Week 6 (Test 2) Flashcards
What causes prion formation?
•Misfolded protein (PrPSc)
How do you diagnose prion diseases?
pathology
Later in the lecture he says:
•Initial diagnosis made on clinical grounds
•Western blot for PrPSc in a tonsil biopsy
•Definitive diagnosis by brain biopsy
What about this slide confirms a prion disease?

The white holes which represent the spongiform changes
What are some of the clinical manifestations of
Creutzfeldt-Jakob disease (CJD)?
•Abnormal gait and vision
•Headache, dizziness, paresthesias
•Loss of memory, speech abnormalities, anxiety, and depression
•Hyperreflexia and spasticity
•
•Vegetative state within 1 year
•Viral encephalitis characterized by hydrophobia
rabies
Describe the composition of the rabies virus.
bullet shaped virion; singe strand negative sense RNA
Describe the clinical manifestations of rabies.
•Furious versus paralytic
–Agitation, hyperactivity, fluctuating consciousness, and bizarre behavior
–Sore throat, hypersalivation, and hydrophobia
–Flaccid paralysis
A brain biopsy with this pathology would indicate what disease?

rabies
How do you make a diagnosis of rabies?
used to only be able to diagnose with a brain biopsy but now you can use a direct fluorescent antibody (DFA) stain on tissue from the nape of the neck; this looks for the presence of rabies viral antigen <!--EndFragment-->
How do we treat rabies?
- Rabies immunoglobulin
- Post-exposure vaccination
Describe clostridium bacteria.
- Large Gram positive bacilli
- Heat-resistant spores
- Strictly Anaerobic
- Toxins cause disease
causes gas gangrene and food poisoning
Clostridium perfringens
causes Antibiotic-associated pseudomembranous colitis
*clostridium difficile *
trismus =
lock jaw (tetanus)
risus sardonicus =
patient looks like they are grinning (tetanus)
What actually causes the symptoms of tetanus?
the release of the toxin tetanospasmin from clostridium tetani spores
What’s the mechanism of action of tetanospasmin?
it is a Plasmid-encoded neurotoxin (A-B toxin) which Binds to motor neurons and then undergoes Retrograde vesicular transmission;
It Blocks inhibitory neurotransmitter (GABA) release
which leads to Unregulated excitatory synaptic activity and Spastic paralysis
When do you need a tetanus vaccine booster?
Tetanus booster every 10 yrs or 5 yrs with puncture wound
Which type of botulism is most prevalent?
infant botulism by far;
•Colonization of intestinal tract in 5-20 wk old
•Older children & adults are resistant
What should you do with all botulism and tetanus cases?
report them to the CDC
How many serotypes of clostridium botulinum are there?
7; types A, B, and E are the most common
How would a patient with botulism present?
- Dizziness, nausea
- Cranial nerve issues - double or blurred vision
- *-Progressive flaccid paralysis**
How does botulin toxin cause flaccid paralysis?
it prevents the release of ACh from the neuromuscular junction
How do you diagnose botulism?
symptoms and history, electromyographic studies, and
isolation of C. botulinum or toxin from stool or food
How do you treat botulism?
-Heptavalent (A-G) equine antitoxin (HBAT) [need to test them for sensitivity to horse serum first]
-Supportive therapy
(Mortality is 10%)
What’s the main way kiddos get infant botulism?
<12 month old given honey
How do you treat infant botulism?
-BabyBIG (BIG-IV) [human serum]
- Supportive therapy (ventilation)
[Mortality is 1-2%]
Virus families that include arboviruses:
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•Togaviruses (Alphaviruses) - Equine encephalitis viruses (but not rubella virus)
•Flaviviruses - 1West Nile virus, 2Japanese encephalitis virus, Yellow fever virus, 3Dengue virus
•Bunyaviruses - California encephalitis virus (but not hantavirus)
•Reoviruses - Colorado tick fever virus (but not rotavirus)
<!--EndFragment-->[mnemonic: Transmitted From Bites]
What are the common arbovirus characteristics?
- All have an RNA genome [ Trigger interferon!]
- Enveloped viruses (Colorado tick fever virus, a reovirus, is an exception) –> makes them Labile!
- All replicate in the cytoplasm of infected host cells.
Describe the Togavirus family.
