Degenerative Disorders of the Central Nervous System PART 1 (start-Parkinson's) Flashcards
what does ALS stand for
amyotrophic lateral sclerosis
what is amyotrophy
muscle atrophy (due to peripheral nerve changes)
what does the “lateral” part of ALS mean
motor neurons in the lateral aspect of the spinal cord, brainstem, and cerebral cortex involved
what does “sclerosis” part of ALS mean
degeneration and scarring
what is the most common form of adult onset progressive motor neuron disorder
ALS
how many new cases of ALS/year
5000
what is the most physically devastating of the neurodegenerative disorders
ALS
two types of ALS
- sporadic
2. familial
what type of ALS is more common
sporadic
what is the cause of sporadic ALS
unknown
what type of genetic disorder is familial ALS
autosomal dominant
familial ALS: early or late onset
early onset
what gene is mutated in familial ALS
superoxide dismutase gene mutation
are males or females more affected by ALS
males
what disease prevents ALS
polio
general pathogeneses of ALS (2)
- destruction of UMN
2. destruction of LMN
area affected in UMN destruction of ALS
cerebral cortex
what two tracts are involved in UMN involvement in ALS
- corticospinal tract
2. corticobulbar tract
where are the alpha motor neurons that are affected in LMN involvement in ALS
anterior horn of cord and cranial nerve nuclei in brainstem
possible causes of ALS (2)
- excitotoxicity
2. oxidative damage
how does excitotoxicity play into ALS
excess glutamate
how does oxidative damage play into ALS
free radicals
are LMN symptoms symmetrical or asymmetrical
asymmetrical
are LMN symptoms proximal or distal
distal
LMN symptoms involve what part of the body and what motion is weak
cervical extensor weakness (head drop)
are LMN symptoms hypo or hyperreflexive
hyporeflexive
are LMN symptoms hypo or hypertonic
hypotonic
are LMN symptoms atrophy or hypertrophy
atrophy
muscle ____ are noted with LMN symtpoms
cramps
what else may be present with LMN symptoms
fasciculations
are UMN symptoms spastic or nah
spastic
are UMN symptoms hypo or hyperreflexia
hyperreflexia
what reflexes are present with UMN symptoms (3)
clonus, babinski, hoffmann
are UMN symptoms muscles strong or weak
weak
as the disease progresses, UMN/LMN? symptoms may decrease
UMN
for ALS, what other kinds of symptoms may be present
bulbar
with UMN involvements, what palsy is present in ALS
Pseudobulbar Palsy
what is present with Pseudobulbar Palsy
spasticity
with LMN involvements, what palsy is present with ALS
Bulbar Palsy
what is present with Bulbar Palsy
flaccid
presentation of bulbar symptoms (4)
- dysarthria
- dysphagia
- sialorrhea
- pseudobulbar affect (UMN)
preserved areas in ALS (4)
- eye movements
- posterior columns
- bowel and bladder function
- cognition (50% preserved)
early signs of ALS (3)
- insidious asymmetrical weakness of distal aspects of one limb
- cramping with volitional movement - early morning stiffness
- muscle fasciculations - spontaneous twitching of muscle fibers
late manifestations of ALS (2)
- respiratory complications –> respiratory failure
2. oral motor complications –> risk of aspirations
for diagnosing ALS, what can EMG reveal (2)
- fibrilations
2. fasciculations
for diagnosing ALS, what can muscle biopsy reveal
denervation atrophy
for diagnosing ALS, what can muscle enzymes reveal
CPK levels elevated
for diagnosing ALS, how is the CSF
normal
for diagnosing ALS, can changes be seen on a myelogram
yes
what is Definitive Diagnostic Criteria
UMN + LMN signs in three or more regions
for definitive diagnostic criteria, are structural lesions included
NOPE
how long may it take for a definitive diagnosis of ALS
15-21 months
for ALS, EMG changes may not be seen for…
6-12 months
is there a known cure for ALS
nope
what is the only FDA approved drug for ALS
Riluzole
what does Riluzole do
inhibit glutamate
what does Riluzole do relative to ALS
slow progression by 10-15%
riluzole increases survival of patients with ALS by…
3 months
what is the expected survival after diagnosis of ALS
2-5 years
what is the most common cause of death from ALS
respiratory compromise (pneumonia)
what may prolong life for someone with ALS
PEG and ventilation
what is alzheimer’s disease
progressive disease process typically causing dementia
alzheimer’s disease is characterized by slow decline/changes in… (5)
- memory
- language
- visuospatial skills
- personality
- cognition
the rate of alzheimer’s disease increases with…
each decade in life (more common in 90 year olds than 80, than 70, than 60, etc.)
