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96 Cards in this Set

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  • Back
What are the cortical dementias?
1. Alzheimer's Disease
2. Pick's Disease
3. Diffuse Lewy Body Dementia
4. Creutzfeldt-Jacob's Disease
What are the subcortical dementias?
1. Huntington's Chorea
2. Parkinson's Disease
3. Progressive Supranuclear Palsy (PSP)
4. Wilson's Disease
5. Hallerverorden-Spatz disease
6. Syndenham's Chorea
7. Subacute Sclerosing Panencephalitis (SSPE)
8. Shy Drager Syndrome
9. Olivopontocerebellar Atrophy
10. Striato-Nigral Degeneration
What is the second most prevalent dementia?
Multi-infacrt dementia
Dementia is:
a) A progressive illness noted by plaques & tangles;

b) Often accompanied with cogwheel rigidity;

c) A set of symptoms which may be associated with many disorders;

d) Synonymous with declines in intellect
c) A set of symptoms which may be associated with many disorders
What is the essential feature of dementia?
Memory impairment (required) AND at least one other kind of cognitive deficit (e.g., aphasia, apraxia, agnosia, executive function)
Dementia always has an underlying organic cause.

TRUE or FALSE
TRUE
Estimates of the prevalence of dementia among the elderly have ranged from ____% to ___% of persons over 65 for severe demential, and ___% to ___% for those with milder dementia.
1.3% to 6.2%

2.6% to 15.4%
Which type of dementia affects at least half of the dementia cases?
Alzheimer's Disease (affecting 5-6% of people above 65).
Can a 4-year-old develop dementia?
Yes.

"If a 4 year old developed a chronic neurologic disorder that interfered with previously acquired functions so as to significantly lower IQ or adaptive functioning, he would be considered to have both dementia and retardation."
What is the neuroanatomical characteristic of Dementia of the Alzheimer's Type?
A loss of cholinergic neurons in the nucleus basalis of Meynert leading to development of senile plaques and neurofibrillary tangles
What is the age of onset for Dementia of the Alzheimer's Type?
Between 40 and 90; most common after 65
What are the risk factors for Dementia of the Alzheimer's Type?
1. Female
2. Family history of Down Syndrome
3. TBI
4. History of thyroid disease
The primary motor, somatosensory, visual, and auditory cortices are relatively spared in Dementia of the Alzheimer's Type.

TRUE or FALSE
TRUE
Which of the following areas is affected the most in Dementia of the Alzheimer's Type?

a. Frontal lobes
b. Medial temporal lobes
c. Basal temporal cortex
b. Medial temporal lobes

(See pg. 873 of Blumenfeld)
What neuroanatomical changes are seen in Dementia of the Alzheimer's Type?
1. Cerebral atrophy
2. Neuronal loss and neurofibrillary tangles, prominent in:
a. Nucleus basalis
b. Septal nuclei
c. Nucleus of the diagonal band
3. Amyloid plaques
In Alheimer's a dominant early feature is _________ & the next common feature is ____________, along with ____________, & ___________.
Memory loss
Word finding difficulty
Apraxia
Visuospatial deficits
Are individuals with Alheimer's are more likely to have problems with explicit or implicit learning?
Explicit
Behavioral difficulties occur early in Alzheimer's.

TRUE or FALSE
FALSE

(Consider frontotemporal dementia if behavioral problems are present early on)
Hallucinations are uncommon in Alzheimer's.

TRUE or FALSE
TRUE

(Consider demential with Lewey bodies if present)
Motor disturbances are usually present early on in Alzheimer's.

