
• Episodic Buffer: in recognition of the fact that working
memory subcomponents must communicate both with each
other and with long-term memory storage, the Episodic Buffer
was postulated as a temporary, limited-capacity store for
integrated representations (i.e. multimodal representations of
episodes including verbal and visuospatial features).
The Central Executive
– is the more complex overarching component that orchestrates the function of the
above subcomponents
– is proposed to fractionate into several roles: focusing, dividing and switching
attention; and interacting with long-term memory.

• Encoding: inattention or amnesia???
• Storage
• Retrieval: a retrieval deficit is suggested where
there is disproportionate impairment of free
recall in comparison with recognition memory.

Amnesia
• anterograde amnesia: An inability to establish
new memories after the pathological event.
• retrograde amnesia: the inability to recall
memories that had been established before
the pathological event (recent lost before
remote why???) the Ribot effect

Prospective memory
• This refers to remembering to act on an intention at a later
time – (remembering to remember)
• a form of episodic memory
• The intended action may be :
1. time-based (e.g. attending a doctor's appointment at 10 a.m.)
2. event-based (e.g. taking the cake out of the oven when the alarm
sounds)
3. or activity-based (e.g. turning on the answering machine when
the office has closed for the day).
• When patients complain of poor memory, it is often these
prospective memory failures that are reported as most
bothersome. Prospective memory is crucial for many aspects
of independent living.
• Prospective memory functioning relies on interacting aspects
of long-term memory, working memory and executive
function

Recall vs recognition??
• “recall” refers to explicit retrieval of
information from storage either
spontaneously (free recall) or in response to a
prompt (cued recall)
• “recognition” refers to the ability to identify
familiar or learned information when it is
presented (e.g. by saying yes/no or selecting
from a choice).

Memory brain representation
• episodic long-term memory is heavily dependent on the
medial temporal lobes, limbic system and their connections
(including the hippocampus, fornix, amygdala, mamillary
bodies, parahippocampal gyrus and cingulate cortex), thalamus
and basal forebrain.
• semantic memory mainly activates the frontal and temporal
cortexes
• Implicit long-term memory relies on regions outside the medial
temporal lobes and diencephalon, with:
1. perceptual priming thought to be subserved by posterior cortical
regions
2. procedural motor skill learning by basal ganglia and cerebellar
areas.
• Prospective memory is associated with the anterior
prefrontal cortex (Brodmann Area 10) and connections to
dorsolateral prefrontal cortex, cingulate and parietal regions.

• Autobiographical episodic defects are
commonly seen after lesions to the medial
temporal and diencephalic structures
• while defects in factual semantic memory
result more commonly from the frontal and
temporal cortexes damage.

Assessment
• Ideally, begins with an initial screen, following which a
more detailed neuropsychological assessment may be
carried out.
• It is important to note that well known and commonly
used screens such as the Abbreviated Mental Test
(Hodkinson, 1972), Mini-mental State Examination
(Folstein et al., 1975), Addenbrooke's Cognitive
Examination (Hsieh et al., 2013) and Montreal Cognitive
Assessment (Nasreddine et al., 2005) do not test all
aspects of memory function.
• It is therefore possible that someone who is experiencing
specific or disproportionate difficulties with prospective
memory or retrograde long-term memory, for example,
might be deemed unimpaired on a typical brief screen.

A comprehensive neuropsychological
assessment
• Should cover:
– working memory
– episodic and semantic long-term memory (using both
anterograde and retrograde time frames where
possible)
– prospective memory.
– Lateralization effects
• verbal memory tends to be dependent on the left (language
dominant) hemisphere and visuospatial memory on the right,
materials in each modality should be used where available.
– Test both recall and recognition of material from long-term
memory, to help distinguish between problems with learning
vs retrieval.

