Session 3:
Cognitive Problems
Definitions
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Dementia: clinical state characterized by loss of function in multiple
cognitive domains; diagnostic features include : memory impairment and at
least one of the following: aphasia, apraxia, agnosia, disturbances in
executive functioning. Cognitive impairments must be severe enough to
cause impairment in social and occupational functioning and there must be
a decline from from a previously higher level of functioning.
Acute confusional state: impairment of cognitive function that is not
progressive, but is reversible. The impairment of consciousness varies,
often being worse at night. It may be described as a transient organic brain
syndrome characterized by concurrent disorders of attention, perception,
thinking, memory, psychomotor behaviour and the sleep-waking cycle.
Delirium: acute cognitive and behavioral change with attentional problems
(analogous to above)
Encephalopathy: diffuse brain dysfunction (includes acute confusional state
and delirium)
Amnestic syndrome: Partial or total loss of memory, usually resulting from
shock, psychological disturbance, brain injury, or illness. (cf Bourne Identity)
Mental retardation: a disability characterized by significant limitations both in
intellectual functioning and in adaptive behavior as expressed in conceptual,
social, and practical adaptive skills beginning before age 18.
Schizophrenia: any of several psychotic disorders characterized by
distortions of reality and disturbances of thought and language and
withdrawal from social contact
Schizophrenia
Two (or more) of the following, each present for a
significant portion of time during a 1-month period (or less if
successfully treated):
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delusions
hallucinations
disorganized speech (e.g., frequent derailment or incoherence)
grossly disorganized or catatonic behavior
negative symptoms, i.e., affective flattening, alogia, or avolition.
Note: Only one symptom is required if delusions are bizarre or
hallucinations consist of a voice keeping up a running
commentary on the person's behavior or thoughts, or two or
more voices conversing with each other.
Man found down
• BP: 116/68; 104 HR; 99.5 F; 14 RR
• Opens eyes to voice; grimaces to pain;
unable to follow commands; blinks to
threat bilaterally
• Normal oculocephalics; symm reactive
pupils; facial symmetry
• Reduced tone with withdrawal of all
extremities to pain
Laboratory Findings
• Na 152, K 4.1, BUN 76,
Cr 2.1; Glc 116
• AST/ALT: 23/47; INR 1.9
• Urine tox neg;
• serum alc 0
• Head CT: bifrontal
hygromas without mass
effect; old parietal
encephalomalacia; basal
ganglia calcification
• CXR: old granulomas
• EEG: diffuse triphasic
waves
What is needed to work up
confusion?
• Structural imaging:
– Brain CT
– Brain MRI
• Infection/hemorrhage/tumor
evaluation:
– Spinal tap
• Seizures/Brain
death/psychogenic/other:
– EEG
Focal status epilepticus
Other
Herpes Encephalitis
Confusion in the Nursing Home
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Dementia with superimposed conditions
Infection: UTI, pneumonia
Medication errors/overdose
End-stage medical diseases: CHF, renal
Poorly managed diabetes
Stroke
Encephalitis/meningitis
Seizure/post-ictal state
Other
38 year old man
• Talking crazy/staggering around
• No recent ETHO though has a history of chronic
liver disease, coagulopathy, hypertension,
seizures, pancreatitis and head trauma
• Medications: ? Phenytoin and nadolol
• Exam: disheveled; 96 F; 179/100 BP; HR 112;
disoriented to place, season and is
confabulating with poor attention and recall;
gaze-evoked nystagmus and incomplete right
eye abduction on right gaze; absent reflexes and
wide-based ataxic gait.
Issues
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Cognitive syndrome: encephalopathy
Diagnosis
Treatment
Where is the pathology
Subtle bilateral
abnormal hyperintense
signal in the
paraventricular region
of the medial thalami
seen on diffusion, flair
and T2. Possible
subtle abnormal signal
of periaqueductal gray
matter seen on flair
and T2.
50 yo with mental
status changes
and abnormal eye
movements.
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Findings
Subtle bilateral abnormal
hyperintense signal in the
paraventricular region of the
medial thalami seen on diffusion,
flair and T2. Possible subtle
abnormal signal of periaqueductal
gray matter seen on flair and T2.
Further history revealed alcohol
abuse.
Diagnosis
Wernicke's encephalopathy
Discussion
MRI of the brain with contrast:
MRI demonstrates acute lesions
of Wernicke-Korsakoff syndrome
in medial thalamic and
periaqueductal regions. This can
be a useful diagnostic procedure
in patients presenting with
suggestive history and stupor or
coma, where ataxia and
ophthalmoplegia are not
detectable.