TRUE or FALSE
FALSE
Are neurofibriallary tangles associated with cognitive deterioration?
There is some debate in the literature; however, most recent findings suggest that the amount of tangles is directly correlated with the degree of cognitive deterioration
In Alheimer's, cortical atrophy, as much as 1/3 of the brain's volume as the disease progresses, occurs. Is this atrophy uniform?
No

Most affected: Parietal tertiary areas; inferior temporal cortex; and limbic cortex

Least affected: primary sensory and motor areas
Where are the most severe degenerative changes in Alzheimer's?
Limbic cortex; specifically, the entorhinal cortex, which is responsible for relaying information to and from the neocortex and hippocampus. This equates to loss of the hippocampal formation.
The strongest association between dementia and cell degeneration occurs with dysfunction in:

a) serotonergic neurons
b) norepinephrin neurons
c) cholingeric neurons
d) dopamineric neurons
c) cholinergic neurons

Acetylcholine is clearly reduced in individuals with Alzheimer's; therefore, the treatment is focused on enhancing cholinergic function)
What chromosomes have been found to be associated with late-onset Alzheimer's?
Chromosomes 19 & 12
What chromosomes have been associated with early-onset Alzheimer's?
Chromosomes 21 & 1
Describe Stage 1 (first 1-3 years) of Alzheimer's
Memory - new learning deficit, mildly impaired remote memory
V/S - topographic disorientation, poor complex construction
Language - empty speech with few substantive words and paucity of ideas; anomia
Personality - indifference, occasional irritability
Motor Systems - normal
EEG - normal
CT/MRI - normal
Describe Stage 2 (2-10 years) of Alzheimer's
Memory - recent and remote recall more severely impaired
V/S - poor constructions, spatial disorientation; patients can not find way about or copy constructions
Language - fluent aphasia, impaired comprehension; but, relatively preserved repetition
Cognitive Skills - severely impaired
Calculation - acalculia
Personality - indifference, irritability
Motor Systems - ideomotor apraxia, restlessness, pacing
EEG - slowing of background rhythm
CT/MRI - normal or ventricular dilation and sulcal enlargement
PET/SPECT - bilateral parietal and frontal hypometabolism/hypoperfusion
Describe Stage 3 (8-12 years) of Alzheimer's
Memory - severely impaired
Intellectual - severely impaired
Language - reduced to echolalia, palilalia (when a word is rapidly and involuntarily repeated), or mutism
Motor Systems - limbs assume a rigid and flexed position
Sphincter control - unrinary and fecal incontinence
EEG - diffuse slowing
CT/MRI - diffuse cerebral atrophy with ventricular dilation and sulcal enlargement
PET/SPECT - bilateral parietal and frontal hypometabolism/hypoperfusion
What are the possible causes of Alzheimer's?
1. Genetics
2. Trace metals - aluminum salts caused similar neurofibrillary degeneration in animals
3. Immune reactions - the immune system loses its ability to recognize its own body and therefore develops antibodies which can attack the brain and cause neuronal degeneration
4. Slow viruses - which takes years to develop (like with Creutzfeldt-Jakob's disease)
Primary progressive dementia of the Alzheimer's type usually involves a ____ deterioration.
slow
The first phase of Alzheimer's is _________ (noticed by the individual & sometimes those close to person) & accompanying _________ caused by forgetfulness.
The forgetful phase

Anxiety
In Alzheimer's the _____ phase leads to "severe deficits in memory for recent event, difficulty in _______ & _____, and ___________________ (e.g., word finding difficulty) despite generally intact vocabulary and syntax."
Confusion

Orientation, concentration, subtle language deficits
What does the dementia phase of Alzheimer's entail?
Severe disorientation, abnormalities of language, perception and praxis, and behavioral problems including motor restlessness, wandering, & psychotic symptoms (paranoia).
Pick's disease is a cortical dementia.

TRUE or FALSE
TRUE
Pick's disease is a rare disorder consisting of atrophy of _______ & _______ lobes from unknown causes.
Frontal
Temporal
At autopsy Pick's is differentiated from Alzheimer's because atrophy is confined to the _______ & _______ cortex, and _______ & ________ characteristic of Alzheimer's are not present.
Frontal, temporal

Plaques, tangles
Pick's patients have more memory, calculation, and visuospatial impairments than Alzheimer's patients.