Working memory
1. Simple screening tasks for working memory
include serial subtraction and backwards spelling.
2. Verbal working memory, The Paced Auditory
Serial Addition Test (Gronwall, 1977)
3. For visuospatial working memory, block tapping
(e.g. Corsi blocks; Kessels et al., 2000) is a
commonly used task. Block tapping has been
replaced in the latest version of the Wechsler
Memory Scale (WMS-IV; Wechsler, 2010b) with a
symbol span task, which is an abstract visuospatial
analogue of digit or word span paradigms.
4. The Test of Everyday Attention (Robertson et al.,
1994) assesses various aspects of attention and
working memory.

The Test of Everyday Attention
Test subsets
• Map Search: looking at a large map of Philadelphia,
patients search for symbols (selective attention)
• Elevator Counting: subjects listen to a series of tones, and
must indicate a floor number
• Visual Elevator: subjects must count up and down in
response to a series of visually presented "floors"
• Telephone Search: subjects must identify symbols in a
simulated telephone directory, in some versions counting
audio tones at the same time
• Lottery: subjects are asked to listen for their 'winning
number' presented on audio tape, then write down the
two letters preceding a specified number

Long-term memory
• The majority of clinical memory tests focus on anterograde episodic
memory. Any test which involves the presentation of new material
which must then be recalled or recognized after a filled delay is a
test of anterograde episodic memory.
• It is important to incorporate a time delay filled with unrelated
activity so that subsequent retrieval invokes long-term memory
processes rather than just working memory rehearsal.
• No tool assess retrieval beyond a 30 minute delay
– Verbal anterograde memory tests typically use word lists, word pairs, names
or stories,
– visuospatial versions use pictures of objects or faces, or geometric shapes.

Retrograde long-term memory
• Fewer tests are available to assess retrograde long-term
memory in clinical practice.
• The autobiographical memory interview (Kopelman et al.,
1989) elicit episodic descriptions and personal semantic
knowledge from different phases in the lifespan.
• Experimental paradigms often make use of Famous Faces
and Famous Names tests, using stimuli from different eras.
General knowledge questions may be employed to tap
semantic knowledge about world history or factual
knowledge (Which US president was assassinated in 1963?;
What is the capital of France?, etc.).
• Semantic language tests such as the Pyramids and Palm
Trees Test (Howard and Patterson, 1992) facilitate
exploration of object-related semantic knowledge based on
words and/or pictures.

Implicit long-term memory
• rarely assessed in typical clinical practice
In research:
• perceptual priming assessed by word stem
completion
• procedural learning include simple pursuit rotor
tasks as well as maze completion, mirror
drawing and the serial reaction time paradigm,
all of which are designed to elicit practice
effects regardless of conscious awareness of
prior learning.

Procedural memory
• refers to the acquisition and retention of
perceptomotor skills.
• These memories are accessed and applied
without the need for recalling information
relating to the event where the skill was
acquired.
• Mirror- reading tasks involve reading mirror-
transformed words,

Prospective memory
• Batteries for the assessment of this aspect of
memory include:
– the Cambridge Prospective Memory Test (Wilson
et al., 2005)
– the Memory for Intentions Test (Raskin et al.,
2004).

How patient usually present with
amnesia?
Mild:
• Has difficulty recalling recent events, and relies increasingly on list
making or calendar.
• Needs occasional reminders or re-reading to keep track of characters
in a movie or novel.
• Occasionally may repeat self over a few weeks to the same person.
• Loses track of whether bills have already been paid.
Major:
• Repeats self in conversation, often within the same conversation.
• Cannot keep track of short list of ítems when shopping or of plans for
the day.
• Requires frequent reminders to orient to task at hand.
• They may appear disoriented in place or time because they have failed
to learn their location or have lost the ability to monitor and keep
track of ongoing events

Complex attention
Attention: is the capacity to perform a selective analysis of inputs
Types:
• Selective/ Focused attention is the ability to focus on a task or
a part of the environment and to ignore distracters
(conversation with surrounding noise)
• Sustained attention (vigilance)is the capacity to sustain the
focus for a significant period of time (no distractor)(reading
article)
• Divided attention is the capacity to divide or share attention
between different tasks or different parts of the environment.
The ability to divide attention is closely related to information
processing capacity. (Multitasking):(mobile while driving)
• Alternating attention/ Cognitive flexibility is the ability to shift
attention appropriately and adaptively from one part of the
environment to another.(cooking from a cooking book)
Attention could be auditory, visual, or spatial

