Domains of Neuropsychological Function and Related Neurobehavioral Disorders

Question 1

Intelligence

Answer 1

Associated disorders:

Intellectual Disability

Dementia

Savantism

Question 2

Intellectual Disability

Answer 2

Intellectual disability is a developmental disorder with onset before age 18 requiring a substantially subnormal IQ (i.e., approximately ≥ 2 standard deviations below the mean), reflecting limitations in general intellectual functioning, combined with significant deficits in two or more adaptive skills (see also Chapter 13, Intellectual Disability).

Question 3

Dementia

Answer 3

By definition, dementia is a syndrome (i.e., a set of signs and symptoms) that stems from a disease or medical condition involving a decline in or loss of general cognitive ability or multiple areas of cognitive impairment of sufficient severity to impair social and/or occupational functioning.

Performances on tests of general intelligence decline as dementia advances; in cases of Alzheimer’s disease (AD), decline in IQ may not be evident until the middle stages

Question 4

Savantism

Answer 4

This is a very rare syndrome in which individuals with an intellectual disability or autism spectrum disorder have one or more specific or narrow remarkable talents that exist in stark contrast to their intellectual disability.

The cause or causes of savant syndrome are unknown but may be congenital or acquired as a result of central nervous system (CNS) disease or injury.

Savant syndrome is associated with autism spectrum disorders, as well as other psychological disorders and CNS injuries/diseases.

It is approximately six times more common in males than females.

Savant skills most commonly involve exceptional memory, but can also involve exceptional calculation, calendar knowledge, artistic, and/or language abilities.

Question 5

Attention/Concentration and Processing Speed

Answer 5

In general, attention refers to the process whereby individuals receive and subsequently process incoming information.

It is closely associated with perception, executive functioning and memory, particularly working memory (which is dependent on attention but is not synonymous).

Working memory refers to the form of processed information before it is sent to short-term memory (see also the section “Memory” later in this chapter), whereby information that is being actively maintained or rehearsed can be retained for up to several minutes.

If something is passively received (i.e., attention span) but not rehearsed or manipulated, it is lost.

Question 6

AttentionProcessing Speed Associated disorders

Answer 6

Delirium

Attention-Deficit/Hyperactivity Disorder (ADHD)

Hemispatial Inattention (a.k.a. Neglect)

Traumatic Brain Injury (TBI)

Other Disorders and Factors Affecting Attention

• Depression and anxiety, fatigue and lack of sleep/sleep disorders, low or poor arousal, environmental factors (i.e., noise, etc.), and medications are all non-neurological factors that can negatively influence attentional processes.
• Reduced motivation or effort is another factor that can affect attention.

Factors affecting processing speed include
- conditions that diffusely impact various brain structures and white matter integrity (e.g., multiple sclerosis, TBI, vascular cognitive impairment, Parkinson’s disease).

Question 7

Comprehension of Spoken and Written Language

Answer 7

Comprehension difficulties can be of two types: syntactic and lexical/semantic.

Lesions involving anterior speech areas can result in disturbed comprehension of phonological (syntactic) information used to construct word names.

Lesions in posterior language areas more often result in disturbed comprehension of the sequencing of meaningful word sounds to convey meaning (lexical/semantic).

Question 8

Repetition

Answer 8

Repetition is easily tested by beginning with single, simple words then multisyllabic words, followed by short sentences and longer sentences, increasing in complexity.

Establishing whether or not repetition is intact is important because the ability to repeat typically indicates that the perisylvian language centers are functional.

Question 9

Naming problems or anomia (the terms “anomia” and “dysnomia” can be used interchangeably) can be present in all types of aphasia syndromes and can involve problems naming an object, color, and body part or finding a specific word in spontaneous speech.

Answer 9

Word-finding difficulties can be evident in spontaneous speech when a patient pauses to search for a word or uses too many words to describe something better described in fewer words (i.e., circumlocution), or it can be elicited by presenting a stimulus and asking the patient to name it.

