Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_24_библиотеки_им_акад_М_И_Перельмана
.pdf
500
https://t.me/medicina_free
K. Bürk
May S, Gilman S, Sowell BB etal (2007) Potential outcome measures
and trial design issues for multiple system atrophy. Mov Disord
22:2371–2377
Palma JA, Vernetti PM, Perez MA et al (2021) Limitations of the
Unied Multiple System Atrophy Rating Scale as outcome measure
for clinical trials and a roadmap for improvement. Clin Auton Res
31(2):157–164. https://doi.org/10.1007/s10286- 021- 00782- w
Patel M, Isaacs CJ, Seyer L et al (2016) Progression of Friedreich
ataxia: quantitative characterization over 5 years. Ann Clin Transl
Neurol 3:684–694
Rascol O, Cochen de Cock V, Pavy-Le Traon A etal (2021) Fluoxetine
for the Symptomatic Treatment of Multiple System Atrophy: the
MSA-FLUO trial. Mov Disord 36(7):1704–1711. https://doi.
org/10.1002/mds.28569
Ristori G, Romano S, Visconti A et al (2010) Riluzole in cerebel-
lar ataxia: a randomized, double-blind, placebo-controlled pilot
trial. Neurology 74(10):839–845. https://doi.org/10.1212/
WNL.0b013e3181d31e23
Rummey C, Corben LA, Delatycki MB etal (2019) Psychometric prop-
erties of the Friedreich Ataxia Rating Scale. Neurol Genet 5(6):371.
https://doi.org/10.1212/NXG.0000000000000371
Schmahmann JD, Gardner R, MacMore J etal (2009) Development of a
Brief Ataxia Rating Scale (BARS) Based on a Modied Form of the
ICARS. Mov Disord 24(12):1820–1828. https://doi.org/10.1002/
mds.22681
Schmitz-Hübsch T, Coudert M, Bauer P etal (2008a) Spinocerebellar
ataxia types 1, 2, 3, and 6: disease severity and nonataxia symptoms. Neurology 71(13):982–989. https://doi.org/10.1212/01.
wnl.0000325057.33666.72
Schmitz-Hübsch T, du Montcel ST, Baliko L etal (2006a) Scale for
the assessment and rating of ataxia: development of a new clinical scale. Neurology 66(11):1717–1720. https://doi.org/10.1212/01.
wnl.0000219042.60538.92
Schmitz-Hübsch T, Fimmers R, Rakowicz M et al (2010)
Responsiveness of different rating instruments in spinocerebellar
ataxia patients. Neurology 74(8):678–684. https://doi.org/10.1212/
WNL.0b013e3181d1a6c9
Schmitz-Hübsch T, Giunti P, Stephenson DA et al (2008b) SCA
Functional Index: a useful compound performance measure for
spinocerebellar ataxia. Neurology 71(7):486–492. https://doi.
org/10.1212/01.wnl.0000324863.76290.19
Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L et al (2006b)
Reliability and validity of the International Cooperative Ataxia
Rating Scale: a study in 156 spinocerebellar ataxia patients. Mov
Disord 21(5):699–704. https://doi.org/10.1002/mds.20781
Schoch B, Regel JP, Frings M etal (2007) Reliability and validity of
ICARS in focal cerebellar lesions. Mov Disord 22(15):2162–2169.
https://doi.org/10.1002/mds.21543
Schwab R, England A (1969) Projection technique for evaluating sur-
gery in Parkinson’s disease. In: Gillingham F, Donaldson I (eds)
Third symposium on Parkinson’s disease. Livingstone, Edinburgh,
pp152–175
Storey E, Tuck K, Hester R etal (2004) Inter-rater reliability of the
International Cooperative Ataxia Rating Scale (ICARS). Mov
Disord 19(2):190–192. https://doi.org/10.1002/mds.10657
Subramony SH, May W, Lynch D et al (2005) Measuring
Friedreich ataxia: interrater reliability of a neurologic rating
scale. Neurology 64(7):1261–1262. https://doi.org/10.1212/01.
WNL.0000156802.15466.79
Tai G, Corben LA, Woodcock IR etal (2021) Determining the Validity
of Conducting Rating Scales in Friedreich Ataxia through Video.