TRUE or FALSE
FALSE
Pick's patients typically have more _________ changes than Alzheimer's patients.
Personality
How are verbal skills affected by Pick's disease?
It produces aphasia, but also has a greater tendency to produce stereotyped verbal output (i.e., repeating the same story or joke over and over)
What are Kluver-Bucy symptoms?
Visual agnosia, emotional changes, altered sexual behavior, hypermetamorphosis and oral tendencies
How can EEGs distinguish between Pick's and Alzheimer's patients?
EEGs will be normal in Pick's patients during the late stages of the disease.
Individuals with dementia due to Lewy Body Disease have hallucinations.

TRUE or FALSE
TRUE
Patients with Lewy Body Disease are more likely to have detailed _________ hallucinations, ___________ motor symptoms, (greater/lesser) sensitivity to antipsychotic medications, and significant ____________ in functioning.
visual
Parkinsonian
greater
fluctuations
The course of dementia due to Lewy Body Disease is the same as that for Alzheimer's.

TRUE or FALSE
FALSE

The onset is similar; however, Lewy Body Disease has a more rapid course
Because patients with dementia due to Lewy Body Disease have Parkinsonian movement disorders, they are responsive to l-Dopa.

TRUE or FALSE
FALSE

They are not responsive to l-Dopa, but they are overly sensitive to dopamine blocking agents
Where are Lewy Bodies found in Diffuse Lewy Body Dementia?
In the cortex, rather than confined to the basal ganglia as they are in Parkinson's patients.
Creutzfeldt-Jacob's Disease is a common transmitable virus that has a prevalance of 1 per 50,000.

TRUE or FALSE
FALSE

Creutzfeldt-Jacob Disease is very rare, occuring in only 1 per 1 million people.
What is a prion?
It stands for proteinaceous infectious particle.

It is an infectious agent that is made only from protein.
Typical presenting features of Creutzfeldt-Jacob's Disease include:

a)myoclunus, visual distortions
b) ataxia
c) rapidly progressive dementia, an exagerated startle response
d) hallucinations
e) a, b, and d
f) a and c
g) a, b, and c
h) all of the above
i) none of the above
g) a, b, and c
Creutzfeldt-Jacob's Disease can only be contracted by being exposed to infectious material.

TRUE or FALSE
FALSE

It can be inherited also.
The course of Creutzfeldt-Jacob's Disease is very slow, that is why it has been known to be a "slow virus."

TRUE or FALSE
FALSE

The course is actually very rapid, 6-12 months in length.

The reason it is known as a "slow virus" is because they can have an incubation period of 2-25 years.
What is myoclonus?
One or a series of shock-like contractions of a group of muscles, of variable regularity, synchrony, and symmetry, generally due to a central nervous system lesion.
What are prion-related dementias?
1. Creutzfeldt-Jacob Disease
2. Kuru
3. Fatal Familial Insomnia
4. Gerstmann-Straussler-Scheinker syndrome
What are the typical features of Frontal Lobe Dementia?
1. Changes in personality
2. Breakdown in social conduct
3. Loss of social awareness and emotional empathy
4. Disinhibition (the major finding)
5. Impulsivity
6. Unconcern
7. Changes in eating conduct (hyperphasia)
8. Stereotyped and perseverative behavior
Dementia is not typically seen in Huntington's Chorea.

TRUE or FALSE
FALSE

Dementia is a uniform part of the disease and may be the initial symptom preceding the appearance of chorea or other abnormalities.
What neuranatomical areas are typically affected in Huntington's Chorea?

a) caudate and putamen
b) frontal cortex and corpus callosum
c) basal ganglia and temporal lobes
d) thalamus
e) a, b, c
f) b, c, d
g) a, b, d
g) a, b, d

A degenerative loss of neurons is seen in these areas due to a genetic abnormality
What is the prevalence of Huntington's Chorea?

a) 1/1,000,000
b) 1/100,000
c) 5/500,000
d) 5/100,000
d) 5/100,000
What chromosome has been associated with Huntington's Chorea?

a) 1
b) 4
c) 21
d) 12
b) 4
Children manifesting signs of Huntington's Chorea will demonstrate cognitive or affective symptoms first.