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Alcohol abuse is the most
common etiology. Prompt
Thiamine administration is
essential and actually was given
to the patient prior the this MRI.
Wernicke encephalopathy is a
medical emergency. Prompt
recognition of the symptom
complex and a high index of
suspicion are crucial to ensure
early treatment. Early treatment
can rapidly reverse the
ophthalmoplegia and improve
ataxia/dysequilibrium and early
mental confusion, as well as
prevent development of the
amnestic state. In advanced
cases, where severe prolonged
deficiency has led to permanent
structural damage, permanent
deficits most often are manifested
as the amnestic state and severe
ataxia.
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Reference: emedicine.
Contributor: Sanders
Acute Alcohol Intoxication
Alcohol Withdrawal
Withdrawal seizures
Delirium tremens
Alcohol hallucinosis
Headache/hangover
Chronic Alcohol Effects
Cerebellar degeneration
Vascular risks
ICH
SDH
Thrombotic
Embolic
Seizures
Cognitive
Spinal cord: B12 def
Neuropathy
Muscular atrophy
Heavy drinkers compared with light
or non drinkers are:
twice as likely to die of heart disease
twice as likely to die of cancer
twelve times as likely to die of cirrhosis
of the liver
three times more likely to die in a car
accident
six times more likely to commit suicide
60 year old man
• Making mistakes; forgetful; unable to
complete his report; no longer interested
• Irritable and defensive; lost his way home
• Guarded/suspicious
• Inattentive with digit span of 5; ¼ recall &
confabulates 2 others
• Occasional paraphasias
• Difficulty with 3 step command; problem
with 3 D cube drawing
Cognitive Syndrome
• Differential diagnoses
• Work-up
– Blood: thyroid/B12/RBC folate +/- VDRL
– Imaging?: CT/MRI
• Management
– Behavioral
– Pharmacological
• Acetylcholinesterase inhibitors
• Glutamate modulators
• Prognosis
This 80-year-old man presented with a gradual decline in functioning. Examination
revealed a marked aphasia and poor visual-spatial ability with an MMSE score of 18/30.
These T1-weighted axial MR images reveal diffuse cortical atrophy with prominent sulci
and enlarged lateral ventricles.
Cognitive Syndrome in the Young
• Differential diagnoses
– Infection: HIV
– Tumor
– Drugs
• Tests
Vignette
• 75 year old with
– Dementia
– Hallucinations
– Episodic alterations in consciousness
– Bradykinesia
• Differential diagnoses
Click here to view movie
Initial Symptoms
Years Later
Dementia
Parkinsonism
AD
Dementia
Dementia
Parkinsonism
Parkinsonism
DLBD
Dementia
PDD
Vignette
56 year-old with 6 month history of rapidly
progressive dementia, myoclonus and
increased tone
SPORADIC CJD
There are three investigations which might provide support for a diagnosis
of sporadic CJD. These are:
The EEG
The CSF 14-3-3 estimation
The MR scan
Transverse FLAIR MRI showing bilateral anterior basal ganglia high signal
This is an EEG tracing showing the characteristic periodic complexes.
78 year old woman
• Confusion; started “talking crazy” and was
stubborn
• Speaks with “meaningless words” and
cannot answer yes/no questions
accurately
• Can mime but cannot follow commands,
name or repeat
• Unable to cooperate with most of exam
Questions
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What has happened to this woman?
The nature of her deficit
What mechanism?
Is she aware of her deficits?
In what settings is anosognosia seen?
Would she be able to read aloud, write or
comprehension related to reading?
• Visual fields would show?
• Discuss the Wernicke-Geschwind model of
language and the anatomical localization
EC = Exner’s Writing
Center
SP = Superior
Parietal
Lobule
A = Angular
Gyrus
B=
Broca’s Area
T=
Pars
Triangularis
H = Henschen’s
Music Center
W = Wernicke’s Area
Definitions
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Aphasia: loss of the ability to use or understand language due to a brain lesion
Mutism: the condition of being unable or unwilling to speak
Fluency: "Production and/or perception of verbal elements of communication
that adhere to the sequence, rhythm, and timing patterns approriate for the
communicative context and expectations of the speaker and/or listener"
(Cross, 1998).
Paraphasia: A person with aphasia might use an incorrect word or unrecognizable
word in place of the target word. This is a paraphasia. Paraphasias can be classified
in 3 types. Phonemic or literal paraphasias are word errors that sound very close to
the intended word (e.g., coke for coat). A verbal or semantic paraphasia occurs when
a word that is related in meaning to the target word is substituted (e.g., plum for
peach). The third type of paraphasia is a neologism - an invented word that is not
recognizable as a word in the speaker's language.