Mov Disord Clin Pract. 8(5):688–693. https://doi.org/10.1002/
mdc3.13204
Tezenas du Montcel S, CharlesP GC et al (2012) Factors inuenc-
ing disease progression in autosomal dominant cerebellar ataxia
and spastic paraplegia. Arch Neurol 69(4):500–508. https://doi.
org/10.1001/archneurol.2011.2713
Thompson R, Bhatti DE, Hellman A etal (2020) Ataxia Prevalence in
Primary Orthostatic Tremor. Tremor Other Hyperkinet Mov (NY)
10:54. https://doi.org/10.5334/tohm.570
Tison F, Yekhlef F, Balestre E etal (2002) Application of the International
Cooperative Ataxia Scale rating in multiple system atrophy. Mov
Disord 17(6):1248–1254. https://doi.org/10.1002/mds.10290
Trouillas P, Takayanagi T, Hallett M et al (1997) International
Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology
Committee of the World Federation of Neurology. J Neurol Sci
145(2):205–211. https://doi.org/10.1016/s0022- 510x(96)00231- 6
Velázquez-Pérez L, Cerecedo-Zapata CM, Hernández-Hernández
O, Martínez-Cruz E, Tapia-Guerrero YS, González-Piña R,
Salas-Vargas J et al (2015) A comprehensiveclinical and genetic
study of a large Mexican population with spinocerebellarataxia
type 7. Neurogenetics 16(1):11–21. https://doi.org/10.1007/
s10048- 014- 0424- y
Wenning GK, Tison F, Seppi K et al (2004) Multiple System
Atrophy Study Group. Development and validation of the Unied
Multiple System Atrophy Rating Scale (UMSARS). Mov Disord
19:1391–1402
Wenning GK, Geser F, Krismer F et al (2013) The natural his-
tory of multiple system atrophy: a prospective European cohort
study. Lancet Neurol 12(3):264–274. https://doi.org/10.1016/
S1474- 4422(12)70327- 7
Weyer A, Abele M, Schmitz-Hübsch T et al (2007) Reliability and
validity of the scale for the assessment and rating of ataxia: a study
in 64 ataxia patients. Mov Disord 22(11):1633–1637. https://doi.
org/10.1002/mds.21544
Winser S, Smith CM, Hale LA etal (2017) Psychometric properties of
a core set of measures of balance for people with cerebellar ataxia
secondary to multiple sclerosis. Arch Phys Med Rehabil 98(2):270–
276. https://doi.org/10.1016/j.apmr.2016.07.023
Yabe I, Matsushima M, Soma H etal (2008) Usefulness of the Scale
for Assessment and Rating of Ataxia (SARA). J Neurol Sci 266(1-
2):164–166. https://doi.org/10.1016/j.jns.2007.09.021
The Ataxic Gait
https://t.me/medicina_free
PierreCabaraux andMarioManto
77
Abstract
Cerebellum plays a key role in the control of human gait.
Cerebellar circuitry and its interconnected structures control timing and synergy of muscle contractions in order to
adapt gait to environmental changes, likely by implementing and updating internal models. Ataxic gait is one
of the most disabling symptoms occurring in cerebellar
disorders and tandem gait is one of the most sensitive tests
to assess gait during clinical routine. Several instruments
have been developed to quantify gait ataxia, based on
accelerometers, gyroscopes, surface EMG recordings,
complex systems, which are video based and more
recently the Kinect system. Wearable sensors are emerging. Rehabilitation aims to improve gait ataxia by providing intense training. Novel techniques are being applied
to improve balance and gait in cerebellar patients.
Keywords
Cerebellum · Ataxia · Ataxic gait · Posture · Balance
77.1 Introduction
posed the cerebellum as the center of equilibrium and
William Alexander Hammond (1828–1900) described what
will further be dened as ataxic gait: the “drunken gait” in
patients suffering from cerebellar disorders (Fine etal. 2002;
Margolesky etal. 2019). Since these seminal contributions,
major breakthrough have been reached in the understanding
of the anatomy and pathophysiology of ataxic gait thanks to
animal studies, progress of neuroimaging techniques and
detailed clinical studies.
Cerebellum is responsible for the complex integration of
multiple postural information coming from vestibular, ocular, and proprioceptive inputs. Cerebellar circuitry regulates
static and dynamic parameters leading to a regular stance/
gait (Manto and Habas 2013). Ataxic gait remains one of the
most disabling symptoms in patients suffering from cerebellar disorders, with potential serious complications for daily
life such as loss of autonomy and falls. Although the clinical
features are well described (Buckley etal. 2018), gait is usually not assessed in detail in common clinical practice and
tailored rehabilitation techniques are often lacking.
In this chapter, we will focus on ataxic gait due to cerebellar circuitry impairment. We will present the anatomophysiological fundamental aspects of ataxic gait, the current
methods of assessment and rehabilitation.