- Enveloped viruses
- Icosahedral capsid
- Linear, plus-sense, single-strand RNA genome
- Replicate in the cytoplasm of host cell
What’s a unique feature of the Togavirus family?
•Temporally regulate expression of non-structural and structural proteins through use of subgenomic RNA.
Describe the Flavivirus family
- Enveloped viruses
- Icosahedral capsid
- Linear, plus-sense, single-strand RNA genome
- Replicate in the cytoplasm of host cell
What’s unique about the Flavivirus family?
•Entire genome translated as polyprotein, processed to structural and non-structural proteins
Describe the Bunyavirus family.
- Enveloped viruses
- **Helical capsid
- Single-strand, segmented, negative-sense RNA genome**
What’s unique about the Bunyavirus family?
•Capable of transovarial transmission
–> arthropods that have this can give it to their offspring
Arboviruses typically have a specific ____.
geography;
The presence of the natural host and vector are critical determinants of geography!
Who is most at risk for virus induced encephalitis and why?
children and elderly becuase immuno-competence is important for limiting disease
What’s the gold standard for diagnosing viral encephalitis?
•RT-PCR for specific viral genome
What should you do as soon as you suspect viral encephalitis?
start with acyclovir
when should you consider a diagnosis of Dengue virus?
•Consider Dengue in anyone with a fever who has visited the tropics. Other warning signs include: nausea/vomiting, myalgia, rash, low WBC count, and positive tourniquet test.
Why is Dengue fever referred to as “break-bone fever”?
•Infects dendritic cells and macrophages, elicits vigorous innate immune response, thus the bone-breaking feeling.
Who is at the greatest risk of severe dengue virus disease?
healthy children (have a competent immune system)
Enhanced Dengue virus disease may occur by antibody-dependent enhancement (ADE). What is this?
•non-neutralizing, pre-existing antibodies may enhance viral uptake into phagocytic cells; Instead of neutralizing them, they help the virus get into cell types it wants to infect
What should be on the differential diagnosis for Dengue?
Chikungunya
•Destruction results in apraxia, aphasia & neglect
supramarginal gyrus (area 40)
•Destruction results in contralateral loss of tactile discrimination (astereognosis) & inability to recognize forms and body position
superior parietal
•inability to see more than one object at a time (usually bilateral damage)
Simultanagnosia
•deficit in reaching under visual guidance that cannot be explained by motor, somatosensory, visual field deficits (lesion is often unilateral, affects the contralateral hemifield and alters visuo-spatial integration)
optic ataxia
Visual agnosia is more commonly associated with damage to the _______.
inferior temporal lobe and adjacent visual cortex
List the Components of Balint’s Syndrome
- Psychic paralysis of gaze (difficulty initiating eye movements) with haphazard scanning.
- Optic ataxia.
- Simultanagnosia.
- Caused by bilateral lesions in parieto-occipital cortex due to stroke, trauma or degenerative disease.
Destruction of Brodmann’s area 17 results in what?
•Destruction results in visual field defects
Destruction of Brodmann’s area 18 & 19 results in what?
•Destruction results in visual hallucinations, agnosia, and alexia
- A form of cortical blindness in which the patient denies visual impairment.
- Confabulation is common.
Anton’s syndrome
What causes Anton’s syndrome?
Typically caused by bilateral damage to the occipital lobe extending from primary visual cortex to association cortex.
What are the 4 symptoms of Gerstmann syndrome?
Acalculia= calculation deficit for simple math problems
Agraphia= can’t write
Agnosia= inability to process sensory information
left-right confusion
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•a deficit in visual object recognition where elementary visual capacities are intact (acuity, visual fields).
visual agnosia
•Elementary shape perception is intact so the patient can draw objects but not associate the visual object with its meaning. They can not name objects or show recognition by pointing unless another sensory modality, like sound or smell, is used to access object knowledge.
associative visual agnosia
what causes associative visual agnosia?
localized damage often to inferior temporal lobes and adjacent occipital cortex
Left hemisphere destruction of Area 22 results in ____.
