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What are 4 Neurodegenerative Disorders Discussed in Class?
1) Alzheimer's Disease
2) Parkinson's Disease
3) Huntington's Disease
4) Amyotrophic Lateral Sclerosis (ALS)
Which Neurodegenerative Disease is Hypokinetic? Which is Hyperkinetic?
Hypokinetic: Parkinson's
Hyperkinetic: Huntington's
T/F: Dementia is a specific disease
False, it is a group of symptoms caused by some type of brain damage
Define Dementia
a progressive loss of cognitive functioning which interferes with daily activities
Dementia is More Common in Younger/Older People
more common in older people (risk increases w/ age)
Is Dementia Part of the Normal Aging Process?
it is not part of the normal aging process
What are 4 Specific Mechanisms That Can Cause Brain Damage Leading to Dementia
1) Neuron degeneration
2) Atherosclerosis
3) Brain tissue compression
4) Brain trauma
T/F: It is possible to be genetically predisposed to dementia
true
If a Young Patient Had Dementia, What Would Be the Most Likely Cause?
genetics
What is the Most Common Type of Dementia?
Alzheimer's Disease
What is Alzheimer's Disease?
a progressive neurological disorder that affects cognition (memory, thinking skills) and causes an inability to carry out the simplest tasks
What are the 2 Main Types of Alzheimer's Disease? Which One is Most Common?
1) Late Onset (95% of cases):
2) Early Onset Familial (5% of cases):
What are 2 Types/Causes of Late Onset Alzheimer's Disease? Which One is Most Common?
1) sporadic (doesn't have genetic association) (most common)
2) alteration in apolipoprotein E gene on chromosome 19
What is 1 Cause of Early Onset Non-Familial Alzheimer's Disease?
1) alteration in genes on chromosome 21
T/F: Older people with higher cholesterol have a higher risk of Alzehimer's
true
What are 2 Main Components that Cause Neuron Damage Responsible for Alzheimer's Disease? (the pathophysiology)
1) accumulation of extracellular β amyloid causing senile/neuritic plaques
2) accumulation of intracellular tau protein causing neurofibrillary tangles
Explain How Abnormal Tau Proteins Cause Neurofibrillary Tangles
Tau proteins are part of microtubules in the neurons → misfolded Tau proteins cause microtubules to tangle → neurofibrillary tangles
Over How Many Years Do the Signs & Symptoms of Alzheimer's Extend?
extend over 10-20 years
List 5 Signs/Symptoms Seen in Alzheimer's Patients
1) behavioral changes (irritability, mood swings)
2) progressive memory loss
3) lack of concentration
4) impaired learning/language
5) poor judgment
T/F: There is a definite diagnostic test to diagnose Alzheimer's
false, but after patient is dead a postmortem brain biopsy can be done to confirm alzheimer's
What are 2 Treatments that Can Be Used for Alzheimer's
1) anticholinesterase drugs (temporary improvement)
2) therapy (speech, physical, etc)
What is the Main Goal of Treatment for Alzheimer's?
to slow the progression of the disease & make the patient more comfortable
What is Parkinson's Disease?
a progressive, neurodegenerative disease of the melanin-containing dopaminergic neurons in the substantia nigra pars compacta
T/F: Parkinson's Disease involves severe degeneration of the basal ganglia
true
What are 4 Types of Parkinson's Disease? Which One is the Most Common?
1) Idiopathic (unknown cause)
2) Hereditary/Genetic (autosomal dominant & recessive)
3) Environmental (pesticides)
4) Acquired (medications; head trauma like Muhammad Ali)
Explain the Gross & Microscopic Pathophysiology of Parkinson's Disease.
Gross: loss of pigmented dopaminergic neurons of the substantia nigra pars compacta
Microscopic: the presence of Lewey bodies/neurites
What Causes the Development of Lewey Bodies/Neurites Seen in Parkinson's Disease?
accumulation of α-synuclein → causes dissociation of ubiquitin from carrier protein → increases in ubiquitin in the cytoplasm → causes apoptosis of neurons
T/F: In Parkinson's Disease, degeneration of dopaminergic neurons in basal ganglia causes a dopamine deficiency
true
What are 6 Signs of Parkinson's Disease?