Dysarthria: impaired articulation due to impairment in peripheral nerves or in speech
musculature
Dysprosody: loss of or deficit in the comprehension or production of nonverbal
aspects of language that convey attitudinal, emotional, and similar information to the
listener.
Apraxia: loss of the ability to produce purposeful, skilled movements as the result of
brain damage
Aphasias
Global
Broca
Wernicke
Conduction
Transcortical-M
Transcortical-S
Anomic
Fluency
Comprehension
Naming
Repetition
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73 year old woman
• Sudden onset headache, dizziness with
vomiting; unsteadiness of gait and poor
coordination of the right arm
• What neurological conditions cause
sudden, severe headache?
• What is the localizing value of dizziness,
gait instability, and difficulty controlling the
RUE?
Time Passes
• Patient is no long able to speak clearly; can open eyes
and grunt
Then in ER:
• BP 185/105; afebrile; no nuchal rigidity
• Extensor posturing with stimulation
• No response to voice and no spontaneous limb
movements
• Pupils reactive
• Eyes deviate to left with cold water in left ear without
nystagmus; no response when done to the right ear
• 2 calls
– Test
– Specialist
Questions
• What other parts of the exam is needed
• Eye movements?
• Caloric testing results in
– Normal awake patient
– Comatose patient with intact brainstem
– Brain-dead patient
• Characterize and localize patient’s limb
movements
• What is the diagnosis
• What phone calls were made
• What is the prognosis
Definitions & Underlying Structures
• Coma
• Persistent vegetative state
• Locked-in syndrome
• Brain death
Arousal
• ARAS
Differentiate causes of Coma
• Diffuse processes
– Findings
– Causes
• Structural
– Supratentorial
– Infratentorial
Coma Exam
Findings
Diencephalic
Pupils:
size/light response
Calorics
Corneals
Motor response
Respiration
Midbrain
Pons
Medulla
Management
• How does increased intracranial pressure
(ICP) cause coma?
• What are the treatments for increased ICP
and how do they work?
– Mannitol
– Urea
– Hyperventilation
– Elevate head of bed
– Steroids (for vasogenic only)
LEVELS OF CONSCIOUSNESS:
• Alert normal awake and responsive state
• Lethargic easily aroused with mild stim. Can maintain
arousal.
• Somnolent easily aroused by voice or touch; awakens
and follows commands; req stim to maintain arousal
• Obtunded/Stuporous arousable only with repeated
and painful stim; verbal output is unintelligible or nil;
some purposeful movement to noxious stim
• Comatose no arousal despite vigorous stim, no
purposeful movement- only posturing, brainstem reflexes
often absent
PUPILS:
CN II afferent, CN III efferent. Tests level
of the midbrain as well as autonomic integrity.
Some patterns:
• Hypothalamus:
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Horner’s (miosis, ptosis, and
anhydrosis)
Midbrain:
midpositoin, fixed
Peripheral III:
usually unilateral, more dilated, fixed
Pons:
pin point pupils
Medulla (lat):
Horner’s- preserved response to light
Metabolic:
in general met derangements do not
affect pupils. The major exceptions are
sympathomimetics and anti-cholinergics which dilate,
and opiates which cause pin point pupils.
Other Cranial Nerves
• CORNEALS:
V afferent, VII efferent.
-pons
• OCULOCEPHALIC: requires levels intact from III- VIII
• GAG:
IX, X
-medulla
Motor
Check for asymmetric response as well as movement that localizes to pain,
withdraws from pain, or represents posturing.
Posturing:
Decorticate: extension LE, flexion at elbows/wrists
Better prognosis than decerebrate
Often without concomitant loss neuro-optho reflexes
Usually lesion is above the midbrain
Decerebrate: extension LE, extension/pronation/adduction UE
Often with neuro-ophtho changes
Most commonly lesion at level of midbrain or diencephalon
Glasgow Coma Scale
VERBAL
V5
V4
V3
V2
V1
oriented
confused
inappropriate words
incomprehensible sounds
nil
EYE
E4
E3
E2
E1
spontaneous opening
opens eyes to speech
opens eyes to noxious stim
nil
MOTOR
M6
M5
M4
M3
M2
M1
obeys motor requests
localizes to noxious stim
withdrawal from noxious stim
abnormal flexion response (decorticate posturing)
abnormal extension (decerebrate posturing)
nil