Management of Individuals with Cerebellar Disorders Flashcards

1
Q

do cerebellar disorders result in lack of mobility or stability?

A

lack of stability

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2
Q

what part of the cerebellum is the spinocerebellum?

A

the vermis

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3
Q

what part of the cerebellum is the cerebrocerebellum?

A

cerebellar hemispheres

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4
Q

what part of the cerebellum is the vestibulocerebellum?

A

the floculonodular lobe

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5
Q

what is the role of the spinocerebellum?

A

regulation of muscle tone and coordination

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6
Q

what does damage to the spinocerebellum result in?

A

lack of truncal control/truncal ataxia

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7
Q

what is the role of the cerebrocerebellum?

A

planning and timing of movt

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8
Q

what does damage to the cerebrocerebellum result in?

A

limb ataxia

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9
Q

what is the role of the vestibulocerebellum?

A

postural control and eye movt

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10
Q

what does damage to the vestibulocerebellum result in?

A

visual ataxia

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11
Q

what are the key roles of the cerebellum?

A

motor coordination, accuracy, and amplitude (gross and fine limb control)

balance and posture

fine motor control

motor learning and adaptation

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12
Q

a majority of interventions for cerebellar injury should be focused on what?

A

proximal stability

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13
Q

the cerebellum plays a huge role in what kind of balance?

A

anticipatory postural control (which then effects reactive postural control)

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14
Q

what are the gross motor control functions affected in cerebellar injury?

A

dysmetria and dysdiadochokinesia

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15
Q

what are common symptoms of cerebellar disorders?

A

ataxia

intention tremor

dysmetria (over/undershoot)

dysdiadochokinesia (impaired RAMs)

nystagmus

dysarthria (ataxic speech)

gait disturbances

balance impairments

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16
Q

what is intention tremor?

A

tremor with movt

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17
Q

what are the fxnal impacts of cerebellar disorders?

A

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

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18
Q

are pts with cerebellar disorders are risk of falling forward or backward?

A

backward bc they are retropulsive

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19
Q

do pts with cerebellar injury fall towards or away from the side of injury?

A

towards the side of injury

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20
Q

what do pts with cerebellar disorders often do to gain trunk stability?

A

breath hold, especially early on

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21
Q

what is titubation of the trunk?

A

underlying trunk tremor that looks like ataxia but is smaller, high frequency movt

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22
Q

does slow or fast movt lead to movt decompensation?

A

slow movt

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23
Q

slow movt results in what three things?

A

movt decomposition

dysynergia

hypometria

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24
Q

does slow movt result in overshooting or undershooting movts?

A

undershooting (hypometria)

