Movement Disorders Flashcards

1
Q

what is the definition of a movement disorder?

A

neurological syndromes in which there’s excess movement (hyperkinesia) or a paucity of voluntary/involuntary movement (hypokinesia) unrelated to weakness or spasticity

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2
Q

movement disorders are typically linked to what two areas of the brain?

A

BG and cerebellum

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3
Q

what is the location of the BG?

A

subcortical

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4
Q

what is the location of the cerebellum?

A

subcortical

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5
Q

what is the role of the BG? what would a lesion cause?

A

the role of the BG is motor control

a lesion to the BG would cause movt disorder

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6
Q

what is the role of the cerebellum? what would a lesion cause?

A

the role of the cerebellum is motor control

a lesion to the cerebellum would cause movt disorder

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7
Q

the BG is part of the ____ system

A

motor

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8
Q

the cerebellum is part of the ____ system

A

motor

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9
Q

does the BG have any direct connections to the SC?

A

nope

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10
Q

does the cerebellum have any direct connections to the SC?

A

nope again

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11
Q

would a unilateral lesion to the BG cause contralateral or ipsilateral symptoms?

A

contralateral

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12
Q

would a unilateral lesion to the cerebellum cause contralateral or ipsilateral symptoms?

A

ipsilateral

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13
Q

what are the BG effects on tone?

A

rigidity

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14
Q

what are the cerebellar effects on tone?

A

hypotonia

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15
Q

what are the BG effects on movt?

A

decreased movt: bradykinesia, hypokinesia, akinesia

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16
Q

what are the cerebellar effects on movt?

A

increased movt: ataxia, dysmetria, dysdiadochokinesia

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17
Q

what is the umbrella term for hypokinetic conditions that present w/tremors, bradykinesia, postural instability, rigidity?

A

parkinsonism

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18
Q

what conditions are included under the umbrella term parkinsonism?

A

idiopathic PD

secondary parkinsonism

atypical Parkinson’s (PD+)

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19
Q

what conditions fall under PD+?

A

MSA (multiple systems atrophy)

PSP (progressive supranuclear palsy)

CBD (corticobasal degeneration)

LBD (Lewy body dementia)

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20
Q

what part of the brain is impacted by PD?

A

the BG, specifically the substantia nigra

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21
Q

what does TRAP stand for?

A

Tremors

Rigidity

Akinesia

Postural instability

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22
Q

how is PD often diagnosed?

A

through observation and response to Levodopa

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23
Q

what is the most common med to treat PD?

A

Sinemet (carbadopa/Levodopa)

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24
Q

what are the 3 motor loop pathways required for normal functioning?