Wernicke’s aphasia
right hemisphere destruction of area 22 results in ____.
sensory dysprosodia
(no pitch or timing control–> can’t control the way the words are coming out of their mouths)
▫Refusal to accept reality or fact; acting as if a painful event, thought or feeling did not exist
▫“I like to drink but it isn’t a problem” (in person with cirrhosis, GI bleed)
denial (immature)
▫A return to an earlier period of development
▫Acting child-like in periods of extreme stress
▫Sleeping with transitional object (teddy bear, blankie)
regression (immature)
▫Expressing an unconscious wish through action to avoid becoming consciously aware of it
▫Punching walls when you want to kill your partner
acting out (immature)
▫Attributing your thoughts or feelings to someone else because they are uncomfortable
▫“You’re angry with me, I can tell” (ah, no, I’m thinking about pizza)
Projection (immature)
▫Lesser form of dissociation, wherein parts of oneself are separated from awareness of other parts and behaving as if one had separate sets of values
▫Honest man cheats on taxes; philanderers
compartmentalization (immature)
▫Temporary but drastic modification of a person’s character or sense of identity to avoid distress
▫Often seen in people with h/o severe trauma
dissociation (immature)
▫Converting of unwanted or dangerous thoughts, feelings or impulses into their opposites
▫You want to play with your feces, but instead you wash compulsively (an actual psychodynamic “explanation” of OCD that was obviously wrong)
▫Acting like you hate someone you secretly love (to avoid being hurt)
reaction formation (immature)
▫Division or polarization of beliefs, actions, objects, or persons into good and bad by focusing selectively on their positive or negative attributes
▫“Those other doctors were mean but I can see you really care” (red flag!)
splitting (immature)
Splitting is tied to which personality disorder?
borderline personality disorder
▫Processes whereby the idealized aspects of the self are preserved, and its limitations denied
▫People who talk about their college degree (or have t-shirts, bumper stickers, etc.), jobs, possessions, etc., may be trying to cover up feelings of inadequacy
narcissism (immature)
▫Internalizing the qualities of another person
▫“Identification with the aggressor” (kids in concentration camps boot stepping like Nazi guards), or abused person believing they deserve abuse and abusers as mates
▫Can be healthy, such as taking on best aspects of your parents
introjection (immature)
▫Dealing with emotional conflict or internal or external stressors by excessive daydreaming as a substitute for human relationships or more effective action
▫Having a fantasy of being important and loved despite no reality to either
schizoid fantasy
▫A tendency to experience and communicate psychological distress in the form of physical symptoms and to seek medical help for them
▫Psychological trauma from childhood abuse is too difficult to think about so distress is channeled to more “appropriate” and less difficult to discuss sxs
▫“I’ve got a headache” becomes way of escaping difficult conversations
•Somatization/ hypochondriasis
▫Redirecting of thoughts feelings and impulses directed at one person or object, but taken out upon another person or object
▫Mad at the boss, a person goes home and kicks the dog
▫Taken out on a person, not a wall
displacement (immature)
▫Unconscious blocking of unacceptable thoughts, feelings and impulses
▫Unconscious /less mature version of suppression
▫The person may still be aware of the feelings associated with the repressed issue, but will not know where the feelings come from
▫Extreme anxiety when around someone who looks like a person you hate/fear without realizing
repression (immature)
▫Overemphasis on thinking when confronted with an unacceptable impulse, situation or behavior without employing any emotions
▫Dealing with stress by excessively using abstract thinking and generalizations to avoid or minimize unpleasant feelings
intellectualization (immature)
▫Offer rational explanations to justify thoughts ideas, feelings and behaviors that may otherwise be unacceptable
▫“I would like to spend more time with my daughter, but I am so busy”
▫“It makes sense I make this much money because I worked hard for it”
rationalization (immature)
▫Conscious postponing attention to a conscious impulse or conflict
▫“I can’t worry about this now”
▫Repression is similar but unconscious
supression (mature)
▫Achieving impulse gratification and retention of goals but altering a socially objectionable aim or object to a socially acceptable one
▫Aggressive person with authority figure issues becomes a police officer
sublimation (mature)
▫Using constructive and instinctually gratifying service to others to undergo a vicarious experience
▫Guilty feelings alleviated by unsolicited generosity towards others
▫Charitable giving?
altruism (mature)
▫Using comedy to overtly express feelings/thoughts without personal discomfort and without producing an unpleasant effect on others
▫Making light of difficult situations: “If I don’t pass Step I, I can always go flip burgers!”
humor (mature)
▫Psychologically counterbalancing perceived weaknesses by emphasizing strength in other arenas
▫“I’m not great at NBME exams but I’m better with people than some other students”
compensation (mature)
Describe a strategy often helpful with people with significant Narcissistic traits.
go along with it. play into their narcissism
Name the type of psychotherapy most often cited as evidence-based treatment for Borderline Personality Disorder.