Attention
• General statements about ‘the attention’ of a patient should be
avoided. The situation (or the task) to a large extent determine
which aspects of attention will be essential and whether deficits
will become apparent. A patient’s attention may be adequate for
a social chat, but inadequate for driving a car through dense
traffic in rush hour.
• Attention functions are not autonomous but play a role in all
cognitive processes, such as perception, memory, behavioral
planning and actions, speech production, and reception and
orientation in space, to name.
• Therefore, attention functions are very difficult to separate both
conceptually and functionally from other cognitive functions.
• Moreover, when attention problems are severe, the patient may
be unable to benefit from rehabilitation even when motivation,
reasoning, judgment, and memory functions are relatively intact

Brain presentation of attention
1. Ascending reticular activating system
2. Superior colliculus
3. Thalamus
4. Parietal lobe
5. Anterior cingulate cortex
6. Frontal lobe

Presentation
Mild:
• Normal tasks take longer than previously.
• Begins to find errors in routine tasks
• finds work needs more double-checking than previously.
• Thinking is easier when not competing with other things (radio, TV, other
conversations, cell phone, driving).
Major:
• Has increased difficulty in environments with multiple stimuli (TV, radio,
conversation)
• is easily distracted by competing events in the environment.
• Is unable to attend unless input is restricted and simplified.
• Has difficulty holding new information in mind, such as recalling phone
numbers or addresses just given, or reporting what was just said.
• Is unable to perform mental calculations.
• All thinking takes longer than usual, and components to be processed
must be simplified to one or a few.

Assessment
• The basic approach to the assessment of attention is
clinical observation .
• During bedside conversation or an apparent social chat,
the psychologist may observe whether the patient is alert
and attending to the environment and the investigator, or
not.
• During formal assessment one may note whether patients
are distracted by noises from outside, and whether they
are attending adequately to tasks that in themselves, are
not considered attention tests.
• Observation can be standardized by the use of rating scales
– The Neurobehavioral Rating Scale, devised by Levin et al . (
1987 ),
– the Rating Scale of Attentional Behaviour (RSAB) was
developed by Ponsford and Kinsella ( 1991 ).

• Sustained attention: Maintenance of attention over time
(e.g., pressing a button every time a tone is heard, and
over a period of time, Paced Auditory Serial Addition
Task).
• Selective attention: Maintenance of attention despite
competing stimuli and/or distractors: hearing numbers
and letters and asked to count only letters, cancellation
test, trail making B)
• Divided attention: Attending to two tasks within the same
time period: rapidly tapping while learning a story being
read. Cancellation while digit backward
• Processing speed can be quantified on any task by timing
it (e.g., time to put together a design of blocks; time to
match symbols with numbers; speed in responding, such
as counting speed or serial 3 speed).

‘speech’ Vs. ‘language’
• Language: is a non- instinctive, culturally driven system
of voluntarily produced symbols, comprising receptive
and expressive abilities allowing comprehension and
communication of information respectively.
• Understanding and processing sound, word, phrase,
sentence, and conversation involves retrieving
vocabulary, concepts, grammar, and, on a higher scale,
processing abstract inferences, idioms, or verbal
problem- solving.
• Speech : the highly coordinated rapid motor function
responsible for the actual act of vocal expression of
language.

Localization of language
• The ‘language network’— areas that are activated across a
variety of language tasks across most healthy individuals.
These areas include:
1. Posterior inferior frontal cortex (Brodmann’s area (BA) 44 and
45, BA 6)
2. Posterior middle/ superior temporal cortex (in BA 22 and
often BA 20/ 21)
3. Angular gyrus (BA 39),
4. Posterior inferior temporal cortex (in BA 37).
• it has been established that core language functions are
left- lateralized in the majority of both right-handed left-
handers (95 per cent and 75 percent, respectively).