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25
does fast movt result in overshooting or undershooting movts?
overshooting movts (hypermetria)
26
what tests can be used for slow movts?
foot to nose (FTN) heel to shin (HTS)
27
fast movt results in what three things?
hypermetria dysdiadochokinesia abnormal APRs
28
what tests can be used for fast movts?
rapid eye movts (RAMs) finger to nose (FTN) finger chase
29
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
30
pts with cerebellar disorder demonstrate slower rates of ____, decreased magnitude of ____, or no _____ at all
adaptation
31
is there increased or decreased procedural learning in cerebellar disorders?
decreased
32
do we want to include or avoid error augmentation in treatment of pts with cerebellar disorders?
avoid error augmentation
33
why do we want to limit errors in treatment with cerebellar disorders?
bc they have limited/no ability to correct errors
34
how can we bypass the circuitry injured in cerebellar injury?
errorless learning and high repetitions
35
what are the causes of cerebellar disorders?
vascular injury degenerative disorders toxic/metabolic causes infections neoplasms trauma
36
what are vascular events that can cause cerebellar disorder?
cerebellar infarcts or hemorrhages
37
what are the degenerative disorders that can result in cerebellar disorder?
MSA-c spinocerebellar ataxia Friedreich's ataxia
38
why is HD (and hyperkinetic disorders) treated similarly to cerebellar disorders?
bc they both involved a lack of stability
39
what is the most common disorder causing ataxia?
Friedreich's ataxia
40
what are toxic/metabolic causes of cerebellar disorders?
chronic alcoholism meds (eg. anticonvulsants)
41
is AICA or PICA low BS?
PICA
42
is AICA or PICA high BS?
AICA
43
is lateral medullary/Wallenberg syndrome effecting the AICA or PICA?
PICA
44
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
45
what are some common symptoms among PICA and AICA?
ataxia, Horner's syndrome, and contra loss of pain and temp
46
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
47
does lat sup pontine/Marie-Foix syndrome affect AICA or PICA?
AICA
48
is dysphagia and hoarseness a sign of AICA or PICA?
PICA
49
is hearing loss a sign of AICA or PICA?
AICA
50
what does corticospinal involvement in AICA cause?
hemiplegia, hyperreflexia, spasticity, and other UMN signs
51
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
52
what is an other name for spinocerebellar atrophy/ataxia (SCA)?
olivopontocerebellar atrophy
53
what is SCA?
degeneration of axons w/in multiple neural systems progressive genetic disorder with over 35 subtypes
54
what neural systems are effected in SCA?
cerebellum BS BG SC
55
when is the onset of SCA typically?
20-50 y/o but varies by subtype typically adolescence
56
what is the prognosis for SCA?
rapid progression to dependence w/in a few years
57
what is the life expectancy of someone with SCA?
6-29 years after onset of symptoms
58
what are confounding factors in SCA?
weight management nutrition comorbidities
59
what is the most common subtype of SCA?
SCA 3 (Machado-Joseph's disease)
60
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
61
what are common symptoms of SCA?
dysarthria oculomotor abnormalities limb ataxia progressive gait ataxia
62
what additional impairments may be present in SCA depending on the subtype?
cognitive deficits peripheral neuropathy dystonia dysphagia
63
what are the early signs of SCA?
limb heaviness-->limb ataxia--> gait ataxia fatigue
64
what are the mental fxn impairments in SCA?
difficulty processing, learning, and remembering info
65
what is the sensory fxn impairment in SCA?
peripheral neuropathy
66
what is the CVP, hemo, and immuno fxn impairment in SCA?
excessive use of accessory respiratory muscles
67
what is the voice and speech fxn impairment in SCA?
dysarthria
68
what is the genitourinary and reproductive fxn impairment in SCA?
loss of bladder control
69
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
70
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
71
what is the role of the FXN gene?
frataxin protein for encoding mitochondrial fxn (ATP production)
72
what is the most common form of autosomal recessive ataxia?
Friedreich's ataxia
73
what does a mutation of the FXN gene cause in Friedreich's ataxia?
NS, cardiac, and metabolic dysfxn
74
what are we concerned about with a pt with Friedreich's ataxia?
exercise tolerance and adequate cardiac fxn for exercise
75
when do symptoms develop in Friedreich's ataxia?
in adolescence
76
t/f: DM becomes more unstable as time goes on in Friedreich's ataxia
true
77
what is the prognosis for Friedreich's ataxia?
progressive decline, typically WC by late teens, early 20s
78
what is the #1 cause of mortality in Friedreich's ataxia?
cardiomyopathy
79
Friedreich's ataxia causes atrophy of what structures?
cerebellum spinocerebellar tract medulla corticospinal tract DCML
80
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
81
what causes aggressive scoliosis in Friedreich's ataxia?
abborant muscles pulling
82
t/f: with truncal and gait ataxia in Friedreich's ataxia, unsteadiness and balance changes appear first
true
83
does Friedreich's ataxia involve cerebellar or sensory ataxia?
both
84
what is a disease-specific test/measure for cerebellar disorders?
Scale for the Assessment and Rating of Ataxia (SARA)
85
what are the general tests/measures used in cerebellar disorders?
