Cerebellar Ataxia: Pathophysiology and Rehabilitation

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Cerebellar ataxia: Pathophysiology and rehabilitation
Article in Clinical Rehabilitation · March 2011

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Clinical Rehabilitation
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Cerebellar ataxia: pathophysiology and rehabilitation

Jon Marsden and Chris Harris
Clin Rehabil 2011 25: 195
DOI: 10.1177/0269215510382495
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Clinical Rehabilitation 2011; 25: 195–216
Rehabilitation in practice
Cerebellar ataxia: pathophysiology and rehabilitation

Jon Marsden School of Health Professions, Faculty of Health, University of Plymouth and
Chris Harris School of Psychology, Faculty of Science, University of Plymouth, Plymouth, UK
This series of articles for rehabilitation in practice aims to cover a knowledge

element of the rehabilitation medicine curriculum. Nevertheless they are intended
to be of interest to a multidisciplinary audience. The competency addressed in this
article is ‘The trainee consistently demonstrates a knowledge of management
approaches for specific impairments including spasticity, ataxia.’
Introduction Cochrane Library with the search terms ‘cerebel-

lum or cerebellar’ and ‘ataxia or tremor’ and ‘phys-
Cerebellar dysfunction may result in significant iology or pathophysiology or rehabilitation
functional difficulties with upper and lower limb or therapy’). Articles were further identified through
movement, oculo-motor control, balance and searches of reference lists in identified papers.
walking. Such difficulties can affect employability,
increase carer burden and reduce a person’s per-
ceived quality of life.1 The economic burden of Anatomy
people with spinocerebellar ataxia is estimated to
be around E18 776 per annum.1 This article Gross anatomy
will review the signs and symptoms and patho- Cerebellar ataxia arises due damage or dysfunc-
physiology of cerebellar ataxia, highlighting the tion affecting the cerebellum and/or its input/
theoretical basis of current non-surgical and non- output pathways. In a rostro-caudal direction the
pharmacological rehabilitation techniques. cerebellum can be divided into three lobes: ante-
rior, posterior and flocculo-nodular. Some of these
may take the brunt of certain pathologies. The
Search strategy and selection criteria anterior lobe, for example, is particularly affected
in alcoholic cerebellar degeneration.2 The cerebel-
References for this review were identified from a lum connects to the medulla, pons and midbrain
search of PubMed (from 1966 to 2009) and the via the inferior (restiform body), middle and supe-
rior cerebellar peduncles through which pass affer-
ent and efferent pathways.
Address for correspondence: Professor Jon Marsden, School of
Health Professions, Peninsula Allied Health Centre, Derriford
Functionally, the cerebellum is organized in
Road, University of Plymouth PL6 8BH, UK. a medio-lateral fashion with different areas of
e-mail: [email protected] the cerebellum having a distinct anatomical
ß The Author(s), 2011.
Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0269215510382495

196 J Marsden and C Harris
input–output relationship resulting in a specific main excitatory inputs: the climbing and mossy
lesion–symptom relationship.3–5 The midline fibres. Climbing fibres arise from defined areas of
vermis connects to the fastigial deep cerebellar inferior olive within the brainstem and project to a
nucleus, while the flocculonodular lobe is con- small number of Purkinje cells in specific longitu-
nected with the vestibular nucleus; lesions to dinal zones; one Purkinje cell receives one climbing
these midline areas result in deficits in posture, fibre. Mossy fibres convey inputs from all other
locomotion and oculomotor control. The interme- incoming pathways, such as the spinocerebellar
diate zone of the adjacent cerebellar hemisphere tracts and inputs from the cerebral cortex via the
mainly connects to the interpositus deep cerebellar pontine nuclei. These inputs synapse with the extre-
nucleus (globose and emboliform nuclei in mely numerous granule cells (numbering between
humans) and lesions here result in limb tremor 1010 and 1011) whose axons bifurcate giving rise to
and impairments in limb motion6 and dysarthria. parallel fibres. The parallel fibres run for about
The lateral cerebellar hemisphere connects to the 2–7 mm in a medio-lateral direction, synapsing in
dentate deep cerebellar nucleus; lesions here result passing on Purkinje cells (up to 80 000 parallel
in poor visuomotor coordination.7 More recently, fibres synapse with 1 purkinje cell).12
the finding of extensive connections between the The uniformity of the cerebellar cytoarchitec-
lateral hemisphere and prefrontal cortex in ture suggests that the cerebellum may be perform-
humans and greater apes has highlighted a role ing an identical computational function in these
of the cerebellum in non-motor functions, such different areas. Many putative, not necessarily
as working memory as discussed later.8,9 mutually exclusive, functions of the cerebellum
have been put forward and include a role in:
motor learning and adaptation; coordination;
Blood supply modulation of sensorimotor gain; timing; internal
The cerebellum is supplied by three main arter- representation of the dynamics of the limb and
ies that arise from the vertebrobasilar arteries. The sensorimotor transformations.7,13–19 At the heart
posterior inferior cerebellar artery supplies the of many of these theories lies the interaction
dorsal medulla (including parts of the vestibular between the climbing fibres and parallel fibres at
nuclei and the inferior cerebellar peduncle) and the level of the Purkinje cell.
the vermis and inferior cerebellum. The anterior Understanding the exact role of the cerebellum
inferior cerebellar artery supplies the lateral pons in normal motor control will aid in our interpre-
(including the facial nucleus and parts of the ves- tation and understanding of cerebellar ataxia and
tibular nuclei and the middle cerebellar peduncle) ultimately help to guide treatment strategies.
and the cerebellar flocculus and adjacent parts of
the inferior surface of the cerebellum. The anterior
inferior cerebellar artery also gives rise to the inter- Aetiology and prevalence
nal auditory and labyrinthine arteries. The supe-
rior cerebellar artery is the largest of the three The aetiology of cerebellar ataxia is summarized in
arteries; it supplies the upper pons (including supe- Table 1. Of the hereditary ataxias the prevalence
rior cerebellar peduncle) and the superior part of of Friedreich’s ataxia is estimated at 2–5 per
the cerebellum.10 100 000, while the spinocerebellar ataxias have a
prevalence of between 0.9 and 3.0 per 100 000
depending on the exact type.20,21 The most
Cytoarchitecture common spinocerebellar ataxia is SCA6, which
The cytoarchitecture of the cerebellar cortex is mainly affects the cerebellum with minimal
very uniform throughout its extent.11 The output additional extracerebellar pathology. Of the non-
cells of the cerebellar cortex, the Purkinje cells, hereditary ataxias, the most common cause of cer-
mainly project to the deep cerebellar nuclei with ebellar ataxia is multiple sclerosis (approximate
the exception of cells within the flocculus (see prevalence of 100 per 100 00022); here cerebellar
above). Their dendrites are oriented in a parasagit- signs are felt to occur in between 10 and 50% of
tal direction. The cerebellar cortex receives two cases, depending on the age of onset.23,24

Cerebellar ataxia: pathophysiology and rehabilitation 197
Table 1 Aetiology of cerebellar ataxia The movements themselves are prolonged in dura-
Hereditary Autosomal recessive:
tion and they show a decreased maximal velocity
Friedreich’s ataxia and an increase in spatial variability, that is, the
Ataxia telangiectasia path that is followed varies from trial to trial.29–31
Abetalipoproteinaemia Variability in the spatial path is seen early in the
Mitochondrial disorders movement before there is any time to process
Autosomal dominant:
Spinocerebellar ataxia 1 visual feedback, suggesting there is a problem
Episodic ataxia (EA-1 and EA-2) with movement planning. This also accounts for
Acquired Tumours and paraneoplastic degeneration the prolonged reaction time.32 In keeping with an
Congenital malformations important role of the cerebellum in planning
Immune disease
Stroke predictive movements fast, ballistic movements
Trauma that are entirely preplanned are inaccurate.33
Infections However, inaccuracies in slower movements may
Multisystem atrophy also be seen as without predictive control these
Spongiform enchephalopathy
Corticobasal degeneration
movements now over-rely on time-delayed feed-
Endocrine disorders back signals.
Acquired vitamin deficiency or metabolic People with cerebellar ataxia in particular show
disorders marked deficits in multi-joint movements, called
Toxins (e.g. alcohol, heavy metals,
antiepileptic medication)
dyssynergia. This, in part, results from an inability
to compensate for movement-associated interaction
Reproduced with kind permission from CRC Press.288 torques. Interaction torques are turning moments
brought on by movement about one joint that influ-
ence the motion of adjacent joints.34,35 The deficits
Signs and symptoms in multi-joint movements mean that people with
cerebellar ataxia will tend to decompose their move-
The classical motor signs and symptoms of cere- ments into simpler, more accurate single joint move-
bellar ataxia are summarized in Table 2. ments.7,36 In addition to showing deficits in
Traditionally, an ipsilateral cerebellar hemi- coordination between the joints in one limb, abnor-
sphere lesion is felt to result in ipsilateral signs. malities in intralimb coordination have also been
This is because the cerebellar hemispheres connect described37–39 (but see also ref. 40).
with the contralateral cerebral cortex via the con- The cerebellum may coordinate the activity in
tralateral ventrolateral nucleus of the thalamus different effectors such as between the eye, arm,
(also termed ventral intermediate nucleus in leg or head.41–45 Deficits in ocular control and
humans25) and the cerebral cortex output; the cor- limb control, for example during a reaching or
ticospinal tract subsequently decussates.26 Recent stepping task, co-vary suggesting that they may
findings, however, suggest that stimulation of the be caused by common difficulties in programming
interpositus nucleus in primates can result in bilat- coordinated movements.46,47 Such interactions
eral limb movements27 and that a unilateral lesion mean that the accuracy of eye and limb move-
in humans can affect both limbs.28 These bilateral ments performed in isolation may be further
symptoms may be mediated via bilateral projec- degraded during coupled activities,46,48,49 as usu-
tions to subcortical sites such as the reticular for- ally occurs during functional activities.
mation. These projections may be useful in
mediating bimanual coordination.
Dysmetria and tremor
Pathophysiology A triphasic pattern of muscle activation con-
sisting of alternating agonist–antagonist–agonist
Limb movement activity is normally observed with fast single
Dyssynergia and incoordination joint movements. Both animal and human studies
People with cerebellar ataxia are slow to start highlight that following cerebellar dysfunction/
movements; they have an increased reaction time. inactivation there may be a prolonged duration

Table 2 Motor signs and symptoms and tests in cerebellar disease
Signs and symptoms of Clinical tests Assess

cerebellar ataxia
Limb movement
Dyssynergia Finger to nose Ability to move joints simultaneously/
Heel shin decomposition of multijoint movements
Dysmetria Finger to finger test Great toe–finger test (have Speed of motion
trunk supported to isolate any limb dysfunction) Variability of spatial path
Over/undershoot
Tremor Holding a position, e.g. (1) arms outstretched Tremor amplitude and frequency
Kinetic with palms down; (2) index to index (hold Assess for titubation 3 Hz tremor of
Intention two index fingers medially) the head
Postural Move to/from target (see tests above)
Assess writing/spiriography
Disdiadochokinesia Alternating pronation–supination Rate of movement
Alternating wrist flexion/extension while tapping
the thigh
Ankle dorsi-plantarflexion
Asthenia and hypotonia Passive motion of the limb Assess resistance to motion
Test muscle strength Assess strength
Balance and gait dysfunction
Balance and gait Stand with eyes open/closed Rate, direction and amplitude of sway
dysfunction Stand in tandem/on one foot ( vision) and phase between upper and
Balance in response to perturbation, volitional lower body
upper/lower limb Size of response to postural perturbation
Walking Walking – accuracy of foot placement/
degree of sway/time in double stance/
stride length/base of support
Oculomotor
Nystagmus (e.g. gaze- Ability to fixate into finger 30 cm away with Ocular alignment/presence of nystagmus
evoked; downbeat; gaze in primary position and when looking at (rhythmic oscillatory movements of the
rebound nystagmus) eccentric targets and on returning gaze to eyes) type/presence of eye movements
Fixation deficits (e.g. midline (e.g. flutter/ocular bobbing)
flutter; macroscopic
oscillations)
Saccadic smooth pursuit Follow a target moving up/down/side to side Number of catch-up saccades
Poor vestibulo-ocular Hold arms together out in front with Ability to maintain gaze fixation
reflex cancellation thumbs pointing up. Fixate gaze on the
thumbs while sitting in a chair that is
moved side to side
Dysmetric saccades Ability to rapidly shift gaze from one eccentric Latency, velocity and precision (over/under-
target to another (up and down/side to side) shoot of the target as seen by the need
to make more than one saccade to reach
the target)
Reduced velocity of diver- Ability to shift gaze from targets close to and far Latency, velocity and precision
gent eye movement away from subject.
Ability to follow targets moving to and from
target
Abnormal vestibulo-ocular Sit on a chair and fixate an earth fixed target Ability to fixate on the target
reflex and optokinetic while the chair rotates side to side Movement of the eyes in the direction
response Look at an optokinetic drum (ask patient to of rotation and re-alignment to the
count the stripes) midline
Dysarthria
Ability to maintain sustained vowel phonation; Intelligibility; rhythm; speed; presence of
repeat syllables hesitations, accentuation of syllables and
Repeat a sentence speech/read a standard pas- addition/omission of pauses
sage of text
Reproduced with kind permission from CRC Press.288

of the initial agonist burst that accelerates the Force generation

limb and a delay in the onset of the subsequent Asthenia, a generalized weakness, was described
antagonist muscle burst.50–54 This antagonist by Holmes (1922). This was more marked in the
burst normally acts to decelerate the movement. acute stages and with extensive cerebellar lesions
Its delay therefore results in the person over- and was not universally seen; indeed normal grip
shooting the target,55 a form of dysmetria. force has been reported in cerebellar ataxia.71
Although delayed, activation of the anatagonist In contrast, a reduced rate of force generation
muscle still occurs. However, instead of now (power) has frequently been reported6,72–74 and
being pre-programmed by the cerebellum it is may in part underlie the difficulty in performing
felt to be activated through stretching via a pos- rapidly alternating movements (disdiadochokin-
sible transcortical stretch reflex. This antagonist esis) and contribute to the inability to adequately
contraction can in turn stretch the agonist, result- compensate for the higher interaction torques that
ing in stretch reflex-related activation of this are generated with faster movements.72,75,76
muscle. Thus, alternating agonist–antagonist con- People with cerebellar ataxia also show more
tractions driven by proprioceptive feedback may variability in maintaining a constant level of
arise, resulting in a tremor.52,56–59 force.77 The deficits in force generation may
Cerebellar tremor is a type of action tremor. It is affect precise manipulative tasks. This, along
seen while maintaining a posture (postural tremor) with poor coupling between grip and load forces
and while moving (kinetic tremor). Tremor at rest and inappropriate finger placement on an object,
is not seen with pure cerebellar dysfunction. can lead to excessive self-generated torques acting
Cerebellar tremor is complex and in addition to on the object.6,78–80
proprioceptive feedback driving tremor other
causes exist. When approaching a target there is
a characteristic increase in tremor amplitude Balance and gait dysfunction
(intention tremor).60 Intention tremor may be Postural sway and balance
explained by the effects of the inherent delay in People with lesions affecting the anterior lobe,
visuomotor processing compounding the pres- vermis and the interconnected fastigial nucleus
ence of an already incoordinated movement.61,62 have an increase in postural sway with an associ-
Visuomotor tracking tasks in people with cerebel- ated head and truncal tremor between 2 and
lar ataxia have shown that the smoothness of 5 Hz.81–86 The sway is greatest in the antero-
tracking is improved when vision of either the posterior direction. Instability in the medio-lateral
target or the operator-controlled cursor is direction is also seen in response to alterations in
removed.63–67 Both conditions prevent visual feed- the available sensory input, following a postural
back about the error between the target and the perturbation or while stepping.87–89
ongoing movement. It seems that people with cer- Impairments in both postural responses and
ebellar ataxia use this visual feedback to try to anticipatory postural adjustments have been
correct any abnormalities in tracking. However, described.90 The temporal coordination between
the inherent delay in the time it takes to process anticipatory postural adjustments and volitional
visual feedback and to produce a motor output movements, for example, is impaired.91 A lack of
(around 120 ms) results in a delayed response such anticipatory adjustments will result in imbal-
during which time the limb may have moved in a ance during self-generated movements, for exam-
non-predictable direction due to the underlying ple, not leaning forwards prior to standing on
ataxia. In this case visually driven corrective tiptoes will result in the person pushing themselves
responses would only serve to compound the inac- backwards.
curacy in the movement, resulting in intention In response to an unexpected perturbation
tremor.68 Although, avoiding visual feedback of the support surface, hypermetric postural
during and towards the end of a movement may responses are observed.87,92–94 This seems to
improve the smoothness of the movement, this reflect an inability to set the correct size (or gain)
strategy may decrease movement accuracy.49,69,70 of the response. Similar to healthy participants,

however, people with cerebellar ataxia are able to cerebellar oculomotor areas: the flocculus/parafloc-
decrease the size of the postural response with culus, the nodulus and the vermis, as each gives rise
repeated presentations of a predictable postural to well-recognized (and recognizable) oculomotor
perturbation and change their response magnitude ‘syndromes’111 that may or may not occur together
appropriately to an expected change in the size of or be associated with other cerebellar signs.111–113
perturbation, although the overall size of the These syndromes can be useful diagnostic clues and
response remains increased.92,95–98 may account for visual symptoms (oscillopsia, dizzi-
ness, diplopia).114 However, due to the heavy con-
nections with the brainstem, oculomotor centres in
Gait and falls the pontomedullary junction (horizontal eye move-
While walking, people with cerebellar dysfunc- ments) and the midbrain (vertical and torsional eye
tion show prolonged time in double stance, poor movements), it is often not possible to differentiate
inter-limb coordination99,100 and an increased var- between brainstem and cerebellar lesions.
iability in their stride length and individual joint The flocculus/paraflocculus controls the modu-
kinematics,101–103 although their base of support lation of retinal image motion (retinal slip) and
may not be increased as is often presumed.104 maintains steady fixation in eccentric gaze
Although primary deficits in balance can have a directions (the neural integrator), accurate
direct and marked impact on walking,105 dysme- smooth pursuit, optokinetic nystagmus and
tria/dyssynergia affecting the lower limbs can also normal vestibulo-ocular reflex gain. Visual input
impact on dynamic balance while walking.99,106 from retina and visual cortex is relayed to the infe-
When one foot is off the ground while walking, rior olive via pretectal nuclei, and uniquely,
the body’s centre of mass does not usually lie Purkinje outputs synapse with brainstem centres
over the base of support and so the body is in a (bypassing the deep nuclei). Cardinal clinical
state of disequilibrium; it will tend to fall away signs of the ‘floccular syndrome’ are gaze-evoked
from the stance leg. The trajectory taken by the nystagmus on lateral gaze and jerky (saccadic)
body while walking is preprogrammed and tightly smooth pursuit (ipsiversive to the lesion).115–121
coupled to the future placement of the stepping Rebound nystagmus may also be present.
foot that acts to catch the ‘falling’ body.107,108 Vestibulo-ocular reflex suppression is impaired
Incorrect foot placement due to incoordination which can be readily detected by the failure of a
of the lower limb, as is seen in cerebellar ataxia, (seeing) patient to suppress post-rotatory vestibu-
can therefore result in poor dynamic balance while lar nystagmus.122–127
walking. A more medial foot placement than Vertical eye movements may be normal, but
required, for example, can result in the body fall- in progressive disorders downbeat nystagmus
ing laterally.99 emerges.128 Acute or chronic alcoholism may
Deficits in balance and walking may contribute yield similar signs and may be irreversible.
to the high reported incidence of falls in cerebellar Downbeat nystagmus can give rise to constant
ataxia.82 However, the causes of falls are often oscillopsia (the illusion of oscillation of the
multifactorial and the relative contribution of visual surround) and poor visual acuity that is
intrinsic and extrinsic (environmental) factors in unrelated to head movement and position.113
falls aetiology in cerebellar dysfunction remains Abnormalities in the direction and in the gain
to be elucidated. (both increases and decreases) of the angular
and linear vestibulo-ocular reflex have been
reported.125,129–135 Abnormalities in the vesti-
Oculomotor control bulo-ocular reflex can degrade visual acuity with
Normal oculomotor function is heavily dependent head motion. Vertical translational head motion is
on the cerebellum for adaptive control (plastic- especially prominent while walking, and abnor-
ity).109,110 It helps to understand cerebellar oculomo- malities in the vertical vestibulo-ocular reflex
tor dysfunction with reference to three major may lead to falls.133

The nodulus (and ventral uvula) Vertigo may be experienced secondary to lesions
Nystagmus is observed when people are rotated affecting the midline vermis and flocculonodular
in the dark at a constant angular velocity; the slow lobe.141,142 Prolonged isolated vertigo with imbal-
phase being generated by the vestibulo-ocular ance that mimics symptoms of vestibular neuritis
reflex with the fast phase serving to reposition may be observed in 10% of cases of discrete cer-
the eyes centrally in the orbit. The head velocity ebellar lesions. These most commonly affect the
signal derived from vestibular nerve afferents medial branch of the posterior inferior cerebellar
is felt to be stored within brainstem circuits. artery territory.143 Vertigo may also be positional
Evidence for this storage mechanism comes from and accompanied by positional evoked nystag-
the observation that with a constant velocity rota- mus,142,144,145 thus mimicking benign paroxysmal
tion in the dark, the velocity of the slow phase positional vertigo. In contrast to benign paroxys-
decays exponentially with a time constant of mal positional vertigo, the nystagmus may be either
15–20 seconds, slower than the decay in vestib- vertical (up or downbeat) or torsional in direction
ular nerve afferent signals.136 with the eyes straight ahead, be persistent and
The nodulus modulates this vestibular time- change in direction with different head positions.146
constant, and lesions thereof tend to increase the Additional signs that may alert the clinician to the
vestibular time-constant beyond normal, and can presence of a central cause of vertigo include the
lead to periodic alternating nystagmus, in which presence of skew deviation, gaze evoked nystagmus
the horizontal jerk nystagmus spontaneously towards the affected ear (in the opposite direction
reverses direction with a period of tens of sec- to that seen with peripheral vestibular neuritis),
onds.111 Periodic alternating nystagmus is often vertical saccadic pursuit; a normal head thrust
missed due to the need to observe nystagmus for test and minimal increase in the slow phase velocity
some minutes. Midline cerebellar lesions can also of sponataneous nystagmus in the dark compared
alter the time constant of perceptual vestibular- to fixation in the light as well as the presence of
mediated self-motion.137 associated neurological signs such as dysmetria/
dysynergia or dysarthria.147,148
The dorsal vermis and fastigial nucleus

The dorsal vermis (lobules 6 and 7) and the fastigial Dysarthria, communication and language
nucleus are important for the calibration of saccades. Dysarthria is commonly observed with lesions
The ‘vermis syndrome’ is characterized by saccade affecting the rostral paravermal region of the ante-
hypo- or hypermetria.122,138,139 Detection of dysme- rior lobes.3,149–151 Impairments in both articu-
tria requires careful observation, but extreme hyper- lation and prosody have been described.152
metria may give rise to saccadic oscillations (distinct Commonly speech is described as scanning in
from nystagmus) and is suggestive of a nuclear lesion. nature consisting of hesitations, accentuation of
Smooth pursuit and slow vergence movements may some syllables and the addition of pauses or omis-
also be affected; in particular the velocity of diver- sion of appropriate pauses; in around 50% of
gence is reduced.140 In children, congenital or cases slurring may be evident.153 In addition,
acquired vermian lesions also lead to difficulties in speech can be reduced in rate, and show signs of
triggering saccades (‘saccade initiation failure’ or vocal instability, increased monotony, equalized
‘congenital ocular motor apraxia’) which is often stress and imprecise consonants.154 Acoustic anal-
associated with speech apraxia due presumably to ysis reveals more variable/longer syllable produc-
co-involvement of vermian speech areas (see section tion and pause durations, reductions in vowel
on Dysarthria, communication and language). length contrasts and differences in the frequency
Saccade speed appears not to be controlled by spectrum from those in healthy controls.154–156
the cerebellum, and saccades that are clinically rec- Speech intelligibility may be affected by difficulties
ognizable as being slow are usually caused by in distinguishing the difference between plosives
brainstem disease or drug toxicities. (e.g. [p], [t], [k]) at the end of words.157

Kinematic analysis of oro-facial movements These symptoms are felt to reflect damage to the
highlights similar changes as previously described extensive reciprocal pathways between the cerebel-
for limb movements, such as prolongation of lum and the posterior parietal, superior temporal,
movement duration; decreased maximal velocity prefrontal and parahippocampal cortices. Thus,
and prolonged muscle bursts.158–160 Furthermore, the role of the cerebellum in planning and ongoing
a 3 Hz ‘postural’ tremor may be detected during control of motor function may therefore have a
sustained phonation of vowels161 and oral disdia- corollary in non-motor behaviours.181
dochokinesia can be detected during rapid syllable Such non-motor symptoms may further impact
repetition,162 although this may not predict the on rehabilitation, for example, by affecting the
syllabic rate during sentence production.163 ability to retain information conveyed through
Language impairments that are not attributed verbal or visual instructions; to perform abstract
to dysarthria, such as poor understanding of reasoning or to initiate activities.183,184
speech, reading and naming tasks, and agramma-
tism have been reported following cerebellar
damage164,165 (although see ref. 166). These are Implications for rehabilitation
felt to arise from disruption to reciprocal path- approaches
ways between the right cerebellar hemisphere and
left cerebral hemisphere and highlight the role of
the cerebellum in pre-articulatory speech.167,168 As highlighted in recent systematic reviews of ran-
Mutism following posterior fossa tumour resec- domized controlled trials there is currently a lack
tion in children has also been described in about of high-quality research studies into the rehabili-
29% of cases.151,169,170 The mutism may not tation of cerebellar ataxia.185,186 Despite this, the
develop immediately and is usually self-limiting.170 literature on the treatment of cerebellar ataxia
Here damage to the vermis has been impli- describes some approaches that warrant further
cated,151,169 although cerebellar damage leading investigation. Approaches may be broadly divided
to a temporary reduction in activity in the inter- into those that aim to improve functional ability
connected cerebral cortex has also been by compensating for the underlying deficit and
suggested.151,171,172 those that aim to improve function through restor-
ative techniques that involve adaptation and
recovery within the neuro-musculoskeletal system.
Non-motor symptoms
In the last two decades accumulating evidence Compensatory approaches
suggests that isolated damage to the cerebellum Compensatory approaches include the use of
may also result in a cerebellar cognitive affective strategies to encourage decomposition of move-
syndrome.173 This syndrome includes non-motor ment into simpler single joint movements187;
symptoms such as poor executive function (e.g. visual and verbal cues to aid walking speed and
reduced verbal fluency and working memory); stride length188,189; the use of assistive technology
impaired spatial cognition (eg poor visuo-spatial to aid computer use190; and aids such as custom-
memory) and linguistic difficulties (e.g. dysproso- ized seating and frames to help posture, balance
dia and agrammatism) and has been described in and mobility.191–193
both acquired and hereditary cerebellar dysfunc- Increasing the visco-elastic resistance or the
tion.174–180 The symptoms are more marked in the inertia of a limb will dampen the tremor and
acute/subacute stages of damage/dysfunction, par- serve to decrease the speed and size of the stretch
ticularly when the posterior lobes of the cerebel- reflex that may drive the tremor in some
lum are affected bilaterally (e.g. following an cases.63,194 Thus aids that increase viscous resis-
infarction within the posterior inferior cerebellar tance such as the Neater Eater and Mouse Trap
artery territory). Further, affective symptoms (http://www.neater.co.uk/main.htm) have been
such as apathy and disinhibited behaviour may recommended although their effectiveness has
occur with damage to the midline vermis.181,182 not been thoroughly investigated. The visco-elastic

resistance offered by Lycra garments195 may also uncontrolled case reports. With prolonged inter-
underlie their proposed use in improving proxi- ventions (3–12 months) improvements in inter-
mal and truncal stability and function in people limb coordination209,210 and a reduction in force
with cerebellar signs. However, any functional variability have been described.191 Biofeedback of
improvements with Lycra garments in reported different aspects of motor control has been
single-case studies to date need to be considered assessed. Biofeedback of EMG patterns during a
alongside the possible inconvenience and addi- visuomotor tracking task led to an improvement
tional assistance required in applying the garment, in muscle activation on the trained task after sev-
leading to a potential loss in independence.196 eral weeks of training although the effects on func-
Increasing inertia by loading the appendicular tional tasks were not investigated.211 Biofeedback
or axial skeleton may also dampen tremor and of muscle activity has also been described in com-
reduce dysmetria.63,197,198 Studies into the effec- bination with relaxation therapy to decrease
tiveness of weights in improving upper limb func- tremor resulting in improved feeding.212,213
tion are variable, with improvements and Biofeedback of the centre of pressure motion (a
deterioration in function as well as a lack of measure of postural sway) linked to a computer
effect being reported.49,199–201 This may be because game resulted in improvement in measures of bal-
the addition of a load requires adaptive scaling of ance and falls and the use of a computer game was
agonist–antagonist activity which is affected in linked to increased practice time.214
cerebellar disease. Thus, although an increase in Other studies have assessed the effect of multi-
agonist activity required to initially accelerate the component approaches on balance and walking.
limb may be seen, this may not be accompanied by Some of these are the subject of recent systematic
an increase in antagonist ‘braking’ activity. reviews.185,186 Balance and ocular exercises have
Ultimately, this results in an increase in overshoot- led to an improvement in clinical measures of pos-
ing and potential deterioration in function.201 In tural stability and walking in people with cerebel-
fact the use of additional load may be beneficial as lar ataxia.215 Although this study involved small
an assessment aid in revealing minimal hyperme- numbers (n ¼ 2), the techniques utilized are based
tria.202 Loading the trunk may aid balance, and on those that are effective in treating deficits
here the loading may be either symmetrical or affecting the peripheral and central vestibular
asymmetrical to counterbalance a directional system with which the cerebellum has large recip-
impairment in balance (e.g. anterior loading is rocal connections. Brown et al.216 also used vestib-
provided to counterbalance falling backwards). ular habituation training in combination with
Although it is not always clear whether the strengthening, stretching and gait re-education.
ataxia is of pure cerebellar origin, case reports of Significant improvements in disability scales
these interventions show a favourable outcome on (Disability Handicap Inventory; Dynamic Gait
clinical measures of walking and balance.197,203,204 Index) were seen in the subgroup of people with
Cooling a limb can also temporarily reduce cerebellar ataxia (n ¼ 10). Increases in walking dis-
cerebellar tremor. This may occur through a tance following either locomotor training using
reduction in muscle thixotropy and increase in body weight support on a treadmill (5 days/week
muscle stiffness as well as a reduction in nerve for four months) or by encouraging people to
conduction velocity and muscle spindle afferent decrease the amount of fixation of the arms
feedback.205,206 Cooling-related reductions in when walking217,218 have been reported.
tremor resulting in functional improvements have Improvements in standing balance have also
also been reported in people with essential been seen with a combination of strength and
tremor.207,208 balance exercises.219,220 Both groups utilized
Frenkel’s exercises that were originally developed
for people with sensory ataxia secondary to tabes
dorsalis (Frenkel, 1902). These exercises emphasize
Restorative approaches a reliance on visual feedback to control movement.
The uses of defined restorative approaches tar- Given that visual feedback can improve balance/
geting a specific symptom are rare and involve postural sway in cerebellar dysfunction,81,221

this may be a useful approach although an over- no control groups.245,246 The mechanisms under-
reliance on vision in cerebellar dysfunction result- lying any functional improvements with stimula-
ing in visual vertigo has been described.222 It would tion of the cerebellum remains unclear given the
be interesting to contrast the reliance of different fact that transcranial magnetic stimulation over
sensations on balance following this approach with the cerebellum may in part affect motor cortex
that seen after the vestibular habituation/rehabili- excitability by stimulating adjacent peripheral
tation exercises described above. nerves.247,248
Several small-scale studies on training ocular Understanding when to apply restorative or
control and dysarthria highlight the potential compensatory strategies remains unclear. The rel-
value of further intervention studies in these ative use of compensatory strategies may vary and
areas. In a stepping task that defined the target possibly depends on disease progression and
for foot placement, Crowdy et al. found that sac- severity.
cadic and foot placement accuracy could be
improved by prior practice in making saccadic
eye movements towards the targets.223 A single-
Potential mechanisms and barriers
case study of a person with cerebellar dysfunction
secondary to thiamine deficiency assessed the to recovery
effects of Lee Silverman voice treatment. This
approach emphasizes loudness of phonation and The potential mechanisms of recovery following
is more commonly used in the treatment of people cerebellar damage have not been extensively stud-
with Parkinson’s disease. After a four-week inter- ied. The results of serial experimental cerebellar
vention period (16 sessions), improvements in lesions in primates suggest that following unilat-
acoustic measures (sound pressure level) and per- eral cerebellar lesions, important structures
ceptual measures of articulation/phonation were mediating recovery are the opposite cerebellar
observed and their employer reported increased hemisphere, deep cerebellar nuclei, as well as
satisfaction over the effectiveness of communica- extracerebellar sites within the brainstem and sen-
tion via the telephone.224 sorimotor cortex, the latter depending on whether
More recently, the use of motor cortical stimu- volitional limb movements or walking/balance is
lation as a treatment paradigm has been assessed. being investigated.249–253 Extrapolation of such
These follow animal and human studies showing findings to humans should be made with caution
that cerebellar dysfunction results in a reduction in due to differences in neuroanatomy.254,255
measures of motor cortex excitability.225–234 Koch A cerebellar lesion may also cause loss of activ-
et al. investigated the effect of rapid transcranial ity within the cerebral cortex due to the large inter-
magnetic stimulation (5 Hz) over the motor cortex. connections between these structures. Resolution
The frequency and paradigm of stimulation used of such disachisis may also underlie symptom
had previously been shown to result in an increase recovery.256,257 In humans with cerebellar dysfunc-
in motor cortex excitability and was associated in tion, for example, an increase in the activation of
the cerebellar ataxic group with an improvement the medial premotor system (supplementary motor
in hand function (as determined by the 9-hole peg area) while moving has been reported; this may
test) compared to healthy controls.235 compensate for the lack of activation of the lateral
Direct stimulation of the cerebellum has premotor areas that receive extensive inputs from
also been investigated. Stimulation of the cerebel- the cerebellum.258 Furthermore, following a uni-
lum using transcranial magnetic stimulation in lateral lesion, imbalances in activity between left
healthy participants has also been shown to mod- and right cerebellum and their interconnected cor-
ulate cortical excitability.236–244 In people with tical structures may also contribute to symptom
spinocerebellar degeneration, cerebellar stimula- presentation. Torriero et al. hypothesized that
tion was applied over several sessions with treat- isolated damage to the left cerebellum may
ment lasting from 21 days to eight weeks. reduce excitatory drive to the contralateral right
Improvements in clinical measures of ataxia, bal- dorsolateral prefrontal cortex, resulting in an
ance and gait were reported although there were imbalance in activity between the left and right

cortex. Re-addressing this imbalance temporarily following damage to other central and peripheral
by inactivating the left dorsolateral prefrontal nervous system structures.
cortex using rapid transcranial magnetic stimula-
tion resulted in an improvement in a procedural
learning task.259 Such findings are in keeping with Motor learning, adaptation and recovery
evidence from subcortical stroke, where changes in The role of the cerebellum in motor learning is
interhemispheric inhibition from the unaffected to being investigated at a subcellular, cellular and sys-
the affected side may contribute to paresis.260 tems level in both animals and humans and using
The cause, site and extent of the lesion are neural networks and modelling.18 The cerebellum is
important predictors of the degree of functional part of a distributed system that is involved in
recovery. Functional deficits seem more marked motor learning and is felt to play a pivotal role in
following a haemorrhage compared to an infarct, error-based motor learning and adaptation.272,273
and superior cerebellar artery strokes have a worse In keeping with this, people with cerebellar ataxia
prognosis than strokes affecting the posterior and may show greatly impaired learning of both simple
anterior inferior cerebellar arteries261,262 (although and complex motor skills such as visuomotor adap-
see ref. 263). Superior cerebellar artery strokes tation,274–276 serial reaction time tasks,277,278 adap-
may be associated with damage to the dentate tation to prism glasses279 and force fields,280 as well
nucleus and the superior cerebellar peduncle, the as classical conditioning paradigms when the inter-
main output pathway of the cerebellum. This is in val between the unconditioned and conditioned
keeping with other studies highlighting worse stimulus is short (400 ms).281–285 Importantly,
functional recovery with lesions affecting the such adaptation is constantly occurring in everyday
output pathways of the deep cerebellar nuclei life. Tasks such as adapting the arm to its new
and superior cerebellar peduncle.70,264 ‘length’ and dynamics when holding an object
Extracerebellar damage seems to be a poor (e.g. a pen), adapting the vestibulo-ocular reflex
prognostic indicator of functional recovery.265 when glasses are put on and adapting to the effects
Functional recovery after a cerebellar stroke, for of muscle fatigue286 with repetitive activities all
example, is worse when people initially present seem to require cerebellar activity.
with global signs such as loss of consciousness/ Poor recovery following a cerebellar lesion may
weakness, as opposed to isolated focal signs such be a consequence of damaging structures critically
as ataxia or vertigo.261,266,267 Following stroke, involved in learning and relearning of motor skills.
increasing age is also associated with worse func- However, following a cerebellar lesion learning
tional outcome,267 although this may in part be and adaptation over time has been described,
explained by the association of age-related addi- albeit at a lower rate and extent than that in
tional extracerebellar white matter lesions.268 healthy controls.73,98,287 It remains to be seen
Against an effect of age on prognosis is the finding whether this reflects activity within undamaged
that the ability to compensate for a cerebellar areas of the cerebellum or activity in extracerebel-
tumour post resection does not seem to be better lar structures.
in young children (54 years old) than in older chil-
dren, adolescents and adults.264,269
In other pathologies the presence of additional Conclusion
cerebellar dysfunction strongly impacts on recov-
ery. The recovery from a peripheral vestibular An understanding of the pathophysiology of cere-
nerve lesion and the recovery from a brainstem bellar ataxia and the mechanisms of recovery can
stroke is less pronounced in the presence of an help to guide the development of treatments and
additional cerebellar lesion.270,271 This may to optimize current interventions. Interpreting
simply reflect an increasing accumulation of studies of pathophysiology in turn crucially
pathology. However, it may also reflect that the depends on our understanding of the healthy cer-
cerebellum normally plays an important role in ebellum in both motor and non-motor functions.
motor learning and the recovery of symptoms Although there is currently a lack of high-quality

research studies into the rehabilitation of cerebel- 3 Timmann D, Brandauer B, Hermsdorfer J et al.
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Cerebellar ataxia: Pathophysiology and rehabilitation

Article in Clinical Rehabilitation · March 2011

Jon Marsden Chris M Harris

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Cerebellar ataxia: pathophysiology and rehabilitation

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Cerebellar ataxia: pathophysiology and rehabilitation

This series of articles for rehabilitation in practice aims to cover a knowledge

Introduction Cochrane Library with the search terms ‘cerebel-

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Table 2 Motor signs and symptoms and tests in cerebellar disease

Signs and symptoms of Clinical tests Assess

Reproduced with kind permission from CRC Press.288

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of the initial agonist burst that accelerates the Force generation

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The dorsal vermis and fastigial nucleus

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79 Muller F, Dichgans J. Dyscoordination of pinch 94 Diener H-C, Dichgans J, Bacher M,

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