1) resting tremor
2) masked facies (fixed, mask-like facial expression)
3) muscle rigidity
4) bradykinesia (slow movement)
5) poor balance
6) short shuffling steps
What are 5 Treatments Used for Parkinson's Disease
1) Dopamine replacement therapy/ agonist (Levodopa)
2) Anticholinergic Drugs
3) MAO-B Inhibitor
4) Amantadine
5) Therapy (speech, physical)
What is Huntington's Disease?
a progressively debilitating neurodegenerative disease that is genetically inherited
What is the Pattern of Inheritance of Huntington's Disease?
autosomal dominant
What Specific Chromosome & Gene are Affected in Huntington's Disease?
Chromosome: 4
Gene: Huntingtin (HTT) gene
At What Age Does Huntington's Disease Usually Appear?
around 40s or later
T/F: Huntington's Disease involves a progressive atrophy of the brain
true
List 2 Aspects of the Pathophysiology of Huntington's Disease.
1) depletion of gamma-aminobutyric acid (GABA) in basal ganglia neurons → apoptosis
2) repeat polyglutamine expansions on the Huntingtin gene
What are 4 Signs of Huntington's Disease?
1) mood swings
2) personality changes
3) choreiform movements
4) purposeless movement in arms & face
What is the Main Diagnostic Test to Diagnose Huntington's Disease?
DNA test
What Treatment is Used For Huntington's Disease?
there isn't a true treatment, just therapy to help with symptoms
What is Amyotrophic Lateral Sclerosis (ALS)? What is Another Name for It?
a rapidly progressive & fatal neurodegenerative disease of the upper & lower motor neurons (AKA Lou Gehrig's Disease)
Explain the Pathophysiology of Amyotrophic Lateral Sclerosis (ALS).
Superoxide Dismutase (SOD1) gene mutation causes damage to glutamate uptake channels in astrocytes → reactive oxygen species build up in neurons → early upper/lower motor neuron death
What are 2 Risk Factors for Amyotrophic Lateral Sclerosis (ALS)?
1) Gulf War veteran
2) repeated head trauma (athletes)
T/F: Amyotrophic Lateral Sclerosis (ALS) affects cognition as well as motor function
False, cognition is NOT impaired, only motor function
Loss of Upper Motor Neurons Causes _____________? Loss of Lower Motor Neurons Causes _______________?
Upper Motor Neuron Death: spastic paralysis (muscles stay contracted) & hyperreflexia
Lower Motor Neuron Death: flaccid paralysis (muscles can't contract) & hyporeflexia
Besides Spastic/Flaccid Paralysis and Hyper/Hyporeflexia, What Other 2 Signs Present with Amyotrophic Lateral Sclerosis (ALS)?
1) progressive muscle weakness
2) loss of fine motor coordination (stumbles/falls)
Patients with Amyotrophic Lateral Sclerosis (ALS) Usually Die From _____________?
respiratory failure
What are 2 Treatments Used For Amyotrophic Lateral Sclerosis (ALS)?
1) Riluzole (slows further damage to neurons)
2) Therapy (speech, physical, etc.)
What are 3 General Categories of Cerebrovascular Disorders? Describe Each
1) Ischemia (Local: end of artery blocked. Global: beginning of artery blocked)
2) Hemorrhage (bleeding increases intracranial pressure & causes ischemia)
3) Arteriovenous malformations (abnormal connection /w vein & artery)
What are 2 Specific Cerebrovascular Disorders Discussed in Class? One Results in Tissue Death & the Other Does Not
1) Transient Ischemic Attacks (warning of impending stroke)
2) Cerebrovascular Accident (stroke)
What is a Transient Ischemic Attack (TIA)?
a transient episode of neurological dysfunction caused by focal brain, spinal cord, or retinal ischemia without infarction
T/F: Transient ischemic attack is a warning sign of an impending stroke, just like unstable angina is a warning sign of an impending myocardial infarction
true
T/F: The neurological dysfunction from a transient ischemic attack last less than 1 hour
true
What are 5 Causes of Transient Ischemic Attacks? Which One is the Most Common?
1) Atherosclerosis
2) partial occlusion of artery
3) vascular spasm
4) arteritis (artery inflammation)
5) mass lesions
What are 5 Signs That May Develop After a Transient Ischemic Attack? (Remember: these signs should last less than 1 hour)
1) muscle weakness
2) problems with vision
3) numbness
4) paresthesia (burning/tingling)
5) confusion/speech problems
What is the Preferred Diagnostic Test for Transient Ischemic Attack?
