Cognitive Disorders Flashcards

1
Q

What are the 4 things cognitive disorders affect?

What do they result from?

A
  • Cognitive disorders affect
    • Memory
    • Orientation
    • Attention
    • Judgment
  • Result from primary or secondary abnormalities of the CNS
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2
Q

What are the main categories of cognitive disorders?

A
  • Dementia
  • Delirium
  • Amnestic disorders
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3
Q

What is the Mini Mental State Exam (MMSE)?

A
  • Used to assess a patient’s current state of cognitive functioning
  • Can be used as a daily barometer to evaluate interval changes but should NOT be used to make a formal diagnosis
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4
Q

What does the MMSE test? How is it scored?

A
  • Tests
    • Orientation
    • Registration
    • Attention & calculation
    • Recall
    • Language
  • Perfect score: 30
  • Dysfunction: <25
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5
Q

Dementia vs. Delirium

A
  • Dementia: memory impairment
  • Delirium: sensorium impairment
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6
Q

What is Dementia?

What does it affect?

A
  • Impairment of memory & other cognitive functions w/o alteration in the level of consciousness
  • Most forms progressive & irreversible
  • Major cause of disability in the elderly
  • Affects memory, cognition, language skills, behavior & personality
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7
Q

Dementia

What is the incidence?

What is associated with it?

A
  • Incidence increases w/ age
  • 20% of people >80 yo have a severe form of dementia
  • Delusions & hallucinations in 30% of pts
  • Affective symptoms (depression/anxiety) in 40-50% of pts
  • Personality changes common
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8
Q

What are the 3 most common causes of dementia?

A
  • Alzheimer’s disease (50-60%)
  • Vascular dementia (10-20%)
  • Major depression (“pseudodementia”)
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9
Q

What are the 3 categories of differential diagnoses for dementia?

A
  • Psychiatric
  • Organic
  • Drugs
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10
Q

What is the psychiatric differential for dementia?

A
  • Depression (pseudodementia)
  • Delirium
  • Schizophrenia
  • Malingering
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11
Q

What is the organic differential for dementia?

A
  • Structural
    • Benign forgetfulness of normal aging, Parkinson’s disease, Huntington’s disease, Down’s syndrome, head trauam, brain tumor, normal pressure hydrocephalus, multiple sclerosis, subdural hematoma
  • Metabolic
    • Hypothyroidism, hypoxia, malnutrition (B12, folate, thiamine deficiency), Wilson’s disease, lead toxicity
  • Infectious
    • Lyme disease, HIV dementia, Creutzfeldt-Jakob disease, neurosyphilis, meningitis, encephalitis
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12
Q

What is the minimum workup to exclude reversible causes of dementia?

A
  • CBC
  • Electrolytes
  • TFTs
  • VDRL/RPR
  • B12 & folate levels
  • Brain CT or MRI
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13
Q

What is the drug differential for dementia?

A
  • Alcohol (chronic & acute)
  • Phenothiazines
  • Anticholinergics
  • Sedatives
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14
Q

Differential & diagnostic test for scenario

Dementia with stepwise increase in severity + focal neurologic signs

A

Multi-infarct dementia

CT/MRI

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15
Q

Differential & diagnostic test for scenario

Dementia + cogwheel rigidity + resting tremor

A

Lewy body dementia, Parkinson’s disease

Clinical

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16
Q

Differential & diagnostic test for scenario

Dementia + ataxia + urinary incontinence + dilated cerebral ventricles

A

Normal pressure hydrocephalus

CT/MRI

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17
Q

Differential & diagnostic test for scenario

Dementia + obesity + coarse hair + constipation + cold intolerance

A

Hypothyroidism

T4, TSH

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18
Q

Differential & diagnostic test for scenario

Dementia + diminished position and vibration sensation + megaloblasts on CBC

A

Vitamin B12 deficiency

Serum B12

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19
Q

Differential & diagnostic test for scenario

Dementia + tremor + abnormal LFTs + Kayser-Fleischer rings

A

Wilson’s disease

Ceruloplasmin

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20
Q

Differential & diagnostic test for scenario

Dementia + diminished position & vibration sensation + Argyll-Robertson Pupils (Accommodation Response Present, response to light absent)

A

Neurosyphilis

CSF fluorescent treponemal antibody absorption test (CSF FTA-ABS) or CSF VDRL

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21
Q

What is the hallmark of delirium?