The term ataxic gait refers to gait impairments observed in
vestibular, proprioceptive, and/or cerebellar disorders. The
role of cerebellum in posture and gait was rst pointed out by
Luigi Rolando (1773–1831) and Marie-Jean-Pierre Flourens
(1794–1867) two centuries ago through observations made
on animals. Later, François Magendie (1783–1855) pro-
P. Cabaraux (*)
Department of Neurology, CHU de Charleroi, Charleroi, Belgium
M. Manto
Department of Neurology, CHU de Charleroi, Charleroi, Belgium
Department of Neurosciences, University of Mons, Mons, Belgium
e-mail: mmanto@ulb.ac.be
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
D. L. Gruol et al. (eds.), Essentials of Cerebellum and Cerebellar Disorders, https://doi.org/10.1007/978-3-031-15070-8_77
77.2 Anatomical andFunctional
Substratum ofAtaxic Gait
77.2.1 The Neuroanatomical Basis ofBalance
andGait
Balance is a phenomenon under the control of a complex
neuronal network at the brain level encompassing mainly the
thalamus, basal ganglia, hippocampus, inferior parietal cortex, frontal lobe regions, reticular formation, pontine nuclei,
and cerebellum. These structures participate to motor behavior, sensory integration, cognition, and memory (Surgent
501
502
https://t.me/medicina_free
P. Cabaraux and M. Manto
Fig. 77.1 Inspired from Grimaldi and Manto (2012) and from
Cabaraux etal. (2022). Scheme of anatomical connections of the cerebellar circuitry. Cerebellum is widely interconnected with cerebral cortex, brainstem nuclei (including reticular and vestibular nuclei), and
spinal cord through different loops. These connections allow cerebel-
etal. 2019; Dijkstra etal. 2020). Gait is mainly an automatic
process evolving in the central pattern generators (CPG) of
the spinal cord once initiated voluntary at the encephalic
level. The pattern is modulated intentionally or unconsciously by cerebral structures as the subject needs to change
rhythmic limbs movements, adapt muscle tone, or face
expected/unexpected obstacles (Takakusaki 2013).
lum to integrate sensory inputs generated by the different postural
effectors required for the execution of tandem gait. Green arrows: excitatory signals. Red arrows: inhibitory signals. RST reticulospinal tract;
VST vestibulospinal tract; Rub.ST rubrospinal tract; CF climbing bers;
MF mossy bers; NCL nucleo-cortical loop; STN sub-thalamic nucleus
Cerebellum plays a major role through its multiple connections with cortical and subcortical nodes (Fig. 77.1),
mainly by adjusting at a motoric level the postural and/or
gait plan to afferent sensory information and by implementing or updating predictive internal models. In particular, the
cerebellum is widely interconnected with frontoparietal networks, as shown by mental imagery. The anterior lobe and
77 The Ataxic Gait
https://t.me/medicina_free
503
vermis participate to kinematic simulation of gait and associated stance. Lobules 6 and 7 contribute to executive control,
vividness of mental imagery, and complexity of the task
(Wagner etal. 2008).
In a seminal work, Bastian etal. described a series of 5
children presenting an isolate impairment of tandem gait
after a resection of posterior inferior cerebellar vermis
(Bastian etal. 1998). The authors proposed that the interruption of parallel bers crossing the midline and connecting
Purkinje cells from both cerebellar hemispheres was the
main explanation of incoordination observed during tandem
gait. These observations have underlined the importance of
communications within the cerebellar cortex (linking process) through distances of several millimeters. As Purkinje
cells target cerebellar nuclei, parallel bers ensure communications within distinct portions of cerebellar nuclei.
77.2.2 Functional Role ofCerebellum andIts
Implication inAtaxic Gait: Internal
Models
Cerebellum participates to sensorimotor coordination of
limbs movement and is instrumental for sequencing (Leggio
and Molinari 2015). Cerebellar patients show impairments in
tasks requiring precise timing and synergy of muscle contractions, the cerebellum contributing to the complex tuning
of alternate contractions versus co-contractions. Timing
commands and modulation of magnitudes of muscle contractions are fundamental parameters for intralimb and interlimb joint coordination (Manto and Habas 2013). This is
particularly relevant for gait and balance, where hip and
trunk stabilization are needed to avoid body oscillations and
ensure accurate successive placements of the feet on the
oor, often under visual guidance (Chini etal. 2017).
Compelling evidence indicates that the cerebellum plays
a role in motor predictions using internals models (Manto
and Habas 2013). Two main processes have been
hypothesized:
– The forward internal model refers to the fact that cerebel-
lum computes prediction taking into account the expected
sensory state and actual sensory state, using efference
copies of motor signals in order to adapt motor behavior
(D’Angelo 2018). Predictions are necessary because the
cerebral cortex cannot respond on time solely on the basis
of sensory and perceptual feedback.
– The inverse model is the capacity of cerebellum to trans-
form a desired outcome or effector state into the motor
commands (Lawrenson etal. 2018).