what is the most common cause of dementia
alzheimer disease
who is most at risk for alzheimers
females over 80
what is alzheimers linked to (4)
- HTN
- DM
- obesity
- increased cholesterol
what compound helps prevent alzheimers
omega-3 (fish)
is there a definitive cause of alzheimers
no
in simple terms, what is alzheimers
breakdown in processes necessary to sustain brain cells
what are the neuropathologic hallmarks for alzheimers (4)
- amyloid plaques
- neurofibrillary targets
- decrease acetlycholine activity
- targets corticocorticol and hippocampal cells
what is the significance of Tau proteins and alzheimers
- they transport things from cell body to terminal (keeps cell healthy)
- when inflammatory process happens, Tau proteins change shape and stop working
early symptoms in alzheimers (4)
- mild memory loss
- mild cognitive impairments
- subtle personality changes
- diminished judgement/decision making
what are the later symptoms in alzheimers (5)
- impaired recall of events/memories
- language deficits
- motor changes
- disorders of sleep, eating, and sexual behavior
- mute and bedridden
for alzheimer’s disease, how are movements
slow
for alzheimer’s disease, describe their gait
halting gait
for alzheimer’s disease, where is weakness (general)
generalized
for alzheimer’s disease, there is an increased risk of…
falls (30%)
for alzheimer’s disease, what happens to postural reflexes
diminished
for alzheimer’s disease, what happens to awareness of self in space
reduced
for alzheimer’s disease, what happens to the ability to move around obstacles
reduced
for warning signs of alzheimer’s, what happens regarding memory loss
recent memory loss which affects job performance
for warning signs of alzheimer’s, there is difficulty in performing…
familiar tasks
for warning signs of alzheimer’s, there are problems with…
language
for warning signs of alzheimer’s, there is ____
disorientation
for warning signs of alzheimer’s, there is decreased…
judgement
for warning signs of alzheimer’s, there are abstract…
thinking difficulties
for warning signs of alzheimer’s, what happens to “things”
misplacing things
for warning signs of alzheimer’s, there are changes in…
mood or behavior
for warning signs of alzheimer’s, there are ____ changes
personality
for warning signs of alzheimer’s, there is a loss of…
initiative
for alzheimer’s disease, what should be ruled out…
reversible cause of dementia
what are reversible causes of dementia (3)
- blood count
- chest radiography
- general neuro exam
what is the general progression of alzheimer’s
continuous gradual decline without abrupt changes
what examination can be used for alzheimer’s
Mini-Mental State Examination (MMSE)
what test is a potential indicator of alzheimer’s
clock drawing
what types of imaging can be used for Alzheimer’s
MRI and CT
what can be noted on MRI and CT for Alzheimer’s (2)
- atrophy
2. neurofibrillary tangles
what isn’t well imaged on MRI and CT for alzheimers
amyloid plaques
where are amyloid plaques found for alzheimers later
autopsy
what does a score of 24-30 on the mini-mental state examination
normal
what does a score of 18-23 on the mini-mental state examination
mild cognitive impairment
what does a score of 0-17 on the mini-mental state examination
severe cognitive impairment
is there a cure for alzheimers
no
what are two methods of drug therapy for alzheimers
- cholinesterase inhibitor
2. NMDA receptor targeted therapy
is there a disease modifying treatment for Alzheimers
no, only symptoms
does regular physical activity help people with alzheimers
yes
when is the onset to death for alzheimer disease
7-11 years
what is death secondary to for alzheimers
dehydration or infection
what was the school sisters of notre dame study
yearly tests of nuns with alzheimers