What can it be caused by?

What is the prognosis?

A
  • Hallmark of delirium
    • Waxing/waning of consciousness
  • Can be caused by virtually any medical disorder
  • High mortality rate if untreated
  • Can last days to weeks, can be chornic
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22
Q

What is the DSM-IV criteria for delirium?

A

2 types of delirium

  • Quiet
    • Pt may seem depressed or exhibit symptoms similar to failure to thrive
    • MMSE must be done to distinguish from depression and other diagnostic criteria
  • Agitated
    • Obvious pulling out lines; may hallucinate
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23
Q

How is delirium treated?

A
  • Rule out life-threatening causes
  • Treat reversible causes
    • Example: hypothyroidism, electrolyte imbalance, UTIs
  • Antipsychotics first line
    • Quetiapine (Seroquel)
    • Haloperidol PO/IM - don’t use IV unless on cardiac monitor b/c can cause TdP
  • Positive/negative use of benzos
    • Paradoxical disinhibition
    • Respiratory depression
    • Increased risk for falls
  • 1:1 nursing for safety
  • Frequently reorient patient
  • Avoid napping
  • Keep lights on, shades open during the day
  • In your orders, write “hold for sedation” after medication order so medications are not given when already sedated & calm
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24
Q

What is the pneumonic for the delirium differential?

A

AEIOU TIPS

  • Alcohol
  • Electrolytes
  • Iatrogenic
    • Anticholinergics, benzos, anti-epileptics, BP meds, insulin, hypoglycemics, narcotics, steroids, H2 receptor blockers, NSAIDs, antibiotics, antiparkinsonians
  • Oxygen hypoxia
    • Bleeding, central venous, pulmonary
  • Uremia/hepatic encephalopathy
  • Trauma
  • Infection
  • Poisons
  • Seizures (post-ictal)
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25
Q

Delirium vs. Dementia

  • Definition
  • Onset
  • Duration
  • Orientation
  • Memory
  • Hallucinations
  • Symptoms
  • Reversibility
  • Awareness
  • EEG
A
  • Delirium
    • Clouding of consciousness
    • Acute onset
    • 3 days - 2 wks
    • Orientation impaired
    • Immediate/recent memory impaired
    • Visual hallucinations common
    • Symptoms fluctuate, worse at night
    • Usually reversible
    • Awareness reduced
    • EEG changes (fast waves or generalized slowing)
  • Dementia
    • Loss of memory/intellectual ability
    • Insidious onset
    • Lasts months to yrs
    • Orientation often impaired
    • Recent & remote memory impaired
    • Hallucinations less common
    • Symptoms stable throughout the day
    • 15% reversible
    • Awareness clear
    • No EEG changes
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26
Q

_______ is the most common dementia (80%)

A

Alzheimer’s disease

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27
Q

Alzheimer’s Disease

  • Incidence
  • Women vs. Men
  • Average life expectancy
  • ___% of pts have a family hx
A
  • 5% of all people >65 yo
  • 15-25% of all people >85 yo
  • Women >> men
  • Average life expectancy: 8 yrs after diagnosis
  • 40% of pts have a family hx
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28
Q

What are the clinical manifestations of Alzheimer’s disease?

A
  • Gradual progressive decline of cognitive functions (memory & language)
  • Personality changes
  • Mood swings
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29
Q

What is the DSM-IV criteria for Alzheimer’s Disease?

A

Memory impairment plus at least 1 of the following:

  • Aphasia
    • Disorder of language affecting speech & understanding
  • Apraxia
    • Inability to perform purposeful movements
  • Agnosia
    • Inability to interpret sensations correctly (visual agnosia: can’t recognize previously known object)
  • Diminished executive functioning
    • Problems w/ planning, organizing, abstracting

*personality/mood changes: depression, anxiety, anger, suspiciousness are common; psychotic symptoms such as paranoia are common

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30
Q

Alzheimer’s patients have decreased levels of _____ and _____.