It is currently assumed that the cerebellum stores sensorimotor sequences in its complex network of microzones and
modules, with a prominent participation of working memory.
Cerebellar circuitry compares stored sequences with the
sequences generated by the motor cortex, as pointed by
Molinari (Molinari 2016). If the incoming sequence matches
with the stored motor plan, cerebellum will expect a pattern
memorized and which will lead to an anticipated response.
In case of a mistmatch, an error will be generated and the
prediction system will be corrected, likely by changes in the
discharges of complex spikes originating in the inferior olivary complex. In other words, the mismatch will recalibrate
the forward model (Molinari 2016; Lawrenson etal. 2018).
Patients affected by cerebellar ataxia exhibit impairments
of timing and spatial synergy in muscles contractions (Bareš
etal. 2019). Temporal processing is currently viewed as a
major function of cerebellum (Bareš etal. 2019). Sensory
information such as unexpected stimuli are projected to the
cerebellum via the mossy/climbing ber systems, providing
a temporally constrained mechanism to adapt the ongoing
behavior and change the processing accordingly. Cerebellar
patients show difculties to adjust their gait and balance patterns, while facing unexpected obstacles and postural disturbances. This is interpreted as a dysfunction in the predictive
feedforward control.
77.3 Assessment ofAtaxic Gait
77.3.1 Clinical Assessment
The general assessment of ataxic gait in practice is based
upon a detailed clinical appraisal. Clinical features of cerebellar ataxic gait are well known. They include a wide base
of support with increased double-limb support duration,
unsteadiness, irregular steps, trunk instability with attempts
to stabilize the body, difculties with interlimb or intralimb
coordination, increased variability of steps, reduced step
length and speed, as well as impaired cadence and lateral
veering (Buckley etal. 2018). Ataxic gait due to cerebellar
disorder has to be differentiated from vestibular, proprioceptive, or psychogenic ataxic gait (Table77.1).
Regarding tandem gait, the patient is asked to walk in a
straight line with one foot put immediately and sequentially
in front of the other (Fig.77.2) (Manto and Habas 2013). In
early stages of cerebellar ataxia, impairments of tandem gait
are often observed. Indeed, this sensorimotor task is likely
the most sensitive test to detect signs of ataxic gait at an early
phase (pre-ataxic stage). It is an easy-to-perform test in medical ofce. Patients themselves often consider the task as
highly challenging, even if they do not suffer from cerebellar
ataxia. Tandem gait is also abnormal in many extra- cerebellar
conditions such as orthopedic disorders, vestibular and proprioceptive dysfunction (including peripheral neuropathies),
and even in the elderly, hence need to dene the normal patterns as a function of aging (Margolesky and Singer 2017).
Furthermore, co-morbidities are common with in elderly
504
https://t.me/medicina_free
Table 77.1 Differential diagnosis of gait ataxia
Cerebellar gait Proprioceptive gait Vestibular gait Psychogenic gait
Causes Unilateral or bilateral cerebellar
disorder
Worsening with
eye closed
Specic gait
pattern
Associated
clinical signs
and symptoms
Specic
paraclinical
evaluation
+ +++ +++ - or +
– Step length variable
– Truncal instability
– Deviation depending on
lesion side (ipsilateral if
unilateral, not lateralized if
bilateral)
– Vertigo, oscillopsia,
cephalalgia, nausea/vomiting
– Skew deviation,
multidirectional/pure horizontal
or vertical nystagmus, impaired
ocular pursuit
– Limb ataxia, dysarthria
– Other signs of cerebral
impairment
– Brain MRI
– Auditory (AEP) and
somesthetic evoked potentials
(SSEP)
Peripheral nerve disease,
dorsal spinal column
disorder
– Stomping gait
– No truncal instability
– No laterodeviation
– Sensory loss in
extremities
– Distal amyotrophy
– Diminished vibratory
sense or joint position in
lower extremities
– Diminished of
abolished tendon reexes
– Somesthetic evoked
potentials (SSEP)
– ENMG
Unilateral or bilateral
vestibular disorder
– Deviation side depending
on lesion side (ipsilateral if
unilateral, not lateralized if
bilateral)
– Vertigo, oscillopsia, nausea/
vomiting, tinnitus, hearing loss
– Horizonto-rotatory
nystagmus, HST +, HIT +
– Unterberger Fukuda signs
positive
– VNG
– Caloric testing
– VHIT
– VEMP
P. Cabaraux and M. Manto
Psychiatric disorder
– Fluctuating in
intensity
– Spontaneous
remissions
– Non-reproducible
– Abrupt in onset
– Rare falls
– Inconsistency of
gait
– Distractibility
– No signs of actual
structure damage
– Somatizations
– Search for a
compensation
– Self-inicted
injuries
– Uneconomic
postures
– Normal or not
relevant paraclinical
ndings
Fig. 77.2 A healthy subject performing tandem gait on a pressure platform. From Cabaraux etal. (2022)
patients. An important point concerning tandem gait evaluation is that there is currently no consensus on how to evaluate
this task in terms of number of steps, effects of eye closure,
and positioning of upper limb stick to the trunk.