MRI
What is a Cerebrovascular Accident (Stroke)?
an infarction of brain tissue that results from lack of blood
What are 3 Types of Cerebrovascular Accidents?
1) Thrombotic stroke (artery occluded by thrombus)
2) Embolic stroke (artery occluded by embolus)
1 & 2 are Types of Ischemic Strokes (blood vessel occlusion)
3) Hemorrhagic stroke (blood loss in brain increases intracranial pressure and can cause ischemia)
How Many Minutes of Ischemia are Required for Irreversible Neuron Death
5 minutes
What is the Primary Cause of Hemorrhagic Strokes?
hypertension → arteriosclerosis (causes blood vessel to eventually burst)
T/F: Cerebrovascular Accidents are #1 leading cause of disability and #5 leading cause of death in the US
true
What are 2 Risk Factors Associated w/ Cerebrovascular Accidents?
1) hypoperfusion (low oxygen perfusion)
2) African Americans more likely to have CA
T/F: Cerebrovascular accidents initially cause spastic paralysis that develops to flaccid paralysis later
False, flaccid paralysis develops initially and then becomes spastic paralysis
What 3 Things Does the NIH Stroke Scale Tell You?
1) assists with rapid diagnosis
2) predicts size & severity
3) predicts short & long term outcomes
What are 5 Signs of Cerebrovascular Accidents?
1) hemiparesis (muscle weakness on one side)
2) vision problems
3) facial droop
4) loss of speech
5) confusion
What is the Main Goal of Treatment for a Cerebrovascular Accident?
restore perfusion, counteract ischemia, prevent necrosis
What are 4 Treatments Used For Cerebrovascular Accidents?
1) Fibrinolytic (tPA) (dissolves clot) - given within 4.5 hours or less
2) Aspirin (antiplatelet agent to reduce clotting)
3) Glucocorticoids
4) Therapy (speech, physical, etc.)
What are 4 Methods of Prevention for Cerebrovascular Accidents?
1) STOP smoking
2) control hypertension
3) cholesterol lowering drugs
4) healthy diet/exercise
What are 3 General Types of Meningitis?
1) Bacterial meningitis (most deadly
2) Viral meningitis (most common)
3) Fungal meningitis (seen in immunocompromised)
What are 6 Common Signs of Meningitis?
1) fever (meningitis will always be accompanied by fever)
2) cold hands & feet
3) rash
4) drowsiness
5) muscle pain
6) dislike bright lights
What 2 Reflex Signs are Seen in Meningitis Patients?
1) Kernig Sign (extension of the knee is painful or limited in extension)
2) Brudzinski Sign (passive flexion of neck causes hip & knee flexion)
Explain Cerebrospinal Fluid (CSF) Color, Protein, Glucose, & Cells Between Bacterial & Viral Infections
Bacterial Infection: Color: turbid/yellow. Protein: high. Glucose: low. Cells: neutrophils (PMNs)
Viral Infection: Color: clear/opaque. Protein: high. Glucose: normal. Cells: lymphocytes
What are 3 Treatments Used For Meningitis?
1) aggressive antimicrobial drugs
2) glucocorticoids (reduce inflammation/edema)
3) vaccines
What is Multiple Sclerosis? What is it Caused By?
What: a progressive, inflammatory, demyelinating disease of the CNS
Cause: autoimmune
What Demographic of People is Multiple Sclerosis Most Common?
1) more common in Caucasians
2) more common in females
3) 20-40 years old
What is the Pathogenesis of Multiple Sclerosis?
breakdown of the blood brain barrier → inflammation → destruction of oligodendrocyte myelin sheaths → demyelination → scar formation → plaque formation → neurodegeneration
What are 7 Signs of Multiple Sclerosis?
1) muscle weakness
2) paresthesia (numbness/burning/prickling)
3) optic neuritis
4) vision problems
5) bladder/bowel incontinence
6) speech problems
7) Lhermitte sign (flexion of neck causes jolt down back)
What is the Test of Choice for Diagnosing & Monitoring Multiple Sclerosis?
MRI
What is the Main Goal of Treatment for Multiple Sclerosis?
to prevent or slow down time between relapses
What are 3 Specific Treatments for Multiple Sclerosis?
1) corticosteroids
2) beta interferons
3) glatiramer acetate