A
  • Acetylcholine
    • Loss of noradrenergic neurons in the locus ceruleus of the brainstem
  • Norepinephrine
    • Preferential loss of cholinergic neurons in the basal nucleus of Meynert of the midbrain
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31
Q

What is the gross pathology of the brain of an Alzheimer’s patient?

A

diffuse atrophy

enlarged ventricles

flattened sulci

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32
Q

What is the microscopic pathology of the brain of an Alzheimer’s patient?

A
  • Senile plaques composed of amyloid protein
  • Neurofibrillary tangles derived from Tau proteins
  • Neuronal & synaptic loss
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33
Q

What is the only way to definitively diagnose Alzheimer’s disease?

A

pathological examination of the brain at autopsy

34
Q

How is Alzheimer’s disease treated?

A
  • No cure or truly effective treatment
  • Physical & emotional support, proper nutrition, exercise & supervision
  • NMDA receptor antagonists: memantine
  • Cholinesterase inhibitors to help slow progression
    • Tacrine (Cognex)
    • Donepezil (Aricept)
    • Rivastigmine (Exelon)
  • Treatment of symptoms as necessary
    • Low-dose, short-acting benzodiazepines for anxiety
    • Low-dose antipsychotics for agitation/psychosis (quetiapine)
    • Antidepressants for depression (if pt fulfills criteria for major depression)
35
Q

What is vascular dementia?

What is the process of function loss?

A
  • Caused by microvascular disease in the brain that produces multiple small infarcts
  • Substantial infarct burden must accumulate before dementia develops
  • Classically, patients have a stepwise loss of function
36
Q

What are the clinical manifestations of Vascular Dementia?

A
  • Memory impairment and at least 1 of the following must be present:
    • Aphasia
    • Apraxia
    • Agnosia
    • Diminished executive functioning
  • Personality changes: depression, anger, suspiciousness are common; psychotic symptoms such as paranoia are common
37
Q

What is mild cognitive impairment characterized by?

A

normal daily function but abnormal memory for age

most progress to Alzheimer’s

38
Q

Vascular dementia vs. Alzheimer’s

A
  • Since vascular dementia is caused by small brain infarcts, pts have focal neurological symptoms
    • Hyperreflexia, paresthesias
  • VD onset more abrupt than Alzheimer’s
  • Greater preservation of personality in VD
  • Can reduce risk by modifying risk factors
    • Smoking, HTN, DM
39
Q

How is vascular dementia diagnosed?

How is it treated?

A
  • MRI
  • No cure or truly effective treatment
  • Physical & emotional support, proper nutrition, exercise, supervision
  • Treatment of symptoms as necessary
40
Q

What are the clinical manifestations of Pick’s Disease/Frontotemporal Dementia (FTD)?

A
  • Rare cause of slowly progressing dementia
  • Hallmarks: aphasia, apraxia, agnosia
  • Difficult to distinguish from Alzheimer’s clinically, but personality & behavioral changes are more prominent early in the disease
41
Q

What is the pathology of FTD?

A
  • Atrophy of frontotemporal lobes
  • Pick bodies
    • Intraneuronal inclusion bodies
    • Not necessary for diagnosis
42
Q

How is FTD treated?

A
  • No effective treatment
  • Physical, emotional & nutritional support
  • Treat emotional/behavioral symptoms as needed
43
Q

What is Huntington’s Disease?

A
  • Autosomal dominant disorder
  • Results in progressively disabling cognitive, physical & psychological functioning
  • Ultimately results in death after approx 15 yrs
44
Q

What are the clinical manifestations of Huntington’s Disease?

A
  • Onset: 35-50 yrs of age
  • Hallmarks
    • Progressive dementia
    • Bizarre choreiform movements (dancelike flailing of arms & legs)
    • Muscular hypertonicity
    • Depression & psychosis very common
45
Q

What is the pathology of Huntington’s disease?

How is it diagnosed?

What is the treatment?

A
  • Trinucleotide repeat on short arm of chr 4
  • Primarily affects basal ganglia
  • MRI shows caudate atrophy (sometimes cortical atrophy)
  • Genetic testing & MRI are diagnostic
  • No effective treatment, supportive only
46
Q

Cortical vs. Subcortical dementias

A
  • Cortical dementias: decline in intellectual functioning
    • Alzheimer’s disease
    • Pick’s disease
    • CJD
  • Subcortical dementias: more prominent affective & movement symptoms
    • Huntington’s disease
    • Parkinson’s disease
    • NPH
    • Multi-infarct dementia
47
Q

What is Parkinson’s disease?