Ataxic gait and tandem gait evaluation are part of the
main rating scales assessing cerebellar patients such as
International Cooperative Ataxia Rating Scale (ICARS)
(Trouillas etal. 1997), Brief Ataxia Rating Scale (BARS)
(Schmahmann etal. 2009), or Scale for the Assessment
and Rating of Ataxia (SARA) scale (Marquer etal. 2014).
The items reported in each scales are presented in
Table77.2.
77 The Ataxic Gait
https://t.me/medicina_free
Table 77.2 Scoring gait with the most commonly used ataxia rating scales
Scales References Items Score
ICARS Trouillas etal. (1997) Postural and gait disturbances, walking
capacities
BARS Schmahmann etal. (2009) Postural and gait disturbances, walking
capacities
SARA Marquer etal. (2014) Gait Gait: 0–8
Walking capacities: 0–8
Gait speed: 0–4
Standing capacities: 0–6
Spread of feet: 0–4
Body sway: 0–4
Gait: 0–8
Stance: 0–6
505
77.3.2 Paraclinical Assessment ofAtaxic Gait
Multiple quantitative tools have been developed to evaluate
gait. However, most of these methods are complex and
restricted to laboratories, although wearable sensors are
emerging as an easy solution for daily use in real life.
Sophisticated techniques are helpful in providing quantitative
information allowing to monitor objectively patients, and even
to extract biomarkers which complement uid assessments or
neuroimaging data (Ilg etal. 2016; Manto etal. 2021).
Complex motion capture systems including videos synchronized with multiple sensors xed on the skin are available in gait laboratories. They combine tools to extract the
biomechanics of gait in 3 dimensions. Considering tandem
gait, measures of the center of pressure (CoP) traveled way
on force platform may represent an accurate evaluation
(Fig.77.3). This technique has shown its usefulness to detect
gait pattern decits in metabolic disorders leading to falls
(Renneboog etal. 2006).
Accelerometers are wearable devices directly sticked to
the skin. Accelerometry is becoming the leading sensor
modality to assess gait. The sensors record acceleration in
static conditions such as gravity tasks, or dynamic conditions
such as walking (Mathie etal. 2004). Gait parameters including the number of steps, step interval (or cadence), gait variability, and asymmetry are easily extracted (Suzuki et al.
2018). Long-term recordings using wearable accelerometers
identify ataxic gait features, including impaired inter-joint
coordination and increased variability of gait temporal and
kinetic parameters (Shirai etal. 2019). Gyroscopes are also
relevant for the assessment of gait. Other wearable devices
such as surface electromyography (sEMG) are commonly
combined with kinematic sensors (Mitoma et al. 2000).
Indeed, assessment of muscle discharges shows impairments
of muscular contraction patterns in cerebellar patients, such
as prolonged and shifted muscle activations related to compensatory mechanisms as well as increased agonist–antagonist single-joint and whole-limb muscle coactivation, likely
in an attempt to stabilize joints (Mari etal. 2014; Martino
etal. 2015; Fiori etal. 2020). The Kinect system (Fig.77.3),
initially developed for entertainment, is a low cost technology used to analyze human motor functions including gait
(Eltoukhy etal. 2017). The system requires no sensor on the
skin. A recent study proposed its use in the evaluation of
ataxic patients, suggesting the Kinect system for a digitalized
SARA scale (Summa etal. 2020).
506
https://t.me/medicina_free
P. Cabaraux and M. Manto
a
b
c
Fig. 77.3 Example of tandem gait performed by (a) a patient without
cerebellar ataxia, (b) a patient presenting a hemispheric cerebellar
stroke, and (c) a patient with spinocerebellar ataxia type 6 (c). The three
patients perform a 6-path-long tandem gait starting and nishing both
feet tied to each other. The total traveled ways were recorded for the
three patients and were, respectively, 1763mm for patient a, 2475mm
77.4 Ataxic Gait inSpecic Disorders
oftheCerebellum
Ataxic gait is common when the cerebellar structure is damaged. The underlying disorder may present with an acute (for
instance cerebellar stroke), a subacute (for instance paraneoplastic cerebellar ataxia), or a slowly progressive course (for
instance the so-called degenerative ataxias such as the auto-
for patient b, and 7793mm for patient c. While patient a made no mistake and kept its center of pressure stabilized during the task, the cerebellar patients b and c made multiple mis-steps (showed by arrows) and
were unable to control their center of pressure. L lateral; A anterior; P
posterior. From Cabaraux etal. (2022)
somal dominant spinocerebellar ataxias SCAs discussed
below). The numerous disorders leading to ataxic gait are
discussed elsewhere in this volume.