Anomia can occur with many of the aphasic syndromes and is, therefore, not generally useful for localization, but distinctions between anterior and posterior aphasic syndromes can often be made because patients with nonfluent syndromes may have difficulty with naming as a result of initiation or production problems, whereas patients with fluent syndromes may have difficulty as a result of selection problems or an inability to find the right word.

Question 10

Reading aloud and reading silently for comprehension should each be tested separately.

Answer 10

All aphasic syndromes that include severe impairment of auditory language—with the exception of pure word deafness—are almost always also associated with an acquired impairment of reading or alexia.

Alexia can be seen in aphasias of all kinds and it can be seen in isolation, in which case it can be important for localization.

That alexia can be seen in isolation without other features of aphasia, including agraphia, suggests that the brain has evolved specific areas required for reading that are separate from other language areas.

Question 11

transient global amnesia

Answer 11

medial temporal

diencephalic

Question 12

anoxia/hypoxia

Answer 12

medial temporal

Question 13

anterior communicating artery aneurysm

Answer 13

frontal

basal forebrain

Question 14

Wernicke-Korsakoff

Answer 14

diencephalic

Question 15

herpes encephalopathy

Answer 15

medial temporal

Question 16

PCA infarct

Answer 16

medial temporal

Question 17

Dementia

Answer 17

Whereas AD affects memory encoding

patients with dementia impacting frontal-subcortical systems (i.e., vascular dementia, multiple sclerosis, Parkinson’s disease, etc.) typically display retrieval difficulties and thus often benefit from recognition cueing

Question 18

Hippocampal Pathway (or Papez circuit)

Answer 18

• Entorhinal cortex
• Fornix
• Mammillary bodies
• Mammillothalamic tract
• Cingulate cortex

Hypoxia and Anoxia

Question 19

Amygdaloid Pathway

Answer 19

• Amygdala
• Dorsal medial thalamus
• Dorsomedial cortex

Herpes encephalitis

PTSD

Question 20

Diencephalon

Answer 20

• Anterior nucleus of the thalamus
• Dorsomedial nucleus of the thalamus
• Fornix
• Mammillary bodies
• Korsakoff’s
• CVA

Question 21

Basal Forebrain

Answer 21

• Medial septal nucleus
• Diagonal band of Broca
• Nucleus Basalis of Meynert
• Affected in AD
• ACoA aneurysm

Question 22

Cortex

Answer 22

• Medial and anterior temporal lobe
• Frontal lobe (see basal forebrain)
• Surgical ablation
• TBI
• Herpes
• Anoxia/Hypoxia

Question 23

Executive Functions

Answer 23

• Dorsolateral Prefrontal Syndrome (dysexecutive syndrome) Characterized by poor problem solving, word-list generation, organization, sequencing, abulia/amotivation (“pseudo depression”), and sometimes perseveration.
• Orbitofrontal Syndrome (inferior/ventral frontal syndrome) Characterized by emotional lability, impulsivity, disinhibition, childishness, personality change, and distractibility.
• Medial Frontal/Cingulate Syndrome Characterized by decreased initiation and indifference, but can also have amnesia, incontinence, and leg weakness. Cummings also identified the motor circuit (supplementary motor area) and oculomotor circuit (frontal eye fields).

Question 24

Executive Function Associated Disorders

Answer 24

Dementias

Acquired or traumatic brain injury

addiction/impulse control disorders

focal lesions affecting frontal circuits and systems

Question 25

sensorimotor associated disorders

Answer 25

finger agnosia

right-left disorientation

asterognosia

asomatognosia

apraxia

agraphia

Question 26

Emotional Symptoms Commonly Associated with Neurological Disorders

Answer 26

anosodiaphoria

alexithymia

emotional lability

rumination

pseudobulbar affect

Question 27

Abulia

Answer 27

Passive behavior wherein one exhibits little spontaneous activity and markedly delayed responses and tends to speak briefly or softly.

