Cerebellar Ataxia Flashcards

1
Q

What are the 3 functional regions of the cerebellum?

A
  • Vestibular cerebellum
  • Spinocerebellum
  • Cerebrocerebellum
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2
Q

What is the function of the vestibular cerebellum?

A
  • Controls balance & eye movement

- Via output to vestibular nuclei & input from semicircular canals

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3
Q

What is the function of the spinocerebellum?

A
  • Controls ongoing execution of limb movements

- Regulates muscle activity to allow for variations in load & smoothness

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4
Q

What is the function of the cerebrocerebellum?

A
  • Preparation, anticipation & initiation of movement
  • Precision, timing & sequencing
  • Coordination of movements to a visual target
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5
Q

What does the cerebellar blood supply consist of?

A

Branching off basilar artery:

  • Superior CA (SCA)
  • Anterior inferior CA (AICA)

Branching off vertebral arteries:
- Posterior inferior CA (PICA)

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6
Q

What is the overall role of the cerebellum?

A
  • Regulates motor control
  • Constructs synergies
  • Regulates intensity of movement
  • Perception, cognition& language
  • Movement to movement error correction
  • Timing & sequencing of muscle activation
  • Contributes to postural control during external perturbations or intentional actions
  • Mental practice of movement & visuospatial tasks
  • Perception of time intervals & velocity of moving objects (e.g. crossing the road)
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7
Q

What is cerebellar ataxia?

A
  • Abnormal coordination affecting speed, amplitude, accuracy & force of movement
  • Severe loss of dexterity
  • Result of damage to neurons in the cerbellum
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8
Q

What are the other types of ataxia?

A
  • Sensory (due to loss of proprioception)

- Vestibular (due to dysfunction of vestibular system)

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9
Q

What are the causes of cerebellar ataxia?

A
  • Focal lesions (stroke, TBI, tumour, MS)
  • Exogenous substances (alcohol, anti-epileptics)
  • Vitamin B12 deficiency
  • Non-hereditary cerebellar degeneration
  • Hereditary cerebellar degeneration
  • Arnold-Chiari malformation (cerebellar tonsils pushed down through foramen magnum - may require surgery)
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10
Q

What are some of the causes of non-hereditary cerebellar degeneration?

A
  • Idiopathic late onset cerebellar ataxia
  • Alcohol abuse
  • Coeliac disease
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11
Q

What are the types of hereditary cerebellar degeneration?

A
  • Autosomal dominant (spinocerebellar ataxia, episodic ataxia)
  • Autosomal recessive (Friedrich’s ataxia, ataxia-telangiectasia)
  • Generally always present in childhood (managed by paediatrics)
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12
Q

What are the characteristics of ataxic motor performance?

A
  • Dysmetria (over/undershooting)
  • Rebound phenomenon (delayed antagonist burst)
  • Dysdiadochokinesia
  • Dyssynergia or decomposition (poor coordination & smoothness)
  • Tremor (intentional or postural)
  • Dysarthria (poor coordination of speech production muscles)
  • Nystagmus
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13
Q

What does assessment of ataxic motor performance involve?

A
  • Assessment of rapid alternating movements
  • Assessment of postural sway (EO/EC)
  • Heel/shin
  • Heel over foot
  • Finger nose
  • Rebound test
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14
Q

What does assessment of cerebellar ataxia involve?

A
  • Assessment of activities
  • Observe missing important components
  • Observe adaptive strategies
  • Assess changing speed/direction, stopping on command, dual tasking
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15
Q

What are some of the measures used when assessing cerebellar ataxia?

A

(All valid & reliable)

  • Accuracy
  • Successful repetitions in a given time period
  • 10MWT
  • TUG
  • Step test
  • Functional reach
  • 6MWT
  • 9HPT
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16
Q

What are the typical adaptive motor behaviours in cerebellar ataxia?

A
  • Restriction of amplitude of movement
  • Restriction of degrees of freedom
  • Excessive pre-shaping & use of support surface during UL tasks
  • Excessive BOS, stepping & use of arms during standing & walking
  • Increased speed & difficulty slowing down in walking
  • Increased variability of performance
17
Q

What did Pulliyath et al 1998 find regarding gait in cerebellar ataxia patients?

A

Cerebellar patients demonstrated increase variability, decreased PF at push off & decreased knee yield during gait compared with controls

18
Q

What did Bastien et al 1996 find regarding reaching in cerebellar ataxia patients?

A
  • Cerebellar patients had an abnormally curved wrist path due to dyssynergia or decomposition of movement
  • Suggested this was due to cerebellum’s inability to predict & compensate for torques generated at various joints
  • CIs: Do whole task practice using multiple joints
19
Q

What did Bastien et al 2000 find regarding reaching in cerebellar ataxia patients?

A
  • Elbow flexion tasks were performed significantly better in cerebellar patients when the shoulder & elbow were fixed compared with when they were not fixed
  • CIs: Use fixed position for patients who are chronic progressive, prevent/change in recoverable patients
20
Q

What did Deiner & Dichgans et al 1992 find regarding heel raises in cerebellar ataxia patients?

A
  • During heel raises controls & cerebellar patients demonstrated the same EMG sequence (tib ant, quads, gastrocs), but cerebellar patients had disordered timing & poor build up of EMG activity
  • CIs: Train timing
21
Q

What did Morton et al 2004 find regarding stepping in cerebellar ataxia patients?

A
  • Cerebellar patients had greater dysmetria in knee flexion when stepping over and obstacle than in normal walking
  • CIs: Train flexibility of performance in recoverable patients
22
Q

What did Lang & Bastien et al 2002 find regarding task performance in cerebellar ataxia patients?

A
  • With 25 practices of a task, cerebellar patients can improve performance
  • The addition of a second task resulted in regression to original performance
  • CIs: Train dual tasks in recoverable patients, consider safety of dual tasking in chronic progressive patients
23
Q

What did Ballet et al 1987 find regarding balance & coordination training in cerebellar ataxia?

A
  • Treated TBI patients with cerebellar ataxia with balance & coordination training & reduction in use of UL
  • Results indicated significant improvement in function & independent walking ability
  • CIs: Train balance/walking, decrease adaptive strategies
24
Q

What did Miyai et al 2012 find regarding physio interventions for cerebellar degeneration?

A
  • RCT of patients with cerebellar degeneration (chronic progressive)
  • 2 hours PT & OT daily for 4 weeks
  • Intervention included rehab for coordination, balance & ADLs
  • Results showed increased walking speed, decreased falls & improved ADLs (maintained at 12 & 24 weeks)
  • CIs: High intensity practice can improve chronic progressive patients
25
Q

What did the 2014 Consensus Statement by Ilg et al state?

A
  • Physio is a mainstay of managing people with cerebellar ataxia
  • Physio should be intensive
  • Physio must be life-long
26
Q

How should ataxia be trained?

A
  • Spatial accuracy
  • Timing accuracy
  • Modified task practice
  • External constraints
  • Ensure patient cannot use adaptive strategies
  • Increase complexity
27
Q

What does training for spatial accuracy involve?

A
  • Tasks where excessive movements are constrained

- Feedback is implicit in the task

28
Q

What does training for timing accuracy involve?

A
  • Closed tasks (same every time) which require rhythm

- Open tasks (change each time) which require predictive timing

29
Q

How can complexity of training be increased?

A
  • Withdraw external constraints
  • Increase amplitude of movement
  • Change speed, amplitude, direction & force
  • Add balance requirements
  • Stop mid-task
  • Train automaticity