A
  • Progressive disease w/ prominent neuromal loss in substantia nigra, which provides dopamine to the basal ganglia, causing physical & cognitive impairment
  • 30% of pts develop dementia
48
Q

What are the clinical manifestations of Parkinson’s disease?

A
  • Bradykinesia
  • Cogwheel rigidity
  • Resting tremor - “pill-rolling” tremor most common
  • Masklike facial expression
  • Shuffling gait
  • Dysarthria (abnormal speech)
  • 50% of pts suffer from depression
  • Dementia symptoms resemble Alzheimer’s
49
Q

What is the etiology of Parkinson’s disease?

A
  • Idiopathic (most common)
  • Traumatic
  • Drug or toxin-induced
  • Encephalitic
  • Familial (rare)
50
Q

What is the pathology & pathophysiology of Parkinson’s disease?

A
  • Loss of cells in the substantia nigra of the basal ganglia leads to decrease in dopamine & loss of the dopaminergic tracts
51
Q

How is Parkinson’s disease treated pharmacologically?

A
  • Levodopa
    • Degraded to dopamine by dopadecarboxylase
  • Carbidopa
    • Peripheral dopadecarboxylase inhibitor prevents levodopa from being converted to dopamine before it reaches the brain
  • Amantadine
    • Mechanism unknown
  • Anticholinergics
    • Help relieve tremor
  • Dopamine agonists
    • Bromocriptine, etc.
  • Monoamine oxidase B inhibitors (selegiline)
    • Inhibit breakdown of dopamine
52
Q

How do levodopa & carbidopa interact with the BBB?

A
  • Levodopa
    • Crosses the BBB
    • Free to convert to dopamine once it corsses the BBB
  • Carbidopa
    • Does NOT cross the BBB
    • Prevents conversion of levodopa to dopamine in the periphery
53
Q

How is Parkinson’s disease treated surgically?

A

Thalamotomy or pallidotomy may be performed if no longer responsive to pharmacotherapy

54
Q

What is Creutzfeldt-Jakob Disease (CJD)?

A
  • A rapidly progressive, degenerative disease of the CNS caused by a prion
  • Inherited, sporadic or acquired
  • Small percentage of patients have become infected through corneal transplants
55
Q

What are the clinical manifestations of CJD?

How quickly do these symptoms occur?

A
  • Rapidly progressive dementia 6-12 mo after onset of symptoms
  • >90% of patients have myoclonus (sudden spasms of muscles)
  • Extrapyramidal signs, ataxia, LMN signs
  • Long latency period btwn exposure & disease onset
56
Q

What are prions?

A
  • Proteinaceous infectious particles that are normally expressed by healthy neurons of the brain
  • Accumulations of abnormal forms of prions are responsible for disease
57
Q

What are other examples of prion diseases?

A
  • Kuru
  • Gerstmann-Straussler syndrome
  • Fatal familial insomnia
  • Bovine spongiform encephalopathy
    • “Mad cow disease”
58
Q

What is the gross pathology of CJD?

A
  • Spongiform changes of cerebral cortex
  • Neuronal loss
  • Hypertrophy of glial cells
59
Q

How is CJD diagnosed? (definitive vs. probable)

A
  • Definitive - pathological demonstration of spongiform changes of brain tissue
  • Probable - presence of both rapidly progressive dementia & periodic generalized sharp waves on EEG plus at least 2 of the following:
    • Myoclonus
    • Cortical blindness
    • Ataxia, pyramidal signs, extrapyramidal signs
    • Muscle atrophy
    • Mutism
60
Q

What is the treatment & course of CJD?

A
  • No treatment
  • Relentless course
  • Progressing to death usually w/i a year
61
Q

What is Normal Pressure Hydrocephalus?

What is the etiology?

A
  • Reversible cause of dementia
  • Enlarged ventricles w/ increased CSF pressure
  • Etiology
    • Idiopathic
    • Secondary to obstruction of CSF reabsorption sites due to trauma, infection, hemorrhage
62
Q

What is the clinical triad of NPH?

