Movement Disorders Flashcards
what is the definition of a movement disorder?
neurological syndromes in which there’s excess movement (hyperkinesia) or a paucity of voluntary/involuntary movement (hypokinesia) unrelated to weakness or spasticity
movement disorders are typically linked to what two areas of the brain?
BG and cerebellum
what is the location of the BG?
subcortical
what is the location of the cerebellum?
subcortical
what is the role of the BG? what would a lesion cause?
the role of the BG is motor control
a lesion to the BG would cause movt disorder
what is the role of the cerebellum? what would a lesion cause?
the role of the cerebellum is motor control
a lesion to the cerebellum would cause movt disorder
the BG is part of the ____ system
motor
the cerebellum is part of the ____ system
motor
does the BG have any direct connections to the SC?
nope
does the cerebellum have any direct connections to the SC?
nope again
would a unilateral lesion to the BG cause contralateral or ipsilateral symptoms?
contralateral
would a unilateral lesion to the cerebellum cause contralateral or ipsilateral symptoms?
ipsilateral
what are the BG effects on tone?
rigidity
what are the cerebellar effects on tone?
hypotonia
what are the BG effects on movt?
decreased movt: bradykinesia, hypokinesia, akinesia
what are the cerebellar effects on movt?
increased movt: ataxia, dysmetria, dysdiadochokinesia
what is the umbrella term for hypokinetic conditions that present w/tremors, bradykinesia, postural instability, rigidity?
parkinsonism
what conditions are included under the umbrella term parkinsonism?
idiopathic PD
secondary parkinsonism
atypical Parkinson’s (PD+)
what conditions fall under PD+?
MSA (multiple systems atrophy)
PSP (progressive supranuclear palsy)
CBD (corticobasal degeneration)
LBD (Lewy body dementia)
what part of the brain is impacted by PD?
the BG, specifically the substantia nigra
what does TRAP stand for?
Tremors
Rigidity
Akinesia
Postural instability
how is PD often diagnosed?
through observation and response to Levodopa
what is the most common med to treat PD?
Sinemet (carbadopa/Levodopa)
what are the 3 motor loop pathways required for normal functioning?
hyperdirect (stop) pathway
“go” pathway
“no-go” pathway
which pathway DIRECTLY allows movt to occur?
the “go” pathway
which pathway INDIRECTLY stops unwanted movt from occuring?
the “no-go” pathway
t/f: normal movt requires normal activity in all 3 motor pathways
true
BG disorders affect the ____ of the fxn of the 3 motor pathways
balance
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
idiopathic PD is more common in males or females?
males
what is the fastest growing neurodegenerative disease?
idiopathic PD
what are the primary impairments in PD?
Tremor
Rigidity
Akinesia
Postural instability gait dysfunction (PIGD)
tremors occur in what % of those with PD?
70%
what is akathisia?
inability to remain still
what types of rigidity are common in PD?
cogwheel and lead pipe
what is rigidity?
involuntary increase in muscle tone to PROM
what is bradykinesia/hypokinesia/ akinesia?
decreased movt speed, amplitude leads to cessation/freezing of movt
what is the risk with postural instability gait dysfunction?
decreased postural stability can lead to falls
t/f: PD is usually a dx of exclusion
true
t/f: there is a diagnostic test that can ID PD w/o additional evaluation of medical hx and neuro exam
false
a formal dx of PD must include ____ as a notable symptom
bradykinesia
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
what tests can be included in PD dx?
DAT (dopamine transporter) scan
genetic testing
alpha-synuclein test
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
t/f: DAT scan is NOT commonly used in isolation for dx of PD
true
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
t/f: genetic testing may help estimate risks of developing PD, however not diagnostics
true
what is alpha synuclein?
a presynaptic neuronal enzyme
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
t/f: the alpha-synuclein test is diagnostic on its own
false
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
what are the 4 subsections of the UPDRS?
1) mentation, behavior, mood
2) ADLs
3) motor manifestations
4) complications of therapy
the UPDRS is used in what settings primarily?
in the physicians office
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)
what is the gold standard for treating PD?
Levodopa
t/f: Levodopa is a precursor to dopamine and can cross the blood barrier
true
what % of PWPD suffer from motor complications, fluctuation, and/or dyskinesias after 5 years of treatment
about 50%
long term use of Levodopa and/or progression of PD can lead to what?