what was the correlation between the nuns and alzheimers
- increase idea density = decrease alzheimers
2. increase head size and education = decrease AD
what is Parkinson’s disease
chronic, progressive neurodegenerative disorder
Parkinson’s disease is the loss of…
midbrain dopamine neurons
cardinal features of parkinsons (4)
- rigidity
- tremor
- bradykinesia
- postural instability
how many people in the US with parkinsons
1.5 mil
is parkinsons more diagnosed younger or older
older
age of onset for parkinsons is generally…
50-75 years
what is considered “young onset” of parkinsons
diagnosed before 40 (10%)
is there a known cause of parkinsons
no it is idiopathic
if there was a genetic link, what population is diagnosed
the younger population
for parkinsons disease, ____ exposure is a risk factor
toxic
for parkinsons disease, ______ infection is a risk factor
prceipitating
for parkinsons disease, exercise may increase/decrease? risk
decrease
for parkinsons disease, smoking may increase/decrease? risk
decrease
for parkinsons disease, higher education may increase/decrease? risk
increase
parkinsons is the degredation of ______ of the _______
substantia nigra of the basal ganglia
before clinical features of parkinsons, there is a ___-___% loss
70-80%
in healthy individuals, dopamine acts on both the _____ and ____ pathways of the basal ganglia to reduce inhibitory outflow of the basal ganglia to the thalamus
direct and indirect
when dopamine is absent, there is more ____ of the thalamus and thus _____ activation of the cortex
- more inhibition of the thalamus
- less activation of the cortex
decrease dopamine = increase/decrease? movement generation
decrease movement generation
Two pathways for signals from the cortex to the striatum
- direct
2. indirect
what is the direct pathway
decrease tonic inhibition and allow movement
what is the indirect pathway
increase inhibitory flow from the basal ganglia
is D1 or D2 associated with the direct pathway
D1
is D1 or D2 associated with the indirect pathway
D2
is D1 excitatory or inhibitory
excitatory
is D2 excitatory or inhibitory
inhibitory
for the net result of Parkinsons, what is the inhibitory outflow from the BG like
abnormally high
for the net result of Parkinsons, is thalamic activation of UMNs in the cortex more or less likely to occur
less likely
for the net result of Parkinsons, there is ____ of movement
poverty
4 cardinal features of parkinsons
- tremor
- rigidity
- bradykinesia
- postural instability
for people with parkinsons, what kind of gait is noted
festinating
key features of parkinsons
- stooped/kyphotic posture
- akinesia/freezing
- dual task difficulty
- altered sense of smell
- weakness
- fatigue
- dementia
- depression
is there a single definitive test for parkinsons
no
diagnosis of parkinsons is made on the basis of…
history and clinical exam
how many of the 4 cardinal features need to be present for the diagnosis of parkinsons
2 of 4
are parkinson-plus syndromes present in the diagnosis of parkisons
no they should be excluded
what are parkinson-plus syndromes (2)
- bilateral/symmetrical extrapyramidal signs
2. do not respond to l-dopa or dopamine agonists
wat type of imaging can identify dopamine deficiency
functional MRI with chemical markers
what is a surgical-like technique used for parkinsons
deep brain stimulation
what parts of the brain are involved in deep brain stimulation (3)
- thalamus
- globus pallidus internal segment
- subthalamic nucleus
do clinical manifestations for parkinsons progressively worsen
yes
what drug for parkinsons loses its effectiveness with time
l-dopa
if parkinsons is developed in the 50s/60s, is lifespan significantly reduced
no
people with parkinsons useually die due to…
infection or complication of immobility