Functional MRI
• Panel (a) Areas
activated during
word generation.
• Panel (b) Areas of
activation
throughout picture
naming.
• Panel (c) Areas of
activation during
passive watching and
listening to video
with language

DSM V-Language
• Include the following processes:
– naming
– word finding
– Fluency
– grammar and syntax
– Receptive language
• The production of spontaneous speech involves the use
and coordination of multiple cognitive and physiological
processes including:
– retrieval from semantic and episodic memory
– the ability to sustain and divide attention for error
monitoring,
– the reliance upon working memory for syntax production

Disorders of language
• Aphasia is difficulty producing and/ or
understanding spoken language.
• Impairments in reading (alexia) and writing
(agraphia) are often associated with aphasia.
• Well assessed by research in vascular aphasia

PresentationMild:
• Has noticeable word-finding difficulty.
• May substitute general for specific terms.
• May avoid use of specific names of acquaintances.
• Grammatical errors involve subtle omission or incorrect use of
articles, prepositions, auxiliary verbs, etc.
Major:
• Has significant difficulties with expressive or receptive language.
Often uses general-use phrases such as "that thing“ and "you
know what I mean," and prefers general pronouns rather than
names. With severe impairment, may not even recall names of
closer friends and family.
• Idiosyncratic word usage
• grammatical errors,
• Stereotypy of speech occurs; echolalia and automatic speech
typically precede mutism.

Vascular aphasia
• The classic Broca– Wernicke– Lichtheim– Geschwind model
was the result of the efforts of Broca, Wernicke, and
Lichtheim in the nineteenth century, with later
modification by Geschwind in 1967.
• Broca proposed that part of the second or third
convolutions of the left inferior frontal gyrus has a role in
speech production, called the faculty of spoken language.
• Wernicke observed that lesions in the posterior aspect of
the superior temporal gyrus resulted in impaired
comprehension and fluent but gibberish speech. He
suggested this posterior superior temporal gyrus has a role
in speech perception through its connections with other
language areas.

Vascular aphasia syndromes
• Broca’s aphasia (agrammatism, verbal apraxia, and preserved comprehension)
• Wernicke’s aphasia: comprehension and repetition impairments, anomia, semantic
paraphasias (semantically related word substitutions) and phonemic paraphasias
(phonologically related word or nonword substitutions), and neologisms (jargon words).
Alexia and agraphia are noted
• conduction aphasia: a relatively fluent, though phonologically paraphasic speech; poor
repetition (recently poor repetition was linked to impaired working memory)
• Transcortical motor aphasia: (poor spontaneous speech, with relatively good
repetition and comprehension)
• Transcortical sensory aphasia: fluent, circumlocutory speech with semantic jargon and
poor comprehension. The key feature that distinguishes it from Wernicke’s aphasia is
preserved repetition.
• Transcortical mixed aphasia (isolation aphasia):Repetition is preserved but there is
reduced spontaneous speech, echolalia, and palilalia, or even mutism, along with
impaired comprehension, reading, and writing.
• Global aphasia: reduction of all faculties of language, including comprehension and
speech output
• Subcortical aphasia: (cause language deficits, ranging from anomia to global aphasia.
Fluctuating jargon aphasia with impaired fluency is often observed in patients with
thalamic lesions)

Primary progressive aphasia
• The term primary progressive aphasia (PPA) has been used to
encompass all patients with progressive language impairment
as the initial feature of a degenerative disorder.
Subtypes:
• Semantic dementia: people gradually lose their knowledge of
words
• Progressive non-fluent aphasia (PNFA): people’s speech
becomes effortful and they might say the wrong word or use
the wrong grammar
• Logopenic aphasia (LPA): people start to develop pauses in
their speech as they try to find the right word
• The PPA subtypes do not correspond closely with the acute
aphasia syndromes of stroke, due to differing functional and
structural neuroanatomical patterns of involvement and the
progressive nature of the disease.