A

hyperdirect (stop) pathway

“go” pathway

“no-go” pathway

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25
which pathway DIRECTLY allows movt to occur?
the "go" pathway
26
which pathway INDIRECTLY stops unwanted movt from occuring?
the "no-go" pathway
27
t/f: normal movt requires normal activity in all 3 motor pathways
true
28
BG disorders affect the ____ of the fxn of the 3 motor pathways
balance
29
what are the consequences of degradation of the D1 receptor of the SNc?
loss of dopamine reduces the activity of the motor areas of the thalamus, resulting in decreased voluntary movt increased inhibition of the PPN disinhibited the reticulospinal tracts, producing excessive contraction of postural muscles and rigidity increased inhibition of the MLR reduces its activity and decreased out to the SPGs resulting in freezing and destination of gait
30
idiopathic PD is more common in males or females?
males
31
what is the fastest growing neurodegenerative disease?
idiopathic PD
32
what are the primary impairments in PD?
Tremor Rigidity Akinesia Postural instability gait dysfunction (PIGD)
33
tremors occur in what % of those with PD?
70%
34
what is akathisia?
inability to remain still
35
what types of rigidity are common in PD?
cogwheel and lead pipe
36
what is rigidity?
involuntary increase in muscle tone to PROM
37
what is bradykinesia/hypokinesia/ akinesia?
decreased movt speed, amplitude leads to cessation/freezing of movt
38
what is the risk with postural instability gait dysfunction?
decreased postural stability can lead to falls
39
t/f: PD is usually a dx of exclusion
true
40
t/f: there is a diagnostic test that can ID PD w/o additional evaluation of medical hx and neuro exam
false
41
a formal dx of PD must include ____ as a notable symptom
bradykinesia
42
PD dx must include bradykinesia in addition to one or more of what 3 symptoms?
resting tremor rigidity of arm, leg, or trunk trouble with balance or falls
43
what tests can be included in PD dx?
DAT (dopamine transporter) scan genetic testing alpha-synuclein test
44
what is a dopamine transporter (DAT) scan?
an approved imaging technique for distinguishing essential tremor from PD detects dopamine deficits w/in the substantia nigra
45
t/f: DAT scan is NOT commonly used in isolation for dx of PD
true
46
t/f: PD is linked to a gene that guarantees development of PD
false, PD is NOT linked to a single gene that guarantees development of PD s/s
47
t/f: genetic testing may help estimate risks of developing PD, however not diagnostics
true
48
what is alpha synuclein?
a presynaptic neuronal enzyme
49
what is an alpha synuclein test?
a test that can detect clumps of alpha-synuclein in the CSF to dx PD can detect symptoms of PD b4 they appear
50
t/f: the alpha-synuclein test is diagnostic on its own
false
51
what is the UPDRS?
a tool for evaluation of interventions in PD that may be used at baseline during initial dx to evaluate progress and response to meds
52
what are the 4 subsections of the UPDRS?
1) mentation, behavior, mood 2) ADLs 3) motor manifestations 4) complications of therapy
53
the UPDRS is used in what settings primarily?
in the physicians office
54
what are the meds used to treat PD?
Levodopa and dopamine agonists (Sinemet and Pramipexol) COMT inhibitor MAO-B inhibitor (Rasagiline) rescue meds (injection or inhalation powder)
55
what is the gold standard for treating PD?
Levodopa
56
t/f: Levodopa is a precursor to dopamine and can cross the blood barrier
true
57
what % of PWPD suffer from motor complications, fluctuation, and/or dyskinesias after 5 years of treatment
about 50%
58
long term use of Levodopa and/or progression of PD can lead to what?
"off" time at which symptoms occur most commonly bw doses
59
what are the side effects of levodopa?
motor complications neuropsychiatric disturbances
60
what are the motor complications associated with Levodopa?
dyskinesias dystonias
61
do dykinesias or dystonias result from too much meds?
dyskinesias
62
do dykinesias or dystonias result from not enough meds?
dystonias
63
what is dyskinesia?
faster, more rhythmic movts uncontrolled involuntary movt that may be present as fidgeting, writhing, wriggling, head bobbing, or body swaying
64
what is dystonia?
painful involuntary muscle contractions that force the body into abnormal postures (ie toe curling)
65
what are the neuropsychiatric disturbances associated with Levodopa use?
hallucinations psychosis confusion
