Degenerative Disorders of the Central Nervous System PART 1 (start-Parkinson's) Flashcards
(155 cards)
1
Q
what does ALS stand for
A
amyotrophic lateral sclerosis
2
Q
what is amyotrophy
A
muscle atrophy (due to peripheral nerve changes)
3
Q
what does the “lateral” part of ALS mean
A
motor neurons in the lateral aspect of the spinal cord, brainstem, and cerebral cortex involved
4
Q
what does “sclerosis” part of ALS mean
A
degeneration and scarring
5
Q
what is the most common form of adult onset progressive motor neuron disorder
A
ALS
6
Q
how many new cases of ALS/year
A
5000
7
Q
what is the most physically devastating of the neurodegenerative disorders
A
ALS
8
Q
two types of ALS
A
- sporadic
2. familial
9
Q
what type of ALS is more common
A
sporadic
10
Q
what is the cause of sporadic ALS
A
unknown
11
Q
what type of genetic disorder is familial ALS
A
autosomal dominant
12
Q
familial ALS: early or late onset
A
early onset
13
Q
what gene is mutated in familial ALS
A
superoxide dismutase gene mutation
14
Q
are males or females more affected by ALS
A
males
15
Q
what disease prevents ALS
A
polio
16
Q
general pathogeneses of ALS (2)
A
- destruction of UMN
2. destruction of LMN
17
Q
area affected in UMN destruction of ALS
A
cerebral cortex
18
Q
what two tracts are involved in UMN involvement in ALS
A
- corticospinal tract
2. corticobulbar tract
19
Q
where are the alpha motor neurons that are affected in LMN involvement in ALS
A
anterior horn of cord and cranial nerve nuclei in brainstem
20
Q
possible causes of ALS (2)
A
- excitotoxicity
2. oxidative damage
21
Q
how does excitotoxicity play into ALS
A
excess glutamate
22
Q
how does oxidative damage play into ALS
A
free radicals
23
Q
are LMN symptoms symmetrical or asymmetrical
A
asymmetrical
24
Q
are LMN symptoms proximal or distal
A
distal
25
LMN symptoms involve what part of the body and what motion is weak
cervical extensor weakness (head drop)
26
are LMN symptoms hypo or hyperreflexive
hyporeflexive
27
are LMN symptoms hypo or hypertonic
hypotonic
28
are LMN symptoms atrophy or hypertrophy
atrophy
29
muscle ____ are noted with LMN symtpoms
cramps
30
what else may be present with LMN symptoms
fasciculations
31
are UMN symptoms spastic or nah
spastic
32
are UMN symptoms hypo or hyperreflexia
hyperreflexia
33
what reflexes are present with UMN symptoms (3)
clonus, babinski, hoffmann
34
are UMN symptoms muscles strong or weak
weak
35
as the disease progresses, UMN/LMN? symptoms may decrease
UMN
36
for ALS, what other kinds of symptoms may be present
bulbar
37
with UMN involvements, what palsy is present in ALS
Pseudobulbar Palsy
38
what is present with Pseudobulbar Palsy
spasticity
39
with LMN involvements, what palsy is present with ALS
Bulbar Palsy
40
what is present with Bulbar Palsy
flaccid
41
presentation of bulbar symptoms (4)
1. dysarthria
2. dysphagia
3. sialorrhea
4. pseudobulbar affect (UMN)
42
preserved areas in ALS (4)
1. eye movements
2. posterior columns
3. bowel and bladder function
4. cognition (50% preserved)
43
early signs of ALS (3)
1. insidious asymmetrical weakness of distal aspects of one limb
2. cramping with volitional movement - early morning stiffness
3. muscle fasciculations - spontaneous twitching of muscle fibers
44
late manifestations of ALS (2)
1. respiratory complications --> respiratory failure
| 2. oral motor complications --> risk of aspirations
45
for diagnosing ALS, what can EMG reveal (2)
1. fibrilations
| 2. fasciculations
46
for diagnosing ALS, what can muscle biopsy reveal
denervation atrophy
47
for diagnosing ALS, what can muscle enzymes reveal
CPK levels elevated
48
for diagnosing ALS, how is the CSF
normal
49
for diagnosing ALS, can changes be seen on a myelogram
yes
50
what is Definitive Diagnostic Criteria
UMN + LMN signs in three or more regions
51
for definitive diagnostic criteria, are structural lesions included
NOPE
52
how long may it take for a definitive diagnosis of ALS
15-21 months
53
for ALS, EMG changes may not be seen for...
