Fundamentals of

Behavioral Neurology
Daniel Cohen
[email protected]
Some basics
• Human cortex contains 30 billion neurons,
each typically making >1000 synaptic
contacts
• Distinct cortical areas are specialized for
different jobs
• Most cognitive processes depend on
networks of interconnected areas rather
than one brain region
• Damage to a critical cortical region or
white matter pathways that connect regions
in a network can disrupt function
Major Large-Scale Networks
Domain Function
Default Mode Introspection
Salience Relevance/motivation
Declarative Memory Conscious recollection of facts and prior events
Language Use of symbols for communication
Spatial Attention 3D representation of the world relative to our bodies
Executive Control Allocating attention, engage/inhibit behaviors, problem solving
Object/Person Recognition Link between perceptual features and identity
Object Manipulation Learned motor programs for skilled movements
Acute Neurobehavioral Syndromes
• Left hemisphere
• Aphasia
• Gerstman’s Syndrome
• Apraxia
• Alexia without agraphia

• Right hemisphere
• Hemispatial Neglect

• Bilateral
• Balint Syndrome
• Object agnosia/Prosopagnosia
• Frontal executive syndromes
• Amnesia
• Diffuse
• Acute confusional state (encephalopathy, delirium)
Aphasia
• Primary disturbance in language, ie the ability to use symbols for communication
• Broadly classified by dysfunction in:
• Fluency, the ease of producing motor output to generate language symbols
• Comprehension, the ability to extract meaning from sensory language symbols
• Repetition

• Assessed by:
• Spontaneous speech output
• Response to commands and yes/no questions
• Reading
• Writing to dictation
• Testing the ability to name objects or words in a category (example: fruits)
• Note all patients with aphasia report trouble with word finding (anomia)
• Testing the ability to repeat words or phrases
 Classic (peri-Sylvian) Aphasias
• Broca’s (non-fluent or expressive) aphasia
• Speech is halting, effortful, with reduced phrase length (<5
words), “telegraphic” omitting articles
• Paraphasic Errors in speech
• Phonemic (or phonological):  abnormal sequencing of small
sound chunks (phonemes) to form words (ex: “Smole” for
“Smile”)
• Semantic, substituting related words (ex: “mother” for
“daughter”)
• Writing and typing are slow
• Comprehension generally spared for single words or most
phrases but mildly impaired for complex grammar (such as
passive clauses)
• Not explained by a primary problem with motor control for
face/throat/hand muscles (for example, weakness of the
tongue, lips, or pharyngeal muscles can cause dysarthria, in
which the target output is correctly selected but the
articulation is faulty causing a slurred or distorted sound
 Classic (peri-Sylvian) Aphasias
• Wernicke’s (receptive) aphasia
• Impaired comprehension for listening and reading
• Note, think of severity continuum and not all or none
• Speech is easily produced, with normal rate,
rhythm, and phrase length
• Phonemic and semantic errors present in speaking,
writing, and typing
• Content of speech (or writing/typing) is often
called “word salad”, mirroring the problem with
comprehension and hard for a listener to obtain
meaning from what is said
• Not explained by a primary sensory deficit
• Patients often lack awareness of their deficit and
don’t seem as frustrated as those with Broca’s
aphasia
 Classic (peri-Sylvian) Aphasias
• Conduction aphasia
• Damage to the arcuate fasciculus, the white matter X
bundle between Wernicke’s and Brocas areas; may
also occur from damage to cortex at the interface
of left parietal and temporal lobe
• Primary problem with phonemic processing:
• Phonological errors in spontaneous speech
and more so when testing repetition
• Poor verbal working memory, with inability
to keep a long sound stream in mind
• Fluency and comprehension are (near) normal
• Short word-finding pauses may be present
intermittently
• Comprehension for long phrases may be
impaired
 Classic (peri-Sylvian) Aphasias
• Global aphasia
• Large lesions encompassing both anterior (near
Broca’s area) and posterior (near Wernicke’s)
• Impaired fluency, comprehension, repetition,
naming, reading, and writing
 Transcortical Aphasias
• Lesions outside of the Peri-sylvian region
• Repetition is spared
• Transcortical motor aphasia (purple)
• Sound like Broca’s aphasia but output is
much easier to repetition than spontaneous
• Transcortical sensory aphasia (blue)
• Comprehension like Wernicke’s but
repetition is accurate unlike Wernicke’s
• Transcortical mixed aphasia
• Like global but spared repetition; when
severe may cause echolalia – parroting but
not understanding another speaker
Gerstman’s Syndrome
• Features
• Agraphia (or dysgraphia)
• Acalculia
• Left/right confusion
• Finger agnosia (actually a
problem with naming)
• Lesion typically in the angular
gyrus (green shading) of the
language dominant parietal lobe
• Integrates language with
mapping body coordinates;
primitive math likely relied on
counting with fingers
Apraxia
• Ideomotor apraxia
• Errors in the timing and spatial execution of
individual actions
• Ex: using a screwdriver by rotating the shoulder
and moving the elbow instead of rotation at the
wrist to move the hand
• Inferior parietal and middle frontal gyrus
(Yellow shading in figure), contralateral to
the dominant hand
• Ideational apraxia
• Trouble with sequencing a series of actions
• Localization within the left hemisphere less
clear
Alexia without Agraphia – disconnection syndrome
• You will learn visual pathways, but a short-cut:
• Each occipital cortex receives visual input from the opposite side
of space
• Damage to the left occipital lobe can causes inability to see to the
right of midline

• The back (splenium) of the corpus collosum is

necessary to transmit information about the left
side of space from the right occipital cortex to the
left dominant language network
• Damage disconnects visual symbols, disrupting the ability to read

