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20 Cards in this Set

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pt presents with multiple cognitive deficits including memory impairment, aphasia, apraxia (arm won't do what you tell it to), agnosia (trouble remembering what objects are), or trouble in executive functioning (have trouble cooking). What do they have?

(o)
Alzheimer's Dz

note: the shrinking effect on the brain is bigger due to age than Alzheimers (like an 80 year old with AD would have a larger brain than a normal 90 year old)
on a PET scan, what will be a difference seen in a pt with Alzheimer's dz?
less activity posteriorly
amyloid plaques are commonly seen on silver stain in what? what is another pathological finding of this problem?
Alzheimer's Dz

Neurofibrillary Tangles (used to be neurotubules, will be in the cells)
What are 2 pharmacological tx for AD?
Acetylcholinesterase Inhibitors [Examples: Donepezil (Aricept) Galantamine (Reminyl, Razadyne)
Rivastigmine (Exelon)] --used in mild to moderate disease

NMDA (Glutamate) Receptor Blocker (Memantine (Namenda))-- Moderate level of disease
Progressive dementia clinically similar to Alzheimer’s disease

Early, prominent involvement of frontal and temporal lobes (“frontotemporal dementia”)

(o)
Pick's Disease

will find Pick Bodies intracellularly

note: PET scan will show less activity in the frontal and temporal lobes (who'da thought)
Hypokinesia/akinesia
Bradykinesia
Rigidity (not to be confused with spasticity or paratonia)
Stooped posture
Small-stepped gait
“Rest” tremor, typically unilateral or asymmetric

area all seen in what?
Parkinsonism
associated with degeneration of substantia nigra cells containing intracellular Lewy bodies

(o)
Parkinson's Disease
Usual fluctuations in cognitive function, involving alertness and attention
Visual hallucinations are common
Will also have movement problems
Pathologically, intracellular Lewy bodies appear diffusely in the ****cerebral cortex*****

(o)
Dementia with Lewy Bodies

Clinically resembles a blend between Alzheimer’s and Parkinson’s diseases

note: in Parkinson's, the Lewy Bodies are mostly in the Substantia Nigra
flip for a comparison of dementia with Lewy Bodies, Alzheimer's Disease, Parkinson's
pt presents to your office due to changes in mood. Upon walking you notice that they seem to flick their hand, or kind of dance around. What is this? Genetics?

(o)
Huntington’s Disease

Autosomal dominant with high penetrance
Abnormal gene on chromosome 4

note: the dancing/flicking movement is known as CHOREA
What change in the brain would you see in a pt with Huntington's disease?
Absence of the Caudate Nucleus
A rapidly progressive, fatal dementia involving prominent motor symptoms, such as rigidity, clumsiness, and myoclonic jerks
A transmissible “spongiform” encephalopathy involving abnormal proteins called prions
Diagnosed with certainty only by brain biopsy

(o)
Creutzfeldt-Jakob Disease
What are the 3 categories of Creutzfeldt-Jakob Disease? Which are we most concerned about?
Sporadic CJD appearing without known risk factors accounts for 85% of cases.

Hereditary CJD involves positive family history or positive testing for a genetic mutation. 5-10% of U.S cases.

Acquired CJD involves transmission by exposure to brain or nervous system tissue. 1% of cases. (****transmission from open wounds, retina transplant, etc)
Increased levels of CSF protein 14-3-3 would give you the diagnosis of?
Creutzfeldt-Jakob Disease
inflammatory demyelinating plaques in the central nervous system is the hallmark of what?

(o)
Multiple Sclerosis (MS)

has to do with OLIGODENDROCYTES (not Schwann--peripheral)
T1 “Black Holes” and brain atrophy can be seen in what?
Multiple Sclerosis

note: Gilenya ™ - Fingolimod is the first oral pill tx for MS
-susceptibility gene → HLA class II genes (DR2 haplotype

- Higher prevalence in Caucasians of Northern European Ancestry

- Higher incidence in women

- One of the most common causes of neurologic disability in young adults

Disease??
MS

--------------------------------------------------

Putative MS-associated genes:
• Chemokine receptor: CCR5
• ApoE-43
• CNTF4
- Hallmark is inflammatory, demyelinating plaques in CNS

Most common symptom at the disease onset → sensory symptoms in arms/legs

Most common symptoms → bladder sx, fatigue, spasticity

- Oligoclonal bands found in Gamma-Proteins of CSF
MS
1st sign tends to be one arm doesn't swing while the person walks
Parkinsonism
describe the differences in appearance and significance of tremors that are maximal at rest, upon posture-holding and on intention.
**Maximal at rest → associated with Parkinson's and Parkisonism**
• Often oscillates about pronator-supinator axis INSTEAD of flexor-extensor axis
• Fingers can give appearance of **pill rolling**
• ↓ when muscles are put to use but DOESN'T necessarily disappear

**Postural Tremor**
• Typically oscillates in the flexor-extensor axis
• Maximal on postural holding or when muscles are put to use
• ↓ when at rest
• Can be caused by different medications, hyperthyroidism of familial

**Intention Tremor**
• Gets worse during the finger-nose test
• ↑ in severity when muscles are put to use
- Indicates a ***cerebellum pattern of pathology***