coordination tests (fine and gross motor) gait analysis balance fxnal mobility
86
what are disorder-specific tests/measures for cerebellar disorders?
oculomotor assessment cardiopulmonary fxn and endurance sensory testing strength testing scoliosis assessment
87
what is the SARA?
a semi-quantitative assessment of cerebellar ataxia w a gait and ADL component (includes a standard clinical measure of coordination)
88
what is the range for the score in SARA?
0 (no ataxia) to 40 (severe ataxia)
89
what are the 8 items of the SARA?
gait stance sitting speech finger chase FTN HTS RAMs
90
what balance assessments can be used in cerebellar disorders?
Berg MiniBEST
91
what posture assessments can be used in cerebellar disorders?
mCTSIB assess postural sway and limits of stability under varied sensory conditions
92
what assessment can be used for fear of falling/confidence in mobility in cerebellar disorders?
ABC
93
what tests for fxnal locomotion may be used in cerebellar disorders?
10mWT 3mBWT FGA 4 square step test TUG
94
with disease progression, do we expect balance assessments to improve or worsen?
worsen due to disease progression
95
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
96
what are the tests for RAMs in cerebellar disorders?
supination-pronation alternating toe tapping
97
what are the tests for dysmetria (accuracy) in cerebellar disorders?
FTN HTS
98
what is finger chasing?
FTN with moving target
99
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
100
how do we document dysdiadochokinesia and dysmetria?
0 (normal) to 4 (severe)
101
what is a 1 for dysdiadochokinesia and dysmetria?
slight s/s cause no impact on fxn
102
what is a 2 for dysdiadochokinesia and dysmetria?
mild s/s cause modest impact on fxn
103
what is a 3 for dysdiadochokinesia and dysmetria?
moderate s/s impact considerably, but don't prevent fxn
104
what is a 4 for dysdiadochokinesia and dysmetria?
severe s/s prevent fxn
105
what are the fine motor control assessments used for cerebellar disorders?
9 hole peg test perdue peg board
106
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
107
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
108
what are the 3 components for interventions for proximal stability?
stability controlled mobility variable velocities
109
how can we improve gait in cerebellar disorders?
by working on BALANCE
110
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
111
what are the goals of interventions in cerebellar disorders?
maximize fxn and safety maintain independence prevent complications
112
t/f: we use pre-gait a lot in cerebellar populations
true
113
what are good pre-gait positions to use in cerebellar populations?
quadruped tall kneeling 1/2 kneeling
114
what are motor learning challenges in cerebellar disorders?
impaired adaptation difficulty with error detection and correction
115
t/f: we are looking for ERRORLESS performance in cerebellar treatment
true
116
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)
117
t/f: we have to stabilize vision with nystagmus
true
118
t/f: if the cerebellum isn't totally destroyed, neighboring areas of the cerebellum can adapt or compensate for the impaired components
true
119
what are some mechanisms of recovery after CNS lesions?
neural sprouting fxnal reorganization substitution plasticity
120
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
121
what are some interventions for proprioceptive loss in cerebellar disorders?
theraband pants and vest propriceptive balance-based torso-weighting (BBTW) vest approximation, heavy WB
122
how can we stabilize pts with cerebellar disorders?
add proprioceptive input
123
what is a BBTW vest?
strategic placement of small weights on the torso to counter balance the pts directional instability
124
t/f: BBTW vest has immediate effects on stability
true
125
t/f: BBTW vest allows for extensive practice and reduced errors for both sensory and cerebellar ataxia
true
126
is there evidence to support weights for increased stability?
not currently
127
what are interventions for trunk control and proximal stability?
PNF CKC UE exercises (heavy WB) trunk/core stability BBTW vest
128
what PNF techniques can be used for stability?
slow dynamic reversals and holds alternating and rhythmic stabilization overflow and irradiation COIs
129
what is the progression of positions to work through for trunk stability?
supine-->sitting-->tall/1/2 kneeling-->standing-->walking
130
is pushing or pulling better for trunk stability in cerebellar disorders?
pushing, pulling may cause more errors
131
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)
132
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
133
t/f: split belt treadmill training has potential for improving step length symmetry
true
134
t/f: split belt treadmill training improves 10mWT and standing balance
true
135
t/f: split belt treadmill training improves interlimb coordination
false, it does not
136
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
137
what are some interventions for coordinated muscles fxn in cerebellar disorders?
Frenkel exercises task specific training technology-assisted interventions
138
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
139
what are the Frankel exercises?
I will add these later after class thursday
140
what positions are the Frankel exercises performed in?
lying sitting standing walking
141
what is the progression in Frankel exercises?
independent--> unilateral--> bilateral