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Dialectical Behavioral Therapy (DBT)
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Which personality disorders fall into cluster A?
WEIRD –>
Paranoid
Schizoid
Schizotypal
Which personality disorders fall into cluster B?
WILD –>
Antisocial
Borderline
Histrionic
Narcissistic
Which personality disorders fall into cluster C?
WORRIED–>
Avoidant
Dependent
Obsessive-compulsive
- Distrust and suspiciousness
- Others’ motives interpreted as malevolent
- Reluctant to confide in others due to fear that it would be maliciously used against them
- Holds grudges
- Don’t take responsibility for their own feelings
- Hostile, irritable, angry
- Read negative meanings into neutral remarks
- Perceive attacks on their character and is then quick to counteract
- Suspect infidelity in partner
- Litigious
paranoid PD
Detachment from social relationships, restricted emotional expressions in interpersonal settings
§Seem cold, flattened.
Doesn’t desire close relationships
§Including family and sexual relationships
§Lack close friends/confidants
Solitary activities
Indifferent to praise or criticism
schizoid PD
Social deficits, reduced capacity for close relationships
§Lack close friends
Cognitive or perceptual distortions and eccentricities in behavior
Magical thinking, odd beliefs, paranoid thinking
Bodily illusions
Peculiar appearance, inappropriate affect
Odd thinking/speech
schizotypal PD
Disregard for and violation of others’ rights
Fails to conform to the law
Deceitful, manipulative
§For personal profit or pleasure
Impulsive
Lack remorse
§Indifferent to or rationalize hurting others
antisocial PD
Unstable/chaotic relationships, self-image, and affects
§Alternate between idealizing and devaluing others
§I Hate You, Don’t Leave Me book
Marked impulsivity
§Potentially self-damaging areas (spending, sex, substance use)
§Unpredictable
Frantic efforts to avoid abandonment
§Many have a history of abandonment/abuse
borderline PD
Excessive emotionality and attention seeking
§Rapidly shifting and shallow emotions
Uncomfortable when not the center of attention
§Theatrics, dramatic
Inappropriate sexually seductive and provocative behavior
§Uses physical appearance to draw attention to self
Speech is excessively impressionistic and lacking in detail
Suggestible, easily influenced by others
§Considers relationships to be more intimate than they are
histrionic PD
Grandiose sense of self-importance
§Preoccupied with fantasies of unlimited success, power, brilliance, beauty
§Believes they’re “special” and can only relate to other high-status people
Requires excessive admiration
§Entitled
Lack empathy
§Exploit others to achieve their own needs
§Envious of others
Arrogant, haughty
narcissistic PD
Social inhibition, feelings of inadequacy, hypersensitive to negative evaluation
Fears criticism, disapproval, or rejection
§Unwilling to get involved unless certain of being liked
Preoccupied with being criticized or rejected in social situations
Views self as unappealing or inferior
Reluctant to take personal risks or engage in new activities because they may prove embarrassing
avoidant PD
Need to be taken care of that leads to submissive and clinging behavior and fears of separation
Difficulty making decisions
§Needs others to assume responsibility
Difficulty expressing disagreement
Excessive (even unpleasant) lengths to obtain nurturance and support
Uncomfortable or helpless when alone
Urgently seeks another relationship as a source of care and support when a close relationship ends
§Fears being left to take care of themselves
dependent PD
Preoccupied with orderliness to the point where the major point of the activity is lost
§Rules, lists, order, organization, schedules
Perfectionism that interferes with task completion
Excessively devoted to work at the expense of leisure activities
Overconscientious, scrupulous, and inflexible about morality, ethics, or values
Rigid and stubborn
Preoccupied with mental/interpersonal control
§Unable to delegate tasks
obsessive compulsive PD
what causes Gerstmann syndrome?
destruction of the inferior parietal lobe