A
  • Gait disturbance (often appears first)
  • Urinary incontinence
  • Dementia (mild, insidious onset)
63
Q

How is NPH treated?

A

relieve increased pressure w/ shunt

of the clinical triad, dementia least likely to improve

64
Q

What is delirium?

A

acute disorder of cognition related to impairment of cerebral metabolism

65
Q

Unlike demented patients, delirious patients have…..

A
  • Rapid onset of symptoms
  • Periods of altered levels of consciousness
  • Potential reversal of symptoms w/ treatment of the underlying cause
66
Q

How do delirious patients appear clinically?

A
  • Appear confused
  • Have a fluctuating course w/ lucid intervals
  • May be either stuporous or agitated
  • Perceptual disturbances (hallucinations) common
  • Pts often anxious, incoherent, unable to sleep normally
67
Q

What are the common causes of delirium?

A
  • CNS injury or disease
  • Systemic illness
  • Drug abuse/withdrawal
  • Hypoxia
68
Q

What are some additional causes of delirium?

A
  • Fever
  • Sensory deprivation
  • Medications
    • Anticholinergics
    • Steroids
    • Antipsychotics
    • Antihypertensives
    • Insulin
  • Post-op
  • Electrolyte imbalances
69
Q

What is the differential diagnosis for delirium?

A
  • Dementia
  • Fluent aphasia (Wernicke’s)
  • Acute amnestic syndrome
  • Psychosis
  • Depression
  • Malingering
70
Q

What is the acronym for causes of delirium?

A

I’M DELIRIOUS

  • Impaired delivery (of brain substrates, such as vascular insufficiency due to stroke)
  • Metabolic
  • Drugs
  • Endocrinopathy
  • Liver disease
  • Infrastructure (structural disease of cortical neurons)
  • Renal failure
  • Infection
  • Oxygen
  • Urinary tract infection
  • Sensory deprivation
71
Q

How is delirium treated?

A
  • First & foremost: treat the underlying cause!
  • Provide physical & sensory support
  • Treat drug withdrawal
  • Treat symptoms of psychosis (low-dose antipsychotic) and insomnia (sedative-hypnotic)
72
Q

What is the diagnosis & testing for this scenario?

Delirium + hemiparesis or other focal neurological signs & symptoms

A

Cerebrovascular accident (CVA) or mass lesion

Brain CT/MRI

73
Q

What is the diagnosis & testing for this scenario?

Delirium + elevated BP + papilledema

A

Hypertensive encephalopathy

Brain CT/MRI

74
Q

What is the diagnosis & testing for this scenario?

Delirium + dilated pupils + tachycardia

A

Drug intoxication

Urine toxicology screen

75
Q

What is the diagnosis & testing for this scenario?

Delirium + fever + nuchal rigidity + photophobia

A

Meningitis

Lumbar puncture

76
Q

What is the diagnosis & testing for this scenario?

Delirium + tachycardia + tremor + thyromegaly

A

Thyrotoxicosis

T4, TSH

77
Q

What is the acronym for treatment of delirium?

A

FEUD

  • Fluids/Nutrition
  • Environment
  • Underlying cause
  • Drug withdrawal
78
Q

Avoid using ___________ in delirious patients, as they will often exacerbate delirium.

A

benzodiazepines

79
Q

What are amnestic disorders?

A
  • Impairment of memory w/o other cognitive problems or altered consciousness
  • Always occur secondary to an underlying medical condition
80
Q

What are causes of psychiatric disorders?

A
  • Hypoglycemia
  • Systemic illness (like thiamine deficiency)
  • Hypoxia
  • Head trauma
  • Brain tumor
  • CVS
  • Seizures
  • Multiple sclerosis
  • Herpes simplex encephalitis
  • Substance use (alcohol, benzodiazepines, medications)
81
Q

What is the course & prognosis of amnestic disorders?

A
  • Variable depending on underlying medical cuase
  • Usually transient w/ full recovery
    • Seizures, medication-induced
  • Possibly permanent
    • Hypoxia, head trauma, herpes simplex encephalitis, CVA
82
Q

How are amnestic disorders treated?

A
  • Treatment of underlying cause
  • Supportive psychotherapy if needed (to help patients accept their limits & understand their course of recovery)