6-12 months
54
is there a known cure for ALS
nope
55
what is the only FDA approved drug for ALS
Riluzole
56
what does Riluzole do
inhibit glutamate
57
what does Riluzole do relative to ALS
slow progression by 10-15%
58
riluzole increases survival of patients with ALS by...
3 months
59
what is the expected survival after diagnosis of ALS
2-5 years
60
what is the most common cause of death from ALS
respiratory compromise (pneumonia)
61
what may prolong life for someone with ALS
PEG and ventilation
62
what is alzheimer's disease
progressive disease process typically causing dementia
63
alzheimer's disease is characterized by slow decline/changes in... (5)
1. memory
2. language
3. visuospatial skills
4. personality
5. cognition
64
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.)
65
what is the most common cause of dementia
alzheimer disease
66
who is most at risk for alzheimers
females over 80
67
what is alzheimers linked to (4)
1. HTN
2. DM
3. obesity
4. increased cholesterol
68
what compound helps prevent alzheimers
omega-3 (fish)
69
is there a definitive cause of alzheimers
no
70
in simple terms, what is alzheimers
breakdown in processes necessary to sustain brain cells
71
what are the neuropathologic hallmarks for alzheimers (4)
1. amyloid plaques
2. neurofibrillary targets
3. decrease acetlycholine activity
4. targets corticocorticol and hippocampal cells
72
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
73
early symptoms in alzheimers (4)
1. mild memory loss
2. mild cognitive impairments
3. subtle personality changes
4. diminished judgement/decision making
74
what are the later symptoms in alzheimers (5)
1. impaired recall of events/memories
2. language deficits
3. motor changes
4. disorders of sleep, eating, and sexual behavior
5. mute and bedridden
75
for alzheimer's disease, how are movements
slow
76
for alzheimer's disease, describe their gait
halting gait
77
for alzheimer's disease, where is weakness (general)
generalized
78
for alzheimer's disease, there is an increased risk of...
falls (30%)
79
for alzheimer's disease, what happens to postural reflexes
diminished
80
for alzheimer's disease, what happens to awareness of self in space
reduced
81
for alzheimer's disease, what happens to the ability to move around obstacles
reduced
82
for warning signs of alzheimer's, what happens regarding memory loss
recent memory loss which affects job performance
83
for warning signs of alzheimer's, there is difficulty in performing...
familiar tasks
84
for warning signs of alzheimer's, there are problems with...
language
85
for warning signs of alzheimer's, there is ____
disorientation
86
for warning signs of alzheimer's, there is decreased...
judgement
87
for warning signs of alzheimer's, there are abstract...
thinking difficulties
88
for warning signs of alzheimer's, what happens to "things"
misplacing things
89
for warning signs of alzheimer's, there are changes in...
mood or behavior
90
for warning signs of alzheimer's, there are ____ changes
personality
91
for warning signs of alzheimer's, there is a loss of...
initiative
92
for alzheimer's disease, what should be ruled out...