PNFA SA LA
effortful, nonfluent speech confrontation naming difficulty Impaired word finding
agrammatism word finding is profoundly
impaired
No grammatic error
disordered prosody (the rhythm
or melody of speech
impaired comprehension
of single words
Impaired comprehension
(phrases) mixed with impaired
expression
Phonological errors Speech might seem empty of
content because of frequent use
of words that have imprecise
reference (for example, ‘these’and
‘that’)
Phonological errors
motor-based speech planning
errors (apraxia of speech)
Impaired insight, social cognition Associated with episodic memory
affect
patients typically become mute Impaires the recognition and use
of objects (agnosia and apraxia)
Impaired repetition of phrases
Spared comprehension Spared repetition, motor speech Spared motor speech spared
single word comprehension and
object knowledge

Assessment
Expressive language:
• Confrontational naming (identification of objects or
pictures)
• fluency (e.g., name as many items as possible in a
semantic [e.g., animals] or phonemic [e.g., words
starting with "f“ in 1 minute).
• Grammar and syntax in spontaneous speech
Receptive language:
• Comprehension (word definition and object pointing)
• performance of actions/activities according to verbal
command
Reading and writing and other agnosia and apraxia
tasks should be included

Aphasia batteries
• Boston Diagnostic Aphasia Examination
(BDAE-3)
• Communication Abilities in Daily Living (2nd
ed.)
• Comprehensive Aphasia Test (CAT)

Social cognition
• Social cognition refers to a set of
neurocognitive processes underlying the
individuals’ ability to “make sense of others’
behavior” as a crucial prerequisite of social
interaction
• Social cognition, including emotional face and
prosody perception, empathy, theory of mind,
and humor processing

subdomains
1. Theory of mind: ability to understand the mental states of
others, and to appreciate that these mental states might
differ from our own.
2. Affective empathy: one’s emotional response to the
perceived situations of others.(emotional responses could
be in parallel to other’s emotions(empathy) or distinct from
their emotions (non empathy)
3. Social perception: recognizing basic social and emotional
cues, such as interpreting facial expressions, body language
or voices, or social cues, such as eye gaze
4. Social behavior: one’s response to own social perception

Social cognitive impairments
• Social cognitive impairments are a prominent concern, or even
a core facet, of several neurodegenerative (e.g., behavioral
variant of frontotemporal dementia), neuropsychiatric (e.g.,
schizophrenia, major depressive disorder, and bipolar
disorder), and neurodevelopmental (e.g., autism spectrum
disorder and attention deficit hyperactivity disorder)
conditions, and often occur after acute brain damage (e.g.,
traumatic brain injury and stroke).
• Moreover, such deficits are critical predictors of functional
outcomes because they affect the ability to create and
maintain interpersonal relationships, thereby removing their
benefits in everyday life
• Social cognitive disturbances might be relatively subtle and
harder to detect informally. Structured social cognitive
assessment is, therefore , mandated.

Indicators of impaired social cognition
• Social withdrawal or avoidance of social contact
• Limited eye contact
• Rude or offensive comments without regard for the feelings of others
• Loss of etiquette in relation to eating or other bodily functions
• Extended speech that generally lacks focus and coherence
• Neglect of personal appearance (in the absence of depression)
• Disregard of the distress or loss of others
• Inability to share in the joy or celebrations of others when expected or invited
• Failure to reciprocate socially, even when obvious social cues are given
• poor conversation
• Overtly prejudicial or racist behaviour
• Increased or inappropriate interpersonal boundary infringements
• Failing to understand jokes or puns that are clear to most people
• Failure to detect clear social cues, such as boredom or anger, in conversational
partners
• Lack of adherence to social standards of dress or conversational topics

Neuroanatomical disturbances
• social cognition imposes demands on a large number of
different brain structures and their connectivity
• lesions in the orbitofrontal cortex (OFC) are associated
with disinhibited behaviours, such as social
inappropriateness, hypersexuality and compulsive
gambling.
• Lesions in the anterior cingulate cortex (ACC) are
associated with behavioural disturbances that include
abulia, or its more severe form akinetic mutism.
• Damage to the temporoparietal junction has been shown
to disrupt the ability to view a situation from an another
person’s perspective.
But other domains as visual perception, attention also
contribute

