Management of Individuals with Cerebellar Disorders Flashcards
do cerebellar disorders result in lack of mobility or stability?
lack of stability
what part of the cerebellum is the spinocerebellum?
the vermis
what part of the cerebellum is the cerebrocerebellum?
cerebellar hemispheres
what part of the cerebellum is the vestibulocerebellum?
the floculonodular lobe
what is the role of the spinocerebellum?
regulation of muscle tone and coordination
what does damage to the spinocerebellum result in?
lack of truncal control/truncal ataxia
what is the role of the cerebrocerebellum?
planning and timing of movt
what does damage to the cerebrocerebellum result in?
limb ataxia
what is the role of the vestibulocerebellum?
postural control and eye movt
what does damage to the vestibulocerebellum result in?
visual ataxia
what are the key roles of the cerebellum?
motor coordination, accuracy, and amplitude (gross and fine limb control)
balance and posture
fine motor control
motor learning and adaptation
a majority of interventions for cerebellar injury should be focused on what?
proximal stability
the cerebellum plays a huge role in what kind of balance?
anticipatory postural control (which then effects reactive postural control)
what are the gross motor control functions affected in cerebellar injury?
dysmetria and dysdiadochokinesia
what are common symptoms of cerebellar disorders?
ataxia
intention tremor
dysmetria (over/undershoot)
dysdiadochokinesia (impaired RAMs)
nystagmus
dysarthria (ataxic speech)
gait disturbances
balance impairments
what is intention tremor?
tremor with movt
what are the fxnal impacts of cerebellar disorders?
excessive mobility and lack of stability
difficulty with ADLs requiring gross and fine motor control
decreased trunk control/stability
increased fall risk
challenges with mobility
decreased control of ambulation
coordination of respiratory and postural control muscles
are pts with cerebellar disorders are risk of falling forward or backward?
backward bc they are retropulsive
do pts with cerebellar injury fall towards or away from the side of injury?
towards the side of injury
what do pts with cerebellar disorders often do to gain trunk stability?
breath hold, especially early on
what is titubation of the trunk?
underlying trunk tremor that looks like ataxia but is smaller, high frequency movt
does slow or fast movt lead to movt decompensation?
slow movt
slow movt results in what three things?
movt decomposition
dysynergia
hypometria
does slow movt result in overshooting or undershooting movts?
undershooting (hypometria)
does fast movt result in overshooting or undershooting movts?
overshooting movts (hypermetria)
what tests can be used for slow movts?
foot to nose (FTN)
heel to shin (HTS)
fast movt results in what three things?
hypermetria
dysdiadochokinesia
abnormal APRs
what tests can be used for fast movts?
rapid eye movts (RAMs)
finger to nose (FTN)
finger chase
what are the motor learning tasks the cerebellum is responsible for?
adjusting and refining voluntary limb movts
making and adjusting eye movts
preparing for movt and recovering balance after a perturbation
learning new walking patterns
pts with cerebellar disorder demonstrate slower rates of ____, decreased magnitude of ____, or no _____ at all
adaptation
is there increased or decreased procedural learning in cerebellar disorders?
decreased
do we want to include or avoid error augmentation in treatment of pts with cerebellar disorders?
avoid error augmentation
why do we want to limit errors in treatment with cerebellar disorders?
bc they have limited/no ability to correct errors
how can we bypass the circuitry injured in cerebellar injury?
errorless learning and high repetitions
what are the causes of cerebellar disorders?
vascular injury
degenerative disorders
toxic/metabolic causes
infections
neoplasms
trauma
what are vascular events that can cause cerebellar disorder?
cerebellar infarcts or hemorrhages
what are the degenerative disorders that can result in cerebellar disorder?
MSA-c
spinocerebellar ataxia
Friedreich’s ataxia
why is HD (and hyperkinetic disorders) treated similarly to cerebellar disorders?
bc they both involved a lack of stability
what is the most common disorder causing ataxia?
Friedreich’s ataxia
what are toxic/metabolic causes of cerebellar disorders?
chronic alcoholism
meds (eg. anticonvulsants)
is AICA or PICA low BS?