Common causes of progressive ataxia are represented in
particular by hereditary ataxias, such as autosomal dominant
spinocerebellar ataxias (SCAs), recessive ataxias such as
Friedreich’s ataxia, mitochondrial or X-linked disorders
(Manto and Habas 2013). In such diseases, gait disturbance
77 The Ataxic Gait
https://t.me/medicina_free
507
often presents as the inaugural sign (Luo etal. 2017). Tandem
gait has been shown to be an interesting sensitive test to
assess preclinical stages or pauci-symptomatic cases
(Velázquez-Pérez etal. 2021). Ataxic gait in these diseases
may thus constitute an early biomarker for the evaluation of
novel therapies, of rehabilitation or for the follow-up, while
some studies suggest that small differences may be evaluated
as steps in disease severity (Ilg etal. 2020).
Ataxic gait is also observed in patients suffering from
Essential Tremor (ET) and orthostatic tremor (OT).
Presentation of gait ataxia in ET is similar to that found in
other cerebellar patients, although some ET patients do not
show timing control impairment of gait (Rao and Louis
2019). ET patients can manifest with various degrees of
severity of gait decits during the course of the disease (Rao
and Louis 2019). Balance difculties remain prevalent in ET
patients (Louis etal. 2013). Balance impairments, lower balance condence, and greater number of falls are signicant
predictors of mortality in ET (Zubair etal. 2018). Growing
evidence based on clinical, neuroimaging, and postmortem
studies suggest a range of degenerative changes in the ET
cerebellum, especially the cerebellar cortex (Louis and Faust
2020).
OT patients also manifest ataxic gait patterns similar to
those of other cerebellar patients. OT patients experience
commonly balance dysfunction such as truncal and gait
ataxia, a wide base stance, impaired tandem gait, and positive pull test. The gait dysfunction tends to increase over
time similarly to most ataxias (Vijiaratnam etal. 2018) but
falls are more common in the progressive ataxias (Fonteyn
et al. 2010). BARS has been used to score gait decits
(Thompson etal. 2020). OT has been associated with cerebellar dysfunction such as cerebellar cortical degeneration
(Benito-León and Domingo-Santos 2016; Setta etal. 1998)
and alterations of the main afferent proprioceptive pathway
including spinal cord and peripheral nervous system
(Whitney etal. 2018). Some argue that OT, including truncal
oscillations, would alter proprioceptive signaling to the CNS
leading to a sensory ataxia (Möhwald etal. 2020).
77.5 Current Therapies toCompensate
Ataxic Gait
Ataxic gait commonly leads to falls with all its potential
negative consequences (Schniepp etal. 2014). Rehabilitation
remains the principle method to improve ataxic gait. It
includes balance training, muscle strengthening, cycling
training, compensatory orthotics and aids, respiratory muscle training, and treadmill training (Kelly and Shanley 2016).
Cerebellar patients may benet from intense and repetitive
rehabilitation. Suitable interventions leading to activation of
balance control and limbs coordination may improve ataxia
(Kelly and Shanley 2016). Indeed, working on proprioceptive, visual, and vestibular inputs often reduces the
unsteadiness.
Currently, rehabilitation techniques encompass mainly
balance and walking training. New motivational methods
include virtual rehabilitation with exergames, mimicking
real-world activities and that can be practiced at home
(Synofzik and Ilg 2014). New rehabilitation techniques are
promising to serve as assistive tools for daily life and rehabilitation of cerebellar patients. The so-called robotic rehabilitation induces motor cortex neuroplasticity by intensive,
repetitive, and task-oriented motor activities (Esquenazi
and Talaty 2019). While stationary systems such as treadmills work on automatization of traditional therapies,
robotic- assisted gait training (RAGT) (Fig. 77.4) allows
patients to execute a wider range of movement while
remaining secure (Calabrò etal. 2016). RAGT shows better
functional improvements when added to other technologies
such as non-invasive brain stimulation (Portaro etal. 2019).
This last approach has also shown independently its effectiveness in many types of cerebellar ataxias from degenerative or genetic causes particularly for gait (Chen et al.
2021). The technique is based on neuromodulation of cer-
ebellar cortex by reestablishing Purkinje cells ring dysfunction, and therefore restores the critical role of
cerebellum within the cerebellar- thalamo- cortical loop
(Manto and Ben Taib 2008).
