Pathophys test 2
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_______: chronic degenerative disorder of the CNS that poduces movement problems. Most obvious changes are motor related, but cognitive symptoms can appear as well.
True or false: the cause of parkinson's is iatarogenic.
False: idiopathic (also genetic and environmental component)
_______ parkinsonism is not true PD, but the symptoms appear with other medical problems (meningitis, encephalitis, stroke, vascular disease, repeated head trauma etc.)
4 risk factors for Parkinson's (they are very general)
age, gender, genetic, exposure to environmental toxins
Incidence of parkinson's over the age of 60 increases from a __-__% chance to __to__%.
(men/women) are 1.5x more likely to develop PD.
Pathophysiology of Parkinson's:
Disruption of the ______ motor system. These tracts are associated with the _____ _____, which plays a big role in movement.
- extrapyramidal motor system
- basal ganglia (plays a big role because it contains the substantia nigra)
The substantia nigra is filled with _____ secreting neurons. In PD, many of these neurons are destroyed. Dopamine is an (excitatory/inhibitory) neurotransmitter which helps control movement.
Decreased levels of dopamine causes the remaining neurons to be overy (inhibited/excited)?
In addition to damage to dopamine producing neurons in PD, damage to ______ secreting neurons and the presence of ______ bodies can also occur.
- norepinephrine (effects the ANS)
- Lewy bodies (associated with Dementia and memory loss)
______ gait: common with PD, short, shuffling steps that increase in speed over time and often lead to falls.
Patient's with PD often experience a ______ _____, where a person feels stuck and finds it difficult to start moving again.
- Festinating (propulsive)
- "Freezing Period"
Describe typical standing posture of PD patient's: (essentially three things)
stooped, leaning forward, head and neck flexed
The most common diagnostic test for PD is and MRI/CAT scan. True or false
false, no existing diagnostic test exists for PD
Describe the 5 stages of PD:
- Stage 1: unilateral symptoms
- Stage 2: bilateral symptoms
- Stage 3: balance trouble, but able to amb indep
- Stage 4: assist needed for amb
- Stage 5: wheel-chair or bed-bound
What two drugs are used (most commonly) for PD?
- (carbidopa allows levadopa to cross the BBB)
In alzheimer's disease, genetic mutations on chromosomes __,__, and __ are known to cause early onset or familial ALZ D which effects people between 30-60. People with ______ ______ have an increased chance of getting ALZ D.
- 1,14, and 21
- down's syndrome
1 in ___ persons over 65 have AD. (men or women) have a higher risk of having AD.
Describe the 7 stages of AD:
- 1. No impairment
- 2. Very mild cognitive decline
- 3. Mild cognitive decline
- 4. Moderate cognitive decline
- 5. moderately severe cognitive decline
- 6. severe cognitive decline
- 7. very severe cognitive decline
______ plaques: contain beta-amyloid proteins found in the extracellular deposits in the CNS. Interfere with functioning by stimulating production of free radicals.
________ _______: paired helical filaments of tau proteins (which are normally important for growt hand devlopment of axons) When these proteins are hyperphosphorylated, they form _____ which leads to cell death.
- neurofibrillary tangles
With AD, neuron and synapse loss follows a pattern primarily affecting the pathways in:
- Basalis Nucleus of Mevnert (cholinergic pathway/Ach which influences memory.
- Median Raphe (serotonergic receptors)
- Locus Ceruleus (adrenergic receptors which leads to loss of norepi)
The only confirmatory test for AD is an?
Early onset AD presents before the age of ___.
Name the 2 main types of drugs to treat AD?
Which vitamin is also supplemented?
- 1. Ach Inhibitors (keeps levels of Ach high)
- 2. Memantine (NMDA antagonist which regulates glutamate (learning and memory))
- Vitamin E
____ _____ ____ ____: may improve memory, mood, ciradian rhythm, and level of awareness of AD patients.
transcutaneous electrical nerve stimulation
_____ _____: progressive demyelination of neurons of the brain, spinal cord, and cranial nerves. Characterized by remissions and exacerbations.
What is the cause of MS?
unknown, many believe it is autoimmune; appears to have a genetic component, immunologic, and environmental component as well.
MS is most frequent in _____ in _____ zones (northern US and Canada)
- caucasians (with european descent)
- temperate (warm climates)
True or false: MS is the most common cause of disability in the US.
False: second to motor vehicle accidents
MS is more common in (men or women) and onset of symptoms occur between ____ and _____ with a peak at ____.
_____-_____ MS: most common form of the disease. Characterized by clearly defined exacerbations and remissions followed by partial or complete recovery. 85% of people with MS begin with this course.
_______ ________ MS: characterized by progression of disability of onset, without plateaus or remissions, but can have periods of stabilization. By definition, does not experience acute attacks. 10-15% of people.
Primary progressive MS
______ _______ MS: begins as the relapsing remitting ms, followed by progression of disability that may include occasional relapses, minor remission, and plateaus. Less recovery following attacks, persistently worsening functioning during and between attackss, and fewer and fewer attacks.
Secondary Progressive MS
_______ _______ MS: least common form, shows progression of disability from onset but with clear acute relapses with or without full recovery.
______ ______: conduction of action potentials along myelinated axons from one node of Ranvier to the next.
True or false: Earliest lesions in MS occur as inflammatory response when myelin is lost in the white matter of the brain and spinal cord. Later, larger areas are known as plaques because they are visible.