TRUE or FALSE
FALSE

Children will typically show parkinsonian symptoms first; while later onset will typically manifest the cognitive or affective symptoms first.
Neuropsychological testing may be a helpful predictor for individuals at-risk for developing Huntington's Chorea.

TRUE or FALSE
TRUE

At-risk groups have been shown to perform poorly on frontal lobe tests.
Dementia features of Huntington's Chorea DOES NOT include:

a) apraxia
b) attention/concentration
c) executive abilities
d) memory
e) aphasia
f) agnosia
g) a, f
h) b, c
i) a, e, f
j) all of the above
k) none of the above
i) a, e, f
What are the characteristics of motor abnormality seen in Huntington's Chorea?
1. Movement NEVER involves single muscles but include whole limbs or parts of a limb.
2. Movements are irregular and follow no sense of pattern
3. Eventually the movements become uncontrollable and affect the head, face, trunk, and limbs, impeding all voluntary movements including speech and swallowing.
Other symptoms of Huntington's Chorea include:
1. Slow voluntary movements
2. Impaired saccades/occular disturbance
3. Gait abnormalities
4. Psychiatric disturbance (~50%)
5. Language is affected later in the disease, but it is hard to test because of dysarthria.
What should be considered if someone is demonstrating symtpoms of Huntington's Chorea, but does not have a positive family history for it?
Wilson's Disease
Huntington's Chorea is caused by a hyperactive norepinephrine system.

TRUE or FALSE
FALSE

It is thought that neurotransmitters which normally inhibit the dopamine pathways (GABA and norepinephrine) die during the course of the disease, thereby creating a hyperactive dopamine system, which leads to the chorea.
What causes Parkinson's Disease?
The degeneration of the substantia nigra, which produces the dopamine source for the basal ganglia. As the substantia nigra degenerates, it stops producing dopamine.
Parkinson's patients show a decrease of brain dopamine of over:

a) 25%
b) 50%
c) 75%
d) 90%
d) 90%
Parkinson's Disease is the most common extrapyramidal disorder affecting:

a) 1/100 over age 65
b) 1/1,000 over age 65
c) 1/10,000 over age 65
d) 1/100,000 over age 65
a) 1/100 over age 65
Parkinson's Disease is an inherited disorder.

TRUE or FALSE
FALSE
Clinically diagnosable dementia occurs in approximately what % of Parkinson's patients?
20-60%
What are the features of Parkinson's Disease?
1. Movement disorder
2. Bradyphrenia (mental slowing)
3. Memory problems
4. Executive problems
5. Attentional difficulties
6. Visuospatial difficulties
7. Depression (~30%)
Are cortical disturbances like aphasia and apraxia typically seen in Parkinson's patients?
No
What is the classic triad of Parkinson's Disease?

a) resting tremor, gait disturbance, posture disturbance
b) gait distubance, posture disturbance, bradykinesia
c) resting tremor, cogwheel rigidity, bradykinesia
d) resting tremor, cogwheel rigitiy, gait disturbance
c) resting tremor, cogwheel rigidity, bradykinesia

Disturbance of posture is also quite frequent
What are the POSITIVE SYMPTOMS of Parkinson's Disease?
Actions which are not seen in normals

1. Resting tremor - "pill rolling"; are frequently ASYMMETRICAL; stops during voluntary movements or during sleep
2. Muscular rigidity - cogwheel effect, muscles allow movement for a short distance and then resist movement again
3. Involuntary movements - akathisia, meaning inability to remain in one posture
What are the NEGATIVE SYMPTOMS of Parkinson's Disease?
Inability to engage in behaviors that normals can

1. Posture disorder - inability or difficulty in maintaining a part of the body in normal position (e.g., head drooping)
2. Righting disorders - difficulty in standing from a sitting position
3. Locomotion disorders - difficulty initiating stepping, they shuffle; often once start walking, take faster and faster steps until they end up running (festinating gait)
4. Speech disturbance - difficulties in the physical production of sound
5. Akinesia - poverty or slowness of movement (e.g., blank facial expression, lack of blinking, etc.)
6. Aphagia - difficulty chewing/swallowing.
Medications help reduce motor and cognitive symptoms in Parkinson's Disease.