reversible cause of dementia
93
what are reversible causes of dementia (3)
1. blood count
2. chest radiography
3. general neuro exam
94
what is the general progression of alzheimer's
continuous gradual decline without abrupt changes
95
what examination can be used for alzheimer's
Mini-Mental State Examination (MMSE)
96
what test is a potential indicator of alzheimer's
clock drawing
97
what types of imaging can be used for Alzheimer's
MRI and CT
98
what can be noted on MRI and CT for Alzheimer's (2)
1. atrophy
| 2. neurofibrillary tangles
99
what isn't well imaged on MRI and CT for alzheimers
amyloid plaques
100
where are amyloid plaques found for alzheimers later
autopsy
101
what does a score of 24-30 on the mini-mental state examination
normal
102
what does a score of 18-23 on the mini-mental state examination
mild cognitive impairment
103
what does a score of 0-17 on the mini-mental state examination
severe cognitive impairment
104
is there a cure for alzheimers
no
105
what are two methods of drug therapy for alzheimers
1. cholinesterase inhibitor
| 2. NMDA receptor targeted therapy
106
is there a disease modifying treatment for Alzheimers
no, only symptoms
107
does regular physical activity help people with alzheimers
yes
108
when is the onset to death for alzheimer disease
7-11 years
109
what is death secondary to for alzheimers
dehydration or infection
110
what was the school sisters of notre dame study
yearly tests of nuns with alzheimers
111
what was the correlation between the nuns and alzheimers
1. increase idea density = decrease alzheimers
| 2. increase head size and education = decrease AD
112
what is Parkinson's disease
chronic, progressive neurodegenerative disorder
113
Parkinson's disease is the loss of...
midbrain dopamine neurons
114
cardinal features of parkinsons (4)
1. rigidity
2. tremor
3. bradykinesia
4. postural instability
115
how many people in the US with parkinsons
1.5 mil
116
is parkinsons more diagnosed younger or older
older
117
age of onset for parkinsons is generally...
50-75 years
118
what is considered "young onset" of parkinsons
diagnosed before 40 (10%)
119
is there a known cause of parkinsons
no it is idiopathic
120
if there was a genetic link, what population is diagnosed
the younger population
121
for parkinsons disease, ____ exposure is a risk factor
toxic
122
for parkinsons disease, ______ infection is a risk factor
prceipitating
123
for parkinsons disease, exercise may increase/decrease? risk
decrease
124
for parkinsons disease, smoking may increase/decrease? risk
decrease
125
for parkinsons disease, higher education may increase/decrease? risk
increase
126
parkinsons is the degredation of ______ of the _______
substantia nigra of the basal ganglia
127
before clinical features of parkinsons, there is a ___-___% loss
70-80%
128
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
129
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
130
decrease dopamine = increase/decrease? movement generation
decrease movement generation
131
Two pathways for signals from the cortex to the striatum
1. direct
| 2. indirect
132
what is the direct pathway
decrease tonic inhibition and allow movement
133
what is the indirect pathway
increase inhibitory flow from the basal ganglia
134
is D1 or D2 associated with the direct pathway
D1
135
is D1 or D2 associated with the indirect pathway
D2
136
is D1 excitatory or inhibitory
excitatory
137
is D2 excitatory or inhibitory
inhibitory
138
for the net result of Parkinsons, what is the inhibitory outflow from the BG like
abnormally high
139
for the net result of Parkinsons, is thalamic activation of UMNs in the cortex more or less likely to occur
less likely
140
for the net result of Parkinsons, there is ____ of movement
poverty
141
4 cardinal features of parkinsons
1. tremor
2. rigidity
3. bradykinesia
4. postural instability
142
for people with parkinsons, what kind of gait is noted
festinating
143
key features of parkinsons
1. stooped/kyphotic posture
2. akinesia/freezing
3. dual task difficulty
4. altered sense of smell
5. weakness
6. fatigue
7. dementia
8. depression
144
is there a single definitive test for parkinsons
no
145
diagnosis of parkinsons is made on the basis of...
history and clinical exam
146
how many of the 4 cardinal features need to be present for the diagnosis of parkinsons
2 of 4
147
are parkinson-plus syndromes present in the diagnosis of parkisons
no they should be excluded
148
what are parkinson-plus syndromes (2)
1. bilateral/symmetrical extrapyramidal signs
| 2. do not respond to l-dopa or dopamine agonists
149
wat type of imaging can identify dopamine deficiency
functional MRI with chemical markers
150
what is a surgical-like technique used for parkinsons
deep brain stimulation
151
what parts of the brain are involved in deep brain stimulation (3)
1. thalamus
2. globus pallidus internal segment
3. subthalamic nucleus
152
do clinical manifestations for parkinsons progressively worsen
yes
153
what drug for parkinsons loses its effectiveness with time
l-dopa
154
if parkinsons is developed in the 50s/60s, is lifespan significantly reduced
no
155
people with parkinsons useually die due to...
infection or complication of immobility