PICA
is AICA or PICA high BS?
AICA
is lateral medullary/Wallenberg syndrome effecting the AICA or PICA?
PICA
what are the s/s of lateral medullary/Wallenberg syndrome (PICA)?
ipsi limb and gait ataxia
DYSPHAGIA, HOARSENESS
contra loss of pain and temp
vertigo and nystagmus
Horner’s syndrome
what are some common symptoms among PICA and AICA?
ataxia, Horner’s syndrome, and contra loss of pain and temp
what are the s/s of lat sup pontine/Marie-Foix syndrome (AICA)?
ipsi limb and gait ataxia
contra loss of pain and temp
propriocetive impairment LE>UE
falling toward lesion
VERTIGO (NYSTAGMUS), NAUSEA, VOMITING
ISPI HEARING LOSS/TINNITUS
FACIAL WEAKNESS
facial sensory disturbances
gaze paresis
hemiparesis or hemiplegia
Horner’s syndrome
does lat sup pontine/Marie-Foix syndrome affect AICA or PICA?
AICA
is dysphagia and hoarseness a sign of AICA or PICA?
PICA
is hearing loss a sign of AICA or PICA?
AICA
what does corticospinal involvement in AICA cause?
hemiplegia, hyperreflexia, spasticity, and other UMN signs
what is the difference bw central and peripheral nystagmus?
central nystagmus is caused by the BS and is vertical, combo, or changing directions/different in each eye
peripheral nystagmus is caused by the inner ear and is horizontal, linear, or torsional
what is an other name for spinocerebellar atrophy/ataxia (SCA)?
olivopontocerebellar atrophy
what is SCA?
degeneration of axons w/in multiple neural systems
progressive genetic disorder with over 35 subtypes
what neural systems are effected in SCA?
cerebellum
BS
BG
SC
when is the onset of SCA typically?
20-50 y/o but varies by subtype
typically adolescence
what is the prognosis for SCA?
rapid progression to dependence w/in a few years
what is the life expectancy of someone with SCA?
6-29 years after onset of symptoms
what are confounding factors in SCA?
weight management
nutrition
comorbidities
what is the most common subtype of SCA?
SCA 3 (Machado-Joseph’s disease)
what is SCA 3 (Machado-Joseph’s disease)?
the most common subtype of SCA
UMN/LMN degeneration mix caused by mutation in the ATXN3 gene
what are common symptoms of SCA?
dysarthria
oculomotor abnormalities
limb ataxia
progressive gait ataxia
what additional impairments may be present in SCA depending on the subtype?
cognitive deficits
peripheral neuropathy
dystonia
dysphagia
what are the early signs of SCA?
limb heaviness–>limb ataxia–> gait ataxia
fatigue
what are the mental fxn impairments in SCA?
difficulty processing, learning, and remembering info
what is the sensory fxn impairment in SCA?
peripheral neuropathy
what is the CVP, hemo, and immuno fxn impairment in SCA?
excessive use of accessory respiratory muscles
what is the voice and speech fxn impairment in SCA?
dysarthria
what is the genitourinary and reproductive fxn impairment in SCA?
loss of bladder control
what are the neuro MSK and movt related fxn impairments in SCA?
oculomotor slowing, nystagmus, diploplia
hyperreflexia, truncal/limb ataxia
decreased locomotion
progressive inability to maintain posture
dystonia
what is Friedreich’s ataxia?
neurodegenerative, progressive genetic disorder affecting the CNS, PNS, pancreatic beta cells, and heart from a mutation in both copies of the FXN gene
what is the role of the FXN gene?
frataxin protein for encoding mitochondrial fxn (ATP production)
what is the most common form of autosomal recessive ataxia?
Friedreich’s ataxia
what does a mutation of the FXN gene cause in Friedreich’s ataxia?