• Writing is preserved, but patients can’t read what they

wrote
Canadian Journal Neurosci 2020; 48(5):1-9
Hemi-spatial neglect
Normal Right hemisphere lesion Left hemisphere lesion

X X
Left Right
 Hemispatial Neglect - examples
Drawing – external model Target cancellation Behavioral observation

Drawing – internal model Line bisection  Testing for extinction

Visual perception: parallel processing
Dorsal Stream:
vision to guide action (how/where)

Occipital lobe
primary visual cortex
Ventral Stream: (conscious image detection)
vision to identify (what)
Dorsal visual stream: Balint Syndrome
• Optic ataxia (can be unilateral)
• poor reaching to a visual target, unable to compute the
correct trajectory
• Reaching is typically with a curved or meandering
path but not a straight line
• Eventually hone in on target by trial and error

•  Occulomotor Apraxia
• unable to volitionally direct eye movements to center
(fixate) on a visual target

• Simultanagnosia
• Only able to see small parts of a scene at one time but
not the whole picture
Stimuli from the
NIH Stroke Scale
• Example – cookie theft picture may only see one
person or part of a person at once
• One feature comes into awareness as another vanishes
 Ventral Visual Stream Disorders
Object agnosia
• Inability to recognize an object by sight
• Ventral occipitotemporal junction
• Typically, bilateral damage, at times right
unilateral
• Some patients can accurately draw what
they see but still can’t identify what it is
Prosopagnosia 
• Inability to discriminate identity by facial
features
• Fusiform gyrus – anterior inferior temporal
Frontal Executive Disorders
• Executive Functions are a family of processes that allow for utilizing resources
and time efficiently to execute adaptive, goal-directed, and socially appropriate
actions (think of the skills of a CEO)
• Examples
• Manipulating contents of working memory
• Example Digit span backwards, reversing the months of the year
• Set shifting
• Juggling between two streams of thought such as letter-number sequencing (1a, 2b, 3c, 4d)
• Flexibly shifting an approach when one strategy isn’t working (Wisconsin Card Sort Task)
• Inhibiting “pre-potent” responses
• Example: Stroop task – name the color of font rather than read the word: RED BLUE WHITE
• Planning
• Example test: Tower of London
• Involves thinking moves ahead to reach a goal
with the least number of moves
 Alpha 2 receptors

Frontal Executive Syndromes

increase functional
connectivity

• Dorsolateral prefrontal – online holding information

• Distractible
• Lose train of thought
• Can learn only small amounts at once (info overload)
• Misplace items

• Orbitofrontal – balancing social rules with drive

• Disinhibited behavior (no filter, no brakes)
• Stimulus bound (use objects “just because they are there”)
• Trouble regulating emotion
• Mostly the lateral orbitofrontal cortex involved with inhibition

• Medial frontal – energizing, engaging drive/reward

• Pathological apathy/indifference (called abulia extreme is akinetic mutism)
• Emotional blunting
• May appear depressed but lack the subjective sadness
• No “gas pedal” or “ignition switch”
• Medial orbitofrontal and cingluate
Amnesia: Case of HM
• Epilepsy surgery at age 27
• Bilateral medial temporal lobe resection
• Inability to learn new facts or recall events
following surgery (dense anterograde
amnesia)
• No recall of events 3 years prior to surgery,
loss of detail for events 3-11 years prior
(graded retrograde amnesia); medial temporal
lobe not needed to recall memories after
several years, termed “system consolidation”
• Digit span (working memory) normal at 6
digits Scoville & Milner (1957)

• Preserved procedural memory

Syndrome of Transient Global Amnesia (TGA)
• Striking presentation in which an individual can’t retain new information after delays of 1-2
minutes (repetitive questions, like a “broken record”)
• Dense Anterograde Amnesia lasts for up to 24 hours, typically 2-12 hours with a mean of 6h
• Retrograde Amnesia spans hours, days, weeks, rarely months or years
• Retrograde Amnesia “shrinks” as the syndrome resolves, typically with a permanent
retrograde gap of hours to 1 day
• Mechanism is unclear (venous congestion and/or glucocorticoid stress response)
• Benign prognosis, recurrence rate ballpark 5% (range 2-26%)
• Distinct pattern of memory impairment from fugue states (“Hollywood Amnesia”)
Acute Confusional State (delirium,
encephalopathy)
• Timescale typically over hours, days, or weeks
• Inability to maintain a coherent stream of thought or action
• Deficit not limited to one cognitive domain (ex: language), sensory modality (ex: vision), or
output channel (ex: speaking vs writing)
• Hallmark is poor attention
• Poor orienting to examiner
• Need frequent prompts to stay on task
• Highly distractible by irrelevant stimuli
• Variable responses to the same question or task (low reliability)
• May appear drowsy or increased motor activity (pacing, restless)
• Alertness and attention “wax and wane” over minutes-to-hours
• Can be hard to distinguish from Wernicke’s aphasia – in confusion phrases are generally
grammatically correct but are out of context to situation, often rambling about an irrelevant topic
• Typically diffuse insult (including metabolic), occasionally focal right parietal lesion
Chronic Cognitive Impairment - terminology
• More enduring cognitive decline lasting months or years
• Mild Cognitive Impairment (MCI) – new term Mild Neurocognitive Disorder
• Subjective and objective change in cognition that is not normal for the aging process, but
the individual remains “essentially” independent
• Most commonly memory domain, termed amnestic MCI
• May be multiple cognitive domains
• Dementia – new term Major Neurocognitive Disorder
• Change in cognition compared to baseline that is severe enough to render someone no
longer independent
• Typically involves memory and at least one other cognitive domain
• It is a syndrome based on function, not a disease process
• Differential includes many mechanisms from D&E VITAMINS
• Very rarely reversible, such as from “depressive psuedodementia” or nutritional deficiency