In the extreme the patient may be immobile, akinetic, and mute, but will continue to appear awake, sitting with their eyes open.

Question 28

Circumlocution

Answer 28

The purposeful substitution of multiple words or descriptions for a word or other words that the patient is unable to find.

This manifests as talking around word-finding difficulties, rather than using specific words.

These are not considered paraphasic errors, and circumlocution is not the equivalent of tangentiality.

Question 29

Crystallized Intelligence (Gc)

Answer 29

This refers to the breadth and depth of knowledge and understanding that is acquired through learning, education, experience, and acculturation; it is often resistant to the effects of aging and neurologic injury or illness.

Question 30

Dysarthria

Answer 30

A speech disorder involving difficulty in articulation.

Not an aphasia or language disorder although sometimes confused with them.

Patients with dysarthria are able to read and write normally which readily differentiates them from patients with expressive aphasia.

Question 31

Dysphonia

Answer 31

A speech disorder involving difficulty in vocalization.

Question 32

Fluid Intelligence (Gf)

Answer 32

This refers to the ability to reason, adapt, solve problems, form concepts, and understand relationships in unfamiliar or novel circumstances.

Unlike Gc, it is more susceptible to the effects of aging, injury, and illness.

Question 33

Literal (or Phonemic) Paraphasias

Answer 33

The substitution or the rearrangement of sounds or syllables in otherwise correct words (e.g., fig instead of pig).

Question 34

Phonology

Answer 34

The underlying speech sounds in a language and the rules governing the production of speech sounds.

Question 35

Pragmatics

Answer 35

The context in which the words are used.

Question 36

Prosody

Answer 36

An element of speech that contributes to the conveyance of meaning through rhythm, stress, pitch, loudness, tempo, and intonation.

It can indicate the basic structure of a sentence, the emotional content, or the main point in an utterance.

Question 37

Semantics

Answer 37

The meanings of words and the rules governing the use of words. It is the sequencing of meaningful words sounds to convey meaning.

Question 38

Semantic Paraphasias

Answer 38

The substitution of a correct word or phrase for another semantically related word or phrase (e.g., dog instead of cat).

Semantic paraphasias in the form of multi-word paraphasic errors or “paragrammatism” is one feature that differentiates the syndrome of Wernicke’s aphasia from other fluent aphasic syndromes.

Question 39

Syntax

Answer 39

The rules governing the structure of phrases and sentences.

Question 40

Verbal Paraphasias

Answer 40

The substitution of one correct word for another.

Some substitutions may have no obvious semantic relationship with the correct word, while others involve substitution of a correct word or phrase for another semantically related word or phrase.

Sometimes paraphasias take the form of neologisms (e.g., “zoctor” for doctor).

Question 41

A cancellation task with a single target would be a valid measure of ____.

Answer 41

selective attention

Selective attention is the choosing of a target from amidst distractors.

• Divided attention is concentrating on two or more tasks simultaneously.
• Attention span is similar to capacity.
• Sustained attention is maintaining attention over a period of time.

Question 42

The patient is a 72-year-old man who sustained a thrombotic stroke of the left middle cerebral artery three months ago.

Examination now reveals significant word-finding difficulty but otherwise grammatically accurate, fluent, and well-formed speech and intact repetition.

His speech lacks substantive words and there are frequent circumlocutions.

The most likely type of aphasia is ____.

Answer 42

anomic aphasia

The characteristics of an anomic aphasia are fluent speech and intact comprehension and repetition but impaired naming.

The patient appears to recognize objects but cannot come up with their name.

They may be able to describe concepts they are trying to express but cannot come up with the specific name; for example, they might say, “The animal with a mane that lives in the jungle and roars,” but they cannot say, “lion.”

Question 43

In the case described in question #2 above, where was the patient’s lesion likely to have been?

Answer 43

extrasylvian area

The extrasylvian lesion effectively isolates the parts of the brain needed for the interpretation of language from the parts of the brain that merely connect the interpretation of speech sounds or phonology to speech production.