“off” time at which symptoms occur most commonly bw doses
what are the side effects of levodopa?
motor complications
neuropsychiatric disturbances
what are the motor complications associated with Levodopa?
dyskinesias
dystonias
do dykinesias or dystonias result from too much meds?
dyskinesias
do dykinesias or dystonias result from not enough meds?
dystonias
what is dyskinesia?
faster, more rhythmic movts
uncontrolled involuntary movt that may be present as fidgeting, writhing, wriggling, head bobbing, or body swaying
what is dystonia?
painful involuntary muscle contractions that force the body into abnormal postures (ie toe curling)
what are the neuropsychiatric disturbances associated with Levodopa use?
hallucinations
psychosis
confusion
t/f: the longer someone is on Levodopa, the smaller the therapeutic window gets
true
what is an invasive treatment option for PD?
deep brain stimulation
what is a non-invasive treatment option for PD?
focused US
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
t/f: DBS can reduce/control rigidity, bradykinesia, tremor, and dyskinesia
true
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
t/f: pt satisfaction with DBS is high
true
are the effects of DBS fast or slow?
slow
t/f: the electrodes in DBS can be turned on/off
true
what is focused US?
MRI used to direct US to target brain location typically to decrease motor symptoms immediately
does focused US typically work fast or slow?
fast
t/f: DBS is irreversible and permanent
true
is focused US used for one or both sides of the brain at a time?
one side at a time
t/f: focused US only helps motor symptoms on one side of the body
true
what are the side effects of focused US?
numbness/tingling, unsteadiness, gait dysfunctions, muscle weakness, speech abnormalities
what is Hoehn and Yahr stage 1 PD?
one side of the body is effected
what is Hoehn and Yahr stage 2 PD?
both sides affected but balance remains intact
what is Hoehn and Yahr stage 3 PD?
impaired balance, fxning intact
what is Hoehn and Yahr stage 4 PD?
walking and standing are difficult without help
what is Hoehn and Yahr stage 5 PD?
WC bound or bed-ridden
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
t/f: the pt isn’t always aware of their deficits in PD
true
when does facial rigidity usually start?
stage 4
what are the 3 components of balance?
visual, vestibular, and somatosensory
is anticipatory balance feedback or feedforward?
feedforward
is reactive balance feedback or feedforward?
feedback
what is a good test for reactive and anticipatory balance in PD?
miniBEST
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
why is there poor anticipatory and reactive postural control in PD?
inadequate “go” pathway
what are the gait dysfunctions associated with PD?
freezing of gait (FoG)
festination of gait (FSG)
retropulsion
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
t/f: FoG makes pts more likely to fall
true
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
what is festination of gait (FSG)?
gait with increased step cadence
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
what is retropulsion?
involuntary steps backwards
what typically trigger retropulsion?
posterior weight shift or LOB (loss of balance)
when does retropulsion have a tendency to occur?
during “off” times
will neurogenic OH respond to hydration, position change, and muscle activation?
nope, you have to adjust meds
what are the mental impairments associated with PD?
mood disorders, depression, anxiety, psychosis, dementia, apathy, impaired executive functioning and attention, bradyphrenia (slow processing)
what are the sensory functions associated with PD?
olfactory impairments (anosmia)
pain
paresthesia
why are olfactory functions effected in PD?
bc olfactory cells have dopaminergic neurons
what is one of the most limiting non-motor effects of PD for PTs?
OH (CV or neurogenic)
what are the genitourinary and reproductive problems associated with PD?
urinary problems and constipation
what are the issues with functions of skin and related structures associated with PD?
restless leg syndrome
excessive sweating
sleep disturbances
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
OH is drop in SBP > ___ mmHg or DBP >___ mmHg
20, 10
t/f: progression of PD should be slow and fast progression is a sign of PD+
true
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
would PD or PD+ have limited response to dopamine therapy?
PD+
is PD or PD+ more likely to start BL?
PD+
is PD or PD+ more likely to have psych involvement?
PD+
which has a more rapid progression, PD or PD+?
PD+
what are the symptoms of Parkinson’s type MSA (MSA-P)?
bradykinesia
tremors
impaired balance
autonomic NS dysfxn
what are the symptoms of cerebellar type MSA (MSA-C)?
nystagmus
trunk and limb ataxia
gait ataxia
ataxic dysarthria
dysphagia
respiratory impairment
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
what are the autonomic dysfxns associated with PD+?
unexplained difficulty voiding and/or urge urinary incontinence
neurogenic OH
what is MSA?
multiple systems atrophy
degradation of multiple areas of the brain
what is the life expectancy of MSA following dx?
7-9 years
when is the average age of onset of MSA?
40s-50s
what is corticobasal ganglionic degeneration (CBD)?
frontotemporal disorder
tauopathy
what is a frontotemporal disorder?
degeneration of the frontal and temporal lobes
what is a tauopathy?
accumulation of Tau proteins in the brain
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
are males or females more affected by CBD?
females
what is the average age of onset of CBD?
40-70 yo
what is the life expectancy of CBD after dx?
6-8 years
what is progressive supranuclear palsy (PSP)?
frontotemporal disorder
tauopathy
what are the 4 fxnal domains of PSP?