66
t/f: the longer someone is on Levodopa, the smaller the therapeutic window gets
true
67
what is an invasive treatment option for PD?
deep brain stimulation
68
what is a non-invasive treatment option for PD?
focused US
69
what is deep brain stimulation?
electrodes are placed in the subthalamic nucleus (STN) or globus pallidus internal (GPi) and connected to a neurostimulator inferior to the clavicle
70
t/f: DBS can reduce/control rigidity, bradykinesia, tremor, and dyskinesia
true
71
who are candidates for DBS?
those who respond to Levodopa/carbadopa those with PD dx>5 years those with severe motor impairments/fluctuations those with significant issues of "off" times
72
t/f: pt satisfaction with DBS is high
true
73
are the effects of DBS fast or slow?
slow
74
t/f: the electrodes in DBS can be turned on/off
true
75
what is focused US?
MRI used to direct US to target brain location typically to decrease motor symptoms immediately
76
does focused US typically work fast or slow?
fast
77
t/f: DBS is irreversible and permanent
true
78
is focused US used for one or both sides of the brain at a time?
one side at a time
79
t/f: focused US only helps motor symptoms on one side of the body
true
80
what are the side effects of focused US?
numbness/tingling, unsteadiness, gait dysfunctions, muscle weakness, speech abnormalities
81
what is Hoehn and Yahr stage 1 PD?
one side of the body is effected
82
what is Hoehn and Yahr stage 2 PD?
both sides affected but balance remains intact
83
what is Hoehn and Yahr stage 3 PD?
impaired balance, fxning intact
84
what is Hoehn and Yahr stage 4 PD?
walking and standing are difficult without help
85
what is Hoehn and Yahr stage 5 PD?
WC bound or bed-ridden
86
what are the motor impairments of PD?
bradykinesia limited facial expressions (facial masking) speech deficits (soft and monotone, slow/fast speech, poor awareness of volume) tremor micrographia (small writing) visual impairments swallowing deficits (often leads to drooling) rigidity dystonia postural instability, ankle PF, toe curling
87
t/f: the pt isn't always aware of their deficits in PD
true
88
when does facial rigidity usually start?
stage 4
89
what are the 3 components of balance?
visual, vestibular, and somatosensory
90
is anticipatory balance feedback or feedforward?
feedforward
91
is reactive balance feedback or feedforward?
feedback
92
what is a good test for reactive and anticipatory balance in PD?
miniBEST
93
what are the balance deficits in PD?
decreased postural extension and awareness decreased unconscious proprioception decreased arm swing and trunk rotation poor anticipatory and reactive postural responses retropulsion requires more steps to recover from a loss of balance
94
why is there poor anticipatory and reactive postural control in PD?
inadequate "go" pathway
95
what are the gait dysfunctions associated with PD?
freezing of gait (FoG) festination of gait (FSG) retropulsion
96
what is freezing of gait (FoG)?
absence/marked reduction of forward progression despite intention to walk difficulty adjusting the speed or size of steps quickly enough to changing environment
97
t/f: FoG makes pts more likely to fall
true
98
when does FoG typically occur?
doorways, crowds, small spaces, w/turns, w/distractions/dual-tasking standing and trying to initiate steps immediately trying to step with weight still on the forefoot
99
what is festination of gait (FSG)?
gait with increased step cadence
100
when does festination of gait (FSG) typically occur?
with complex motor tasks and dual tasking when reaching too far outside of BOS with increased flexed posture after being told to "hurry up" when reaching for a chair too soon b4 sitting
101
what is retropulsion?
involuntary steps backwards
102
what typically trigger retropulsion?
posterior weight shift or LOB (loss of balance)
103
when does retropulsion have a tendency to occur?
during "off" times
104
will neurogenic OH respond to hydration, position change, and muscle activation?
nope, you have to adjust meds
105
what are the mental impairments associated with PD?
mood disorders, depression, anxiety, psychosis, dementia, apathy, impaired executive functioning and attention, bradyphrenia (slow processing)
106
what are the sensory functions associated with PD?
olfactory impairments (anosmia) pain paresthesia
107
why are olfactory functions effected in PD?
bc olfactory cells have dopaminergic neurons
108
what is one of the most limiting non-motor effects of PD for PTs?