True (plaques seen in lateral ventricles of brain, brain stem, and optic nerves)
True or False: The neurological exam is conclusive for MS.
False: no single test is conclusive
______ is a common first sign of MS follwoed by weakness in LE due to plaques in the corticospinal tract.
3 common sensory abnormalities on clinical exam for MS:
- impairment of vibration and joint position sense
- decreased pain and light touch perception distally
- patchy areas of reduced pain and light touch perception
(pain also) but that makes four and i didn't want to go back and change my original question.
4 Goals of MS treatment:
- improve speed of recovery from attacks
- reduce number of attacks and lesions
- slow progression
- relieve other complications
______ _______ _____: progressive neurodegenerative disease that attacks nerve cells in the brain and spinal cord resulting in muscle weakness and atrophy.
amyotrophic lateral sclerosis
Etiology of ALS: 2 choices
- unknown (90%)
- genetic (10%)
Average age of incidence of ALS ____-____ years old. (males or females) are 1.5 times more likely to have and (what ethnicity) is 1.6 more likely to have ALS.
After being diagnosed with ALS, death usually occurs within __-__ years.
ALS degenerates both ____ and ____ neurons. As motor neurons degenerate, the _____ and _____ columns of the spinal cord are replaced by fibrous astrocytes.
- upper and lower
- anterior and lateral
_______ degeneration: process by which motor axons die. Death occurs in the _____ horn of the cell body. Causes degeneration of associated motor axon. Surrounding Schwann cells breakdown the axon's myelin sheath, which results in the axons breaking into pieces.
- Wallerian degeneration
- anterior horn
ALS causes degeneration of the _______ upper motor neurons that reside in the cerebral cortex specifically ____ _____. Loss of these upper motor neurons causes spastic paralysis, hyperreflexia, and stiffness.
- corticospinal upper motor neuron
- precentral gyrus
ALS causes the degeneration fo the lower motor neurons that reside in the ______ ______ of the _____ ____ and _____. Loss of these motor neurons leads to flaccid paralysis, decreased muscle tone, decreased reflexes, muscle weakness, and muscle atrophy.
- anterior horn
- spinal cord and brainstem
ALS does not affect: 4 things
- Cranial Nerves III, IV, and VI
- Sensory neurons
- Cognitive function
- Bowel and bladder function
______ is the only exogenous factor that may contribute to ALS.
_____ is the 1st and only drug that slows the disease ALS and prolongs life
_____ _____ ______: syndrome where there is inflammation in the peripheral nervous system. Thought to be autoimmune. Occurs about 1-3 weeks after an infection, surgery, or vaccination.
In GBS, peripheral nerves in the (arms or legs) are affected first. Critical period occurs when paralysis of the _____ and ______ begins.
- diaphragm and respiratory muscles
GBS leads to impaired nerve conduction mainly in the _____ fibers. Axons can regenerate if the cell bodies stay intact.
efferent (motor) fibers
Recovery from GBS usually occurs __-__ years after the initial onset. Most people recover completely __%.
_____ _____: autoimmune disorder that leads to skeletal muscle weakness and rapid muscle fatigue. Acetylcholine receptors become impaired at the NMJ.
MG in general is more common in (men or women).
women are usually most susceptible between the ages __-__.
men are usually over the age of __?
In MG, ____ autoantibodies bind to ach receptors and block or destroy the sites. If ach cannot bind to the receptor site, muscle stimulation can't occur.
Muscles of the ____ and ____ are the first to be affected.
group of inherited disorders characterized by degeneration of skeletal muscle
MD is a metabolic defect caused by a deficit of ______ resulting in cell degeneration and necrosis
T/F: Few muscular dystrophies are inherited and involve a mutation in genes
False: all involve a mutation
Can MD be caused by spontaneous mutations?
What are the 3 types of inheritance for MD?
- Autosomal Dominant
- Autosomal Recessive
- X-linked Recessive
In MD, muscle appears ______ because muscle fibers are replaced with ____ & _______ ______ tissue
- fat & fibrous connective tissue
holds the cytoskeleton to the extracellular matrix
Know inheritance, age of onset, distribution & speed of progression for:
Fascioscapulo humeral MD
Limb Girdle MD
- Duchenne's - x-linked recessive, 2-3 years, hips legs & shoulder girdle, rapid
- FH MD - autosomal dominant, before 20, shoulder neck & face, slow to moderate
- Myotonic - autosomal dominant, birth to 50 yrs, face & hands, slow
- Limb Girdle - autosomal recessive, all ages, shoulders & pelvis, varied
3 ways to test for MD
- Physical Exam
- Muscle Biopsy
- Blood test for creatine kinase (elevated with death of muscle)
an autosomal recessive neurodegenerative disease characterized by degeneration of spinal cord motor neurons, atrophy of skeletal muscle & muscle weakness
Spinal Muscular Atrophy (SMA)
cause of SMA
mutation of SM1 gene (survival motor neuron 1)
SMN protein from the SM1 gene is crucial for the survival and health of __-motor neurons
type ___ SMA: Werdnig-Hoffman disease
most severe, affects infants (<8 months), death before 2 years, never able to sit
type ___ SMA: less severe, children progressively weaker, 7-18 months, death after 2 years, never stands
type ___ SMA: least severe, can begin as a child or an adult, >18 months, death as an adult, is able to stand and walk
4 general tests for SMA
- genetic (pre conception and blood for post)
- prenatal (chronic villi sampling & amniocentesis)
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