Repetition is intact because speech sounds can be directly mapped into speech articulation without using the parts of the brain necessary for comprehension of speech.

Question 44

In acquired prosopagnosia, ____.

Answer 44

spontaneous recovery has been documented in some cases

There is also preliminary evidence of possible treatments for both acquired and developmental prosopagnosia.

Question 45

You are consulted to see a 79-year-old, right-handed gentleman who sustained a right thalamic stroke.

While preparing your doctoral intern for the initial assessment what might you provide in terms of education?

He may ____.

Answer 45

not express emotions

His right-sided CVA likely eliminates the possibility of an expressive aphasia.

B and D are indicative of orbitofrontal damage.

Right hemisphere lesions can result in aprosodias and other conditions which impact an individual’s ability to express emotion.

Question 46

On examination, you find that a patient picks up a toothbrush and the toothpaste and tries to put the toothpaste on the toothbrush without taking off the cap.

This could be an example of ____.

Answer 46

ideational apraxia

This limb apraxia is characterized by difficulty correctly sequencing motor movements.

Question 47

Your patient presents with nonfluent, sparse language and symptoms of apathy.

To differentiate this condition as an aphasia syndrome from a primary psychiatric disorder, you would want to first evaluate for the presence of ____.

Answer 47

motor or sensory deficits

Psychiatric conditions are rarely associated with focal motor or sensory neurologic symptoms, whereas hemiparesis is not uncommon in Broca’s aphasia.

While language errors can occur in acute psychosis as well as anosognosia but in these instances orientation will often remain intact and it is unlikely there will be a focal motor or sensory disorder.

Question 48

Which brain area plays a key role in motivating selective attention toward a salient stimulus?

Answer 48

anterior cingulate

Motivation and saliency involve executive systems such as the anterior cingulate (limbic).

Question 49

Posner and Petersen’s attentional model would predict difficulty with ____ as a result of damage to the ascending reticular activating system (ARAS).

Answer 49

alerting

Orienting and shifting (involves the posterior network)

detection (anterior network)

alerting and arousal (ARAS)

Question 50

Classification of aphasia is based primarily on which three parts of the language assessment?

Answer 50

fluency, comprehension, repetition

Fluent versus nonfluent speech localizes anterior from posterior aphasic syndromes;

impairment in comprehension distinguishes Wernicke’s from conduction aphasia;

and the ability to repeat implies that perisylvian language areas are intact.

Question 51

symptoms of Gerstmann’s syndrome

Answer 51

The four components of Gerstmann’s syndrome are

finger agnosia

acalculia

right-left disorientation

agraphia

Question 52

Intact spatial abilities, but disturbed recognition of objects implies disruption in the ____ pathway.

Answer 52

ventral

The ventral pathway is the “what” pathway; its disruption would affect recognition of objects.

The dorsal pathway, in contrast, is the “where” pathway, which affects spatial perception.

Question 53

Which of the following might you see with damage to the dorsolateral frontal lobe?

Answer 53

abulia

orbitofrontal damage: Emotional lability, lack of empathy, and Witzelsucht

Question 54

A 5-year-old boy demonstrates delayed language, parallel play, and an obsession with electrical outlets, but can recite the day of the week of any date.

He most likely demonstrates ____.

Answer 54

savantism

The patient described has autistic spectrum disorder, which has some association with savantism.

Question 55

You are called to evaluate a patient in the hospital with a history of alcohol abuse who tried to elope from his assisted living facility by climbing a tree two days ago.

Upon admission, his toxicology screen was negative.

Testing indicated poor orientation and attention, fair memory, and poor reasoning.

Your tentative diagnosis is ____.

Answer 55

delirium

The patient’s fair memory eliminates Korsakoff’s and TGA; his poor orientation and attention are consistent with delirium.

Symptoms following a true TIA would resolve quickly on the same day, which is not the case here.