OH (CV or neurogenic)
109
what are the genitourinary and reproductive problems associated with PD?
urinary problems and constipation
110
what are the issues with functions of skin and related structures associated with PD?
restless leg syndrome excessive sweating sleep disturbances
111
what are the clinical considerations for PD?
reduced ROM, endurance, and strength poor posture muscle atrophy neurogenic orthostasis on/off times freezing falls/fear of falls
112
OH is drop in SBP > ___ mmHg or DBP >___ mmHg
20, 10
113
t/f: progression of PD should be slow and fast progression is a sign of PD+
true
114
what are the progression risks that make us think of PD+ rather than PD?
rapid progression of gait impairment, requiring WC w/in 5 years of onset poor/absent response to dopaminergic therapy early bulbar dysfunction (<5 years after onset) inspiratory respiratory dysfxn (stridor, frequent sighs) severe autonomic failure (<5 years after onset) recurrent (>1/year) falls from imbalance w/in 3 yrs of onset anterocollis (flexed forward head) or limb contractures within 10 years of onset absences of common non-motor features (sleep dysfxn, RBD/PLMS, daytime somnelence, autonomic dysfxn, hyposmia, mood disturbances) otherwise unexplained pyramidal tract signs (Babinski, hyperreflexia, hypertonia) BL symmetric parkinsonism
115
would PD or PD+ have limited response to dopamine therapy?
PD+
116
is PD or PD+ more likely to start BL?
PD+
117
is PD or PD+ more likely to have psych involvement?
PD+
118
which has a more rapid progression, PD or PD+?
PD+
119
what are the symptoms of Parkinson's type MSA (MSA-P)?
bradykinesia tremors impaired balance autonomic NS dysfxn
120
what are the symptoms of cerebellar type MSA (MSA-C)?
nystagmus trunk and limb ataxia gait ataxia ataxic dysarthria dysphagia respiratory impairment
121
what are the corticospinal (pyramidal) signs of PD+?
pisa syndrome (excessive leaning to one side) anterocollis (flexed forward head) or limb contractures (+) Babinski sign spasticity stridor
122
what are the autonomic dysfxns associated with PD+?
unexplained difficulty voiding and/or urge urinary incontinence neurogenic OH
123
what is MSA?
multiple systems atrophy degradation of multiple areas of the brain
124
what is the life expectancy of MSA following dx?
7-9 years
125
when is the average age of onset of MSA?
40s-50s
126
what is corticobasal ganglionic degeneration (CBD)?
frontotemporal disorder tauopathy
127
what is a frontotemporal disorder?
degeneration of the frontal and temporal lobes
128
what is a tauopathy?
accumulation of Tau proteins in the brain
129
what are the symptoms of CBD?
dystonias (UE>LE or cervical) myoclonus limb apraxia (ideomotor) aphasia alien limb syndrome bradykinesia, limb rigidity, postural instability, tremors personality changes and inappropriate behavior in later stages
130
are males or females more affected by CBD?
females
131
what is the average age of onset of CBD?
40-70 yo
132
what is the life expectancy of CBD after dx?
6-8 years
133
what is progressive supranuclear palsy (PSP)?
frontotemporal disorder tauopathy
134
what are the 4 fxnal domains of PSP?
1) oculomotor dysfxn 2) postural instability 3) akinesia 4) cognitive dysfxn
135
what is the oculomotor dysfxn associated with PSP?
slow eye movt vertical supranuclear gaze palsy
136
bc pts with PSP can't move their eyes themselves up/down what may we see?
compensation by moving the entire head
137
what fxnal task is often difficult for pts with PSP?
stairs
138
what are the postural instabilities associated with PSP?
spontaneous loss of balance and falls retropulsion
139
what are additional key symptoms of PSP?
blepharospasm (involuntary blinking/twitching of the eyelids) and eyelid opening apraxia early onset of falling (PSP>MSA) early onset dysphagia and dysarthria
140
are more males or females affected by PSP?
males
141
what is the life expectancy after onset of PSP?
6-9 years
142
what is Lewy body dementia?
PD like symptoms following dementia symptoms
143
what are the PD-like symptoms in Lewy body dementia?
bradykinesia, rigidity/stiffness, shuffling gait, tremors
144
what are the cognitive changes in Lewy body dementia?
dementia (b4 onset of PD symptoms) fluctuations w/concentration, alertness, attention, and wakefulness
145
what are the autonomic dysnfxns associated with Lewy body dementia?
temp and BP irrregulation urinary incontinence constipation sexual dysnfxn