1) oculomotor dysfxn
2) postural instability
3) akinesia
4) cognitive dysfxn
what is the oculomotor dysfxn associated with PSP?
slow eye movt
vertical supranuclear gaze palsy
bc pts with PSP can’t move their eyes themselves up/down what may we see?
compensation by moving the entire head
what fxnal task is often difficult for pts with PSP?
stairs
what are the postural instabilities associated with PSP?
spontaneous loss of balance and falls
retropulsion
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
are more males or females affected by PSP?
males
what is the life expectancy after onset of PSP?
6-9 years
what is Lewy body dementia?
PD like symptoms following dementia symptoms
what are the PD-like symptoms in Lewy body dementia?
bradykinesia, rigidity/stiffness, shuffling gait, tremors
what are the cognitive changes in Lewy body dementia?
dementia (b4 onset of PD symptoms)
fluctuations w/concentration, alertness, attention, and wakefulness
what are the autonomic dysnfxns associated with Lewy body dementia?
temp and BP irrregulation
urinary incontinence
constipation
sexual dysnfxn
what are the behavioral changes associated with Lewy body dementia?
hallucinations
delusion
mood changes (anxiety, depression, apathy)
what are the sleep disorder associated with Lewy body dementia?
REM sleep behavior disorders
excessive daytime sleepiness
insomnia
restless leg syndrome
what are the changes associated with Lewy body dementia?
cognitive changes
movt changes
autonomic changes
behavioral changes
sleep disorders
hallucinations, agitation, and dementia symptoms b4 PD symptoms are Hallmark signs of what?
Lewy body dementia
what is the life expectancy of pts with Lewy body dementia after onset?
2-9 yrs
what % of people with dementia have Lewy body dementia?
5%
t/f: hyperkinetic disorders results from an overactive go pathway
true
in hyperkinetic disorders the GPi is ….
less inhibited
bc the GPi is less inhibited in hyperkinetic disorders, the motor thalamus is…
more facilitated
what is the result of the GPi being less inhibited and the motor thalamus being more facilitated?
involuntary movt
in hyperkinetic disorders, the corticospinal tracts are …
more facilitated
what is the result of the corticospinal tracts being more facilitated?
increased involuntary movt (hyperkinesia like dyskinesia, chorea)
in hyperkinetic disorders, the PPN is…
less inhibited
in hyperkinetic disorders, bc the PPN is less inhibited, the reticulospinal tracts are …
more inhibited
what is the result of inhibited reticulospinal tracts?
decreased activation (stability) or the trunk
are dystonias intermittent or continuous?
can be either
what are the types of dystonias?
focal
segmental
primary/isolated
secondary/acquired
paroxysmal
functional
are dystonias often seen as a secondary incidence or an isolated/primary issue?
secondary
what are common types of dyskinesias?
athetosis
chorea
myoclonus
Levodopa induced dyskinesia (LID)
ballism
dyskinesias are often seen in what two diseases?
HD and MS
what is Huntington’s disease (HD)?
an inherited autosomal dominant condition that results in degeneration of the caudate nucleus and putamen of the BG
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
what are the psychiatric symptoms of HD?
depression
anxiety
what are the cognitive symptoms of HD?
memory loss
executive fxn issues
verbal fluency issues
slowed info processing
what are the cardiac symptoms of HD?
bradycardia
elevated BP
cardiac arrhythmias
HF
is PD primarily a mobility or stability problem?
mobility problem
is HD primarily a mobility or stability problem?
stability problem
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
individuals with HD have an average survival of how many years following onset of symptoms
15-20 years
how old are pts usually when they are diagnosed with HD?
30-50 yo
what is the prevalence of HD?
about 4.1-8.4 per 100,000 individuals in the US
what are the 5 stages of HD?
preclinical stage
early stage
middle stage
late stage
end-of-life stage
what stage of HD is characterized by mild symptoms, but no dx of HD from the doctor?
pre-clinical stage
what stage of HD is characterized by symptoms becoming noticeable enough for diagnosis?
early stage
what stage of HD is characterized by individuals being unable to carry out most activities on their own?
middle stage
what stage of HD is characterized by pts requiring assistance in all areas of life and becoming bedridden?
late stage
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
what are the super 6 tests and measures?
6MWT
Berg
5xSTS
FGA
gait speed
ABC
what is the MDC for the 6MWT?
82 m
what is the MDC for the Berg?
5 points
what is the MDC for the 5xSTS?
.6 sec
what is the MDC for the FGA?
4 points
what is the MDC for gait speed?
.18 m/s
what is the MDC for the ABC?
13
t/f: PD and HD have a multisystem presentation involving motor, non-motor, and secondary impairments
true