TRUE or FALSE
FALSE

Medications can help with motor symptoms, but do not improve cognitive deficits; in fact, some patients may become confused with the medication
Surgical interventions can help which aspects of Parkinson's Disease?
1. Pallidotomy - dyskinesia (abnormality in performing voluntary muscle movements)

2. Thallotomy - tremor
What is the name of the dementia that results from the degeneration of multiple structures including the superior colliculus, red nucleus, dentate nucleus, subthalamic nucleus, and globus pallidus?

a) Parkinson's
b) Alzheimer's
c) Wilson's
d) Progressive Supranuclear Palsy
d) Progressive Supranuclear Palsy
Progressive Supranuclear Palsy is earmarked by parkinsonism symptoms, swallowing difficulty, and supranuclear opthalmoplegia.

TRUE or FALSE
TRUE
Some of the more common features of Progressive Supranuclear Palsy are?
1. Gaze paresis - loss of downward gaze, leading to frequent falls and "dirty tie" sign
2. Axial rigidity - trunk/neck rigidity producing erect or hypererect rather than stooped posture.
3. Bradykinesia
4. Pseudobulbar palsy - masked face, increased jaw and facial jerks, exaggerated palatal and pharyngeal reflexes, dysphagia, drooling, emotional lability
5. Gait disorder - falls and akinesia (motionlessness attributable to a temporary paralysis) can also be present
6. "Wide-eyed stare"
What is the peak onset for Wilson's Disease?
Teens - 20s
What is the cause of Wilson's Disease?
Abnormalities in copper metabolism
Wilson's Disease is NOT inheritable.

TRUE or FALSE
FALSE

Wilson's Disease is a disorder of an autosomal recessive gene on chromosome 13
Are the effects of Wilson's Disease reversible?
Yes - if caught early treatment which includes restriction of copper intake and enhancing copper elimination can reverse haptic, renal, and cerebral dysfunction.
Wilson's Disease includes all but which of the following:

a) Parkinsonina symptoms
b) Psychosis
c) Dementia
d) Mood changes
e) Wandering tendencies
f) Kayser-Fleischer rings
e) Wandering tendencies
Do Kayser-Fleischer rings show up on all patients with Wilson's Disease?
No - these copper corneal rings, while pathognomonic, may not be seen in every patient
What is Hallervorden-Spatz Disease?
A rare inherited and progressive illness with late childhood or early adolescent onset.

It is characterized by dementia with spasticity and rigidity, dystonia or chorea.
What causes Hallervorden-Spatz Disease?
An accumulation of iron in the brain, for which there is no current cure.
What is Sydenham's Chorea?
a.k.a. - "Saint Vitus Dance"

A disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands.
What is Sydenham's Chorea typically associated with?
Rheumatic fever
Sydenham's Chorea typically affects adults between the ages of 30 and 65.

TRUE or FALSE
FALSE

Most patients are children under the age of 18
Sydenham's Chorea is progressive and incurable.

TRUE or FALSE
FALSE

50% of Sydenham's Chorea patients recover spontaneously after 2-6 months, while mild to moderate chorea or other motor symptoms can persist for up to and over 2 years.
Sydenham's Chorea is associated with all but which of the following:

a) Tics
b) Tourette's
c) ADHD
d) Psychosis
e) OCD
f) Lupus
d) Psychosis
What is dementia pugilistica?
A form of head trauma-related dementia seen mainly in boxers who suffer recurrent head injuries, in which there is parkinsonism and cognitive decline.

aka - Punch Drunk Syndrome
What kinds of neurological changes are seen in dementia pugilistica?
1. White matter changes
2. Focal contusions
3. Cerebral atrophy in porportion to the number of fights (especially the corpus callosum and cerebrum)
4. Hydrocephalous
5. Depigmented substantia nigra
6. Neurofribrillary tangles
7. Amyloid plaques