146
what are the behavioral changes associated with Lewy body dementia?
hallucinations delusion mood changes (anxiety, depression, apathy)
147
what are the sleep disorder associated with Lewy body dementia?
REM sleep behavior disorders excessive daytime sleepiness insomnia restless leg syndrome
148
what are the changes associated with Lewy body dementia?
cognitive changes movt changes autonomic changes behavioral changes sleep disorders
149
hallucinations, agitation, and dementia symptoms b4 PD symptoms are Hallmark signs of what?
Lewy body dementia
150
what is the life expectancy of pts with Lewy body dementia after onset?
2-9 yrs
151
what % of people with dementia have Lewy body dementia?
5%
152
t/f: hyperkinetic disorders results from an overactive go pathway
true
153
in hyperkinetic disorders the GPi is ....
less inhibited
154
bc the GPi is less inhibited in hyperkinetic disorders, the motor thalamus is...
more facilitated
155
what is the result of the GPi being less inhibited and the motor thalamus being more facilitated?
involuntary movt
156
in hyperkinetic disorders, the corticospinal tracts are ...
more facilitated
157
what is the result of the corticospinal tracts being more facilitated?
increased involuntary movt (hyperkinesia like dyskinesia, chorea)
158
in hyperkinetic disorders, the PPN is...
less inhibited
159
in hyperkinetic disorders, bc the PPN is less inhibited, the reticulospinal tracts are ...
more inhibited
160
what is the result of inhibited reticulospinal tracts?
decreased activation (stability) or the trunk
161
are dystonias intermittent or continuous?
can be either
162
what are the types of dystonias?
focal segmental primary/isolated secondary/acquired paroxysmal functional
163
are dystonias often seen as a secondary incidence or an isolated/primary issue?
secondary
164
what are common types of dyskinesias?
athetosis chorea myoclonus Levodopa induced dyskinesia (LID) ballism
165
dyskinesias are often seen in what two diseases?
HD and MS
166
what is Huntington's disease (HD)?
an inherited autosomal dominant condition that results in degeneration of the caudate nucleus and putamen of the BG
167
what are the motor symptoms of HD?
chorea dystonia bradykinesia/akinesia motor impersistance (inability to stay still) difficulty performing RAMs (rapid alternating movts) difficulty sequencing movt
168
what are the psychiatric symptoms of HD?
depression anxiety
169
what are the cognitive symptoms of HD?
memory loss executive fxn issues verbal fluency issues slowed info processing
170
what are the cardiac symptoms of HD?
bradycardia elevated BP cardiac arrhythmias HF
171
is PD primarily a mobility or stability problem?
mobility problem
172
is HD primarily a mobility or stability problem?
stability problem
173
t/f: the UPDRS is a good way to document info and use the language of the neurologist so they will easily understand and quantify the severity of the disease
true
174
individuals with HD have an average survival of how many years following onset of symptoms
15-20 years
175
how old are pts usually when they are diagnosed with HD?
30-50 yo
176
what is the prevalence of HD?
about 4.1-8.4 per 100,000 individuals in the US
177
what are the 5 stages of HD?
preclinical stage early stage middle stage late stage end-of-life stage
178
what stage of HD is characterized by mild symptoms, but no dx of HD from the doctor?
pre-clinical stage
179
what stage of HD is characterized by symptoms becoming noticeable enough for diagnosis?
early stage
180
what stage of HD is characterized by individuals being unable to carry out most activities on their own?
middle stage
181
what stage of HD is characterized by pts requiring assistance in all areas of life and becoming bedridden?
late stage
182
what stage of HD is characterized by pts becoming confined to bed completely and dependent on others for all their activities, needing help to live the last few months/years of their life peacefully and in the best way they can?
end-of-life stage
183
what are the super 6 tests and measures?
6MWT Berg 5xSTS FGA gait speed ABC
184
what is the MDC for the 6MWT?
82 m
185
what is the MDC for the Berg?
5 points
186
what is the MDC for the 5xSTS?
.6 sec
187
what is the MDC for the FGA?
4 points
188
what is the MDC for gait speed?
.18 m/s
189
what is the MDC for the ABC?
13
190
t/f: PD and HD have a multisystem presentation involving motor, non-motor, and secondary impairments
true