508
https://t.me/medicina_free
P. Cabaraux and M. Manto
Fig. 77.4 Examples of devices developed for robotic-assisted gait training (RAGT). From Cabaraux etal. (2022)
Finally, dynamic movement orthoses (DMO) may provide an interesting assistance for people suffering from
ataxic gait. Made of elastic bers, these wearable devices
allow complete range of movement, while stabilizing
lower limbs and trunk and stimulating proprioception.
DMO have been shown to improve gait parameters
(Rennie etal. 2000) but scientic evidences remain weak
(Wells etal. 2018).
impaired tandem gait. Accelerometers, surface EMG, and
Kinect system may provide interesting information in ataxic
gait evaluation but required renements.
Rehabilitation interventions remain the classical therapy
to compensate for ataxic gait. Static and movable robotic
training are a promising approach to complement conventional rehabilitation especially when added on to conventional training and/or neuromodulation of the cerebellum.
Wearable dynamic orthoses represent a potential aid to assist
gait, reducing body sway, and stabilizing the trunk and lower
77.6 Conclusions
Since the contributions of Luigi Rolando and Marie-Jean-
limbs without restraining the movement.
Declaration of funding No specic funding
Pierre Flourens, the understanding of ataxic gait has continuously increased. Gait and balance require activation of
multiple parts of the brain including cerebellum. Based on
Conict of Interests The authors declare no conict of
interest.
proprioceptive, exteroceptive, visual, and vestibular feedbacks, cerebellar circuitry and its interconnected structures
Ethical Committee Request Not applicable
control timing, synergy, and adaptation of muscular contractions to adjust tone and movement to environmental changes
by updating internal models.
Consent for Publication All authors have approved the
nal version of the manuscript.
Ataxic gait is among the rst cerebellar decits in cerebellar disorders such as degenerative ataxias and its evaluation may be used as a physiological biomarker in future
Data Availability The concepts discussed in this article are
not based on raw data.
trials. Although clinical features of ataxic gait are well
known, the quantitative assessment remains a matter of
research. Early stages of cerebellar disorders often show
Code Availability There is no software application or cus-
tom code associated with the article.
77 The Ataxic Gait
https://t.me/medicina_free
509
References
Bareš M, Apps R, Avanzino L, Breska A, D’Angelo E, Filip P, Gerwig
M, Ivry RB, Lawrenson CL, Louis ED, Lusk NA, Manto M, Meck
WH, Mitoma H, Petter EA (2019) Consensus paper: decoding the
contributions of the cerebellum as a time machine, from neurons
to clinical applications. Cerebellum (London, England) 18(2):266–
286. https://doi.org/10.1007/s12311- 018- 0979- 5
Bastian AJ, Mink JW, Kaufman BA, Thach WT (1998) Posterior
vermal split syndrome. Ann Neurol 44(4):601–610. https://doi.
org/10.1002/ana.410440405
Benito-León J, Domingo-Santos Á (2016) Orthostatic tremor: an update
on a rare entity. Tremor Other Hyperkinet Mov 6(0):411. https://doi.
org/10.5334/tohm.324
Buckley E, Mazzà C, McNeill A (2018) A systematic review of the
gait characteristics associated with cerebellar ataxia. Gait Posture
60:154–163. https://doi.org/10.1016/j.gaitpost.2017.11.024
Cabaraux P, Agrawal SK, Cai H, Casali C, Damm L, Doss S, Habas C,
Horn AKE, Ilg W, Louis ED, Mitoma H, Monaco V, Petracca M,
Ranavolo A, Rocco SC, Ruggieri S, Schirinzi T, Serrao M, Summa S,
Strupp M, Surgent O, Synofzik M, Tao S, Teras H, Torres-Russotto
D, Travers B, Roper AJ, Manto M (2022) Consensus paper: ataxic
gait. Cerebellum. https://doi.org/10.1007/s12311- 022- 01373- 9
Calabrò RS, Cacciola A, Bertè F, Manuli A, Leo A, Bramanti A, Naro A,
Milardi D, Bramanti P (2016) Robotic gait rehabilitation and substitution devices in neurological disorders: where are we now? Neurol
Sci 37(4):503–514. https://doi.org/10.1007/s10072- 016- 2474- 4
Chen TX, Yang CY, Willson G, Lin CC, Kuo SH (2021) The ef-
cacy and safety of transcranial direct current stimulation for cerebellar ataxia: a systematic review and meta-analysis. Cerebellum
(London, England) 20(1):124–133. https://doi.org/10.1007/
s12311- 020- 01181- z
Chini G, Ranavolo A, Draicchio F, Casali C, Conte C, Martino G,
Leonardi L, Padua L, Coppola G, Pierelli F, Serrao M (2017) Local
stability of the trunk in patients with degenerative cerebellar ataxia
during walking. Cerebellum (London, England) 16(1):26–33.