NS, cardiac, and metabolic dysfxn
what are we concerned about with a pt with Friedreich’s ataxia?
exercise tolerance and adequate cardiac fxn for exercise
when do symptoms develop in Friedreich’s ataxia?
in adolescence
t/f: DM becomes more unstable as time goes on in Friedreich’s ataxia
true
what is the prognosis for Friedreich’s ataxia?
progressive decline, typically WC by late teens, early 20s
what is the #1 cause of mortality in Friedreich’s ataxia?
cardiomyopathy
Friedreich’s ataxia causes atrophy of what structures?
cerebellum
spinocerebellar tract
medulla
corticospinal tract
DCML
what is the clinical presentation of someone with Friedreich’s ataxia?
balance impairment
EARLY gait ataxia
vision changes (nystagmus and oculomotor slowing)
motor impairments (MUSCLE WEAKNESS and progressive limb ataxia)
SENSORY DEFICITS (vibration and proprioception)
dysarthria
SCOLIOSIS (>15-20 deg)
DM
HYPERTROPHIC CARDIOMYOPATHY
what causes aggressive scoliosis in Friedreich’s ataxia?
abborant muscles pulling
t/f: with truncal and gait ataxia in Friedreich’s ataxia, unsteadiness and balance changes appear first
true
does Friedreich’s ataxia involve cerebellar or sensory ataxia?
both
what is a disease-specific test/measure for cerebellar disorders?
Scale for the Assessment and Rating of Ataxia (SARA)
what are the general tests/measures used in cerebellar disorders?
coordination tests (fine and gross motor)
gait analysis
balance
fxnal mobility
what are disorder-specific tests/measures for cerebellar disorders?
oculomotor assessment
cardiopulmonary fxn and endurance
sensory testing
strength testing
scoliosis assessment
what is the SARA?
a semi-quantitative assessment of cerebellar ataxia w a gait and ADL component (includes a standard clinical measure of coordination)
what is the range for the score in SARA?
0 (no ataxia) to 40 (severe ataxia)
what are the 8 items of the SARA?
gait
stance
sitting
speech
finger chase
FTN
HTS
RAMs
what balance assessments can be used in cerebellar disorders?
Berg
MiniBEST
what posture assessments can be used in cerebellar disorders?
mCTSIB
assess postural sway and limits of stability under varied sensory conditions
what assessment can be used for fear of falling/confidence in mobility in cerebellar disorders?
ABC
what tests for fxnal locomotion may be used in cerebellar disorders?
10mWT
3mBWT
FGA
4 square step test
TUG
with disease progression, do we expect balance assessments to improve or worsen?
worsen due to disease progression
what things would be noted in observational gait analysis in cerebellar disorders?
wide BOS
irregular control of limb advancement
irregular control of weight acceptance/single limb stance
decreased arm swing
trunk stiffness
what are the tests for RAMs in cerebellar disorders?
supination-pronation
alternating toe tapping
what are the tests for dysmetria (accuracy) in cerebellar disorders?
FTN
HTS
what is finger chasing?
FTN with moving target
what is the box and blocks test?
a test of gross motor fxn and dexterity
each UE tested for 1 minute picking up 1 block at a time and moving it from box 1 to box 2
how do we document dysdiadochokinesia and dysmetria?
0 (normal) to 4 (severe)
what is a 1 for dysdiadochokinesia and dysmetria?
slight
s/s cause no impact on fxn
what is a 2 for dysdiadochokinesia and dysmetria?
mild
s/s cause modest impact on fxn
what is a 3 for dysdiadochokinesia and dysmetria?
moderate
s/s impact considerably, but don’t prevent fxn
what is a 4 for dysdiadochokinesia and dysmetria?
severe
s/s prevent fxn
what are the fine motor control assessments used for cerebellar disorders?
9 hole peg test
perdue peg board
what is the 9 hole peg test?
test for motor timing and accuracy
pegs taken from cup to board and back to cup with each UE
what is the perdue pegboard test?
assessment for gross and fine finger dexterity strongly correlated with the UPDRS
involves assembly of pieces using each and both UEs
what are the 3 components for interventions for proximal stability?
stability
controlled mobility
variable velocities
how can we improve gait in cerebellar disorders?
by working on BALANCE
what are the key considerations for intervention planning with cerebellar disorders?
motor learning optimization
safety and fall prevention
task-specific training of primary impairments
compensatory strategies (particularly for ADLs)
adaptive equipment needs
environment modifications
pt/caregiver education
interdisciplinary collaboration
long-term management strategies
what are the goals of interventions in cerebellar disorders?
maximize fxn and safety
maintain independence
prevent complications
t/f: we use pre-gait a lot in cerebellar populations
true
what are good pre-gait positions to use in cerebellar populations?
quadruped
tall kneeling
1/2 kneeling
what are motor learning challenges in cerebellar disorders?
impaired adaptation
difficulty with error detection and correction
t/f: we are looking for ERRORLESS performance in cerebellar treatment
true
what are some strategies for motor learning in cerebellar disorders?
use dependent learning (repetition to strengthen motor plans through neuroplasticity)
reward-reinforcement learning (engaging the BG to bypass impaired cerebellar circuitry)
conscious control strategies (verbal cues and visual feedback)
challenge-oriented treatment (gradually increase takes difficulty)
t/f: we have to stabilize vision with nystagmus
true
t/f: if the cerebellum isn’t totally destroyed, neighboring areas of the cerebellum can adapt or compensate for the impaired components
true
what are some mechanisms of recovery after CNS lesions?
neural sprouting
fxnal reorganization
substitution
plasticity
how should we include gaze stability and vision control exercises in interventions for cerebellar disorders?
incorporate visual activities into fxnal activities for anyone w/visual ataxia
what are some interventions for proprioceptive loss in cerebellar disorders?
theraband pants and vest
propriceptive balance-based torso-weighting (BBTW) vest
approximation, heavy WB
how can we stabilize pts with cerebellar disorders?
add proprioceptive input
what is a BBTW vest?
strategic placement of small weights on the torso to counter balance the pts directional instability
t/f: BBTW vest has immediate effects on stability
true
t/f: BBTW vest allows for extensive practice and reduced errors for both sensory and cerebellar ataxia
true
is there evidence to support weights for increased stability?
not currently
what are interventions for trunk control and proximal stability?
PNF
CKC UE exercises (heavy WB)
trunk/core stability
BBTW vest
what PNF techniques can be used for stability?
slow dynamic reversals and holds
alternating and rhythmic stabilization
overflow and irradiation
COIs
what is the progression of positions to work through for trunk stability?
supine–>sitting–>tall/1/2 kneeling–>standing–>walking
is pushing or pulling better for trunk stability in cerebellar disorders?
pushing, pulling may cause more errors
what is the difference bw internally generated perturbations and externally generated perturbations?
internally generated perturbations are a response to self-generated challenges exceeding their capabilities from increased sway, change in body position, increase speed, or increased range (less weight)
externally generated pertubrations are a response to an increase/variable weight/force/resistance, or unpredictable surface change (more weight)
what are some interventions for gait training in cerebellar disorders?
body weight supported treadmill training
split belt treadmill training
overground gait training w/visual gaze stabilization
harness based training
dual task training
added metronome or music for timing control
t/f: split belt treadmill training has potential for improving step length symmetry
true
t/f: split belt treadmill training improves 10mWT and standing balance
true
t/f: split belt treadmill training improves interlimb coordination
false, it does not
what are the focuses of gait training in cerebellar populations?
improving gait parameters (speed, step length, BOS)
enhancing dynamic balance during gait
practicing fxnal mobility tasks
what are some interventions for coordinated muscles fxn in cerebellar disorders?
Frenkel exercises
task specific training
technology-assisted interventions
what exercises work on the pts OWN coordination of muscles activity with slow coordinated movt w/even timing and are often given in an HEP?
Frankel exercises
what are the Frankel exercises?
I will add these later after class thursday
what positions are the Frankel exercises performed in?
lying
sitting
standing
walking
what is the progression in Frankel exercises?
independent–> unilateral–> bilateral