Clinical assessment of social cognition
• TOM: false belief (children), faux pas, The Awareness of Social
Inference Test (TASIT)
• Affective empathy: emotionally arousing videos or
photographs are presented to participants, who are asked to
rate their emotional response. One such measure is the
Multifaceted Empathy Test (MET)
• Social perception: Reading the Mind in the Eyes Test
(RMET), the Ekman Faces tests.
• Social behaviour: Patients’ self-report data might be
distorted owing to a lack of emotional insight, known as
frontal anosodiaphoria. Thus the caregiver is the source of
data needed for assessment. E.g. The Frontal Systems
Behaviour Scale (FrSBe), the Frontal Behavioural Inventory
(FBI), the informant-rated Socioemotional Dysfunction Scale
(SDS

perceptual-motor domain
Identification and manipulation of figures, maps
and items; motor tasks and gestures, recognition of
faces and hand-eye coordination.
Includes
1. Visual perception
2. visuospatial
3. Visuoconstructional
4. Praxis
5. Gnosis

Clinical observations
Difficulty in:
1. Finding way when driving (especially if
rerouted) (visuospatial task)
2. finding way back to the table after going to
public restroom (visuospatial task)
3. Telling time on analog clock
4. Performing learned tasks (using knife/fork and
tools)(Difficulty with praxis)
5. Recognizing faces (prosopagnosia), recognizing
object, voices.

Presentations
Mild:
• May need to rely more on maps or others for directions.
• Uses notes and follows others to get to a new place.
• May find self lost or turned around when not concentrating on
task.
• less precise in parking.
• Needs to expend greater effort for procedural tasks such as
carpentry, assembly, sewing, or knitting.
Major
Has significant difficulties with:
• previously familiar activities (using tools, driving motor vehicle)
• navigating in familiar environments;
• often more confused at dusk, when shadows and lowering
levels of light change perceptions.

Localization
• Visual perception: Nondominant primary visual
and association cortices, temporooccipital areas
• Spatial processing: visual association cortices,
occipitoparietal lobes
• Praxis: left parietal lobes and prefrontal areas

Visual perception assessment
• Perception is not a passive process, but is
modulated by attention. There is feedback from
higher order centers down to primary sensory
cortex. Similarly, attention influences what is
perceived.
• Visual perception can be evaluated by :
– asking the patient to locate an object in the room
– various tests of simultagnosia, whereby the patient
must distinguish between parts and the whole, e.g.,
when trying to see a large letter “A” made up of small
“E’s.”
– Letter Cancellation

Abnormal Visual perception
1. Hemineglect (Abnormal Visual perception,
Visuospatial skill)
– In letter cancellation
– Line bisection
– Copying a symmetric figure flower , star, clock
2. Visual agnosia (Abnormal Visual perception)
– include object naming and ability to provide semantic
information about unnamed items
3. Alexia in absence of agraphia (abnormal visual
perception)

4- Prosopagnosia refers to a patient’s inability to recognize a
person simply by studying their face.
• Once other means of recognition come into play (for
example, if the person has a characteristic voice or gait,
etc), this allows access to unique semantic identifying
information—that is, there is no loss of knowledge of the
person. including one's own face (self-recognition)
• occur with posterior cerebral artery infarcts.(affection of
right fusiform gyrus, infero-medial part tempro-occipital
region) can be bilateral.
– Unilateral left temporo-occipital lesions result in object
agnosia, but spare face recognition processes
• Less common in alzheimer’s disease
• Assessment batteries:
– The Cambridge Face Memory Test
– The Benton Facial Recognition Test

Praxis and apraxia
• Praxis is defined as the ability to perform
skilled or learned movements.
• Apraxia refers to the inability to carry out such
praxis movements in the absence of
elementary motor, sensory or coordination
deficits that could serve as the primary cause.

Apraxia
• Apraxia is a disorder of motor cognition.
• it reflects an impairment of the storage and
transformation of motor representations in the
brain
• Apraxia can be further classified into subtypes
such as ideomotor, ideational, limb kinetic
• task-specific apraxias such as dressing apraxia,
sitting apraxia, apraxia of eyelid opening and
apraxia of gait have been noted

• the left parietal lobe stores a ‘space–time form
picture’ of a movement.
• For a movement to be executed, its picture must be
retrieved and activated and then be associated via
cortical projections with the relevant motor engrams
in the prefrontal regions.
• From here the information passes to the primary
motor cortex before being fed down the
corticospinal tracts.
• For the right upper limb to move the information
remains contained within the left hemisphere, but
for the left upper limb to move the information from
the left parietal lobe must first be sent to the right
prefrontal and frontal regions through the corpus
callosum

IDEATIONAL APRAXIA
• The concepts of movement and intent are degraded.
• Patients may not comprehend the appropriate use for a tool
• Patients presented with a pair of scissors can name the object correctly
but may be unable to describe their use. When the examiner
demonstrates their use, patients may be unable to discriminate between
poorly executed movements and properly executed movements. When
handed the item themselves, they may struggle to use them to cut a
sheet of paper. This demonstrates a loss of the conceptual or semantic
knowledge about what scissors are and what they are for.
• patients with ideational apraxia also cannot pantomime tool use to
command.
• They can’t perform simple gestures and may be unable to show how to
wave goodbye.
• Because the action production system remains intact, such patients
should be able to perform these tasks when the examiner gives them a
visual demonstration (intact imitation)

IDEOMOTOR APRAXIA
• is a disorder of the action production system. Affected
patients display errors in the scaling, timing and
orientation of movements and may also omit or repeat
individual elements of the overall action being assessed.
• It is disorder of gesture performance upon verbal
command, despite having intact knowledge of tasks. For
example, the patient might be able to describe how to
use a spoon, but not able to demonstrate the actual use.
• However, when the ability to perform an action
automatically when cued remains intact, this is known as
automatic-voluntary dissociation. For example, they may
not be able to pick up a phone when asked to do so, but
can perform the action without thinking when the phone
rings.

LIMB-KINETIC APRAXIA
• many patients with corticobasal syndrome
have limb-kinetic apraxic deficits.
• the characteristic feature of which is the loss
of co-ordination between the fine,
individuated finger movements that are
required to perform a skilled task.
• easily demonstrated by asking the patient to
oppose their thumb to their index, middle,
ring and little fingers rapidly in turn.

Assessment of praxis
• examining for apraxia includes:
1. pantomime tool use
2. generate meaningful and meaningless hand
gestures
3. perform a motor sequencing task
• Valid tools:
– Florida Apraxia Screening Test-Revised (FAST-R)
– Apraxia Screen of TULIA

• a poor performance might also result from
impaired working memory or executive function

Visuospatial tasks
• Visuospatial function refers to cognitive processes
necessary to "identify, integrate, and analyze space and
visual form, details, structure and spatial relations" in
more than one dimension.
• Visuospatial skills are needed for movement, depth
and distance perception, and spatial navigation.
• Visuospatial skills are needed for many activities of
daily living: dresssing, transfers, reading the clock,
driving, navigation), they are important predictors for
rehabilitation outcome
• Early impairment in visuospatial function is found in
dementia with Lewy bodies.

VS assessment
• Line bisection
• Clock drawing tasks
• Cube analysis

Line bisection
• Unilateral neglect

Cube Analysis .
• The patient has to interpret three-dimensional
space in two- dimensional representations.
Ask the patient to count the number of solid
bricks.

Visuoconstructive
• Visuoconstructive tasks include components
of perception, motor behavior, and spatial
orientation.
1. copy figures at different levels of complexity.
2. The Rey Osterrieth Complex Figure.
3. Block design test
4. Three-Dimensional Construction(Block
construction)

Letter cancellation task
It assesses:
1. Selective attention
2. Processing speed
3. Visual perception
4. Visuomotor skill

Mild vs Major NCD
Cognitive Testing
• Mild: 1–2 standard deviation (SD) range
• Major: Below 2 SD

Cognition and cognitive syndromes cme

More Related Content

What's hot (6)

Similar to Cognition and cognitive syndromes cme (20)

More from Doha Rasheedy (20)

Recently uploaded (20)

Cognition and cognitive syndromes cme

Editor's Notes