https://doi.org/10.1007/s12311- 016- 0760- 6
D’Angelo E (2018) Physiology of the cerebellum. In: The cerebellum:
from embryology to diagnostic investigations. Elsevier, pp85–108.
https://doi.org/10.1016/b978- 0- 444- 63956- 1.00006- 0
Dijkstra BW, Bekkers E, Gilat M, de Rond V, Hardwick RM,
Nieuwboer A (2020) Functional neuroimaging of human postural
control: a systematic review with meta-analysis. Neurosci Biobehav
Rev 115:351–362. https://doi.org/10.1016/j.neubiorev.2020.04.028
Eltoukhy M, Kuenze C, Oh J, Jacopetti M, Wooten S, Signorile J (2017)
Microsoft kinect can distinguish differences in over-ground gait
between older persons with and without Parkinson’s disease. Med
Eng Phys 44:1–7. https://doi.org/10.1016/j.medengphy.2017.03.007
Esquenazi A, Talaty M (2019) Robotics for lower limb rehabilita-
tion. Phys Med Rehabil Clin N Am 30(2):385–397. https://doi.
org/10.1016/j.pmr.2018.12.012
Fine EJ, Ionita CC, Lohr L (2002) The history of the development of
the cerebellar examination. Semin Neurol 22(4):375–384. https://
doi.org/10.1055/s- 2002- 36759
Fiori L, Ranavolo A, Varrecchia T, Tatarelli A, Conte C, Draicchio
F, Castiglia SF, Coppola G, Casali C, Pierelli F, Serrao M (2020)
Impairment of global lower limb muscle coactivation during walking in cerebellar ataxias. Cerebellum 19(4):583–596. https://doi.
org/10.1007/s12311- 020- 01142- 6
Fonteyn EMR, Schmitz-Hübsch T, Verstappen CC, Baliko L, Bloem
BR, Boesch S, Bunn L, Charles P, Dürr A, Filla A, Giunti P,
Globas C, Klockgether T, Melegh B, Pandolfo M, De Rosa A,
Schöls L, Timmann D, Munneke M, van de Warrenburg BPC
(2010) Falls in spinocerebellar ataxias: results of the EuroSCA
fall study. The Cerebellum 9(2):232–239. https://doi.org/10.1007/
s12311-010-0155-z
Grimaldi G, Manto M (2012) Clinical and pathophysiological features of
cerebellar dysfunction. In hyperkinetic movement disorders. WileyBlackwell, pp 257–278 https://doi.org/10.1002/9781444346183.
ch17
Ilg W, Fleszar Z, Schatton C, Hengel H, Harmuth F, Bauer P, Timmann
D, Giese M, Schöls L, Synofzik M (2016) Individual changes in preclinical spinocerebellar ataxia identied via increased motor complexity. Mov Disord 31(12):1891–1900. https://doi.org/10.1002/
mds.26835
Ilg W, Seemann J, Giese M, Traschütz A, Schöls L, Timmann D, Synofzik
M (2020) Real-life gait assessment in degenerative cerebellar ataxia:
toward ecologically valid biomarkers. Neurology 95(9):e1199–
e1210. https://doi.org/10.1212/WNL.0000000000010176
Kelly G, Shanley J (2016) Rehabilitation of ataxic gait follow-
ing cerebellar lesions: applying theory to practice. Physiother
Theory Pract 32(6):430–437. https://doi.org/10.1080/09593985
.2016.1202364
Lawrenson C, Bares M, Kamondi A, Kovács A, Lumb B, Apps R, Filip
P, Manto M (2018) The mystery of the cerebellum: clues from
experimental and clinical observations. Cerebellum Ataxias 5(1).
https://doi.org/10.1186/s40673- 018- 0087- 9
Leggio M, Molinari M (2015) Cerebellar sequencing: a trick for pre-
dicting the future. Cerebellum (London, England) 14(1):35–38.
https://doi.org/10.1007/s12311- 014- 0616- x
Louis ED, Galecki M, Rao AK (2013) Four essential tremor cases with
moderately impaired gait: how impaired can gait be in this disease?
Tremor Other Hyperkinet Mov (New York, NY) 3:tre-03-200-4597-
1. https://doi.org/10.7916/D8QV3K7G
Louis ED, Faust PL (2020) Essential tremor: the most common form
of cerebellar degeneration? Cerebellum & ataxias 7:12. https://doi.
org/10.1186/s40673- 020- 00121- 1
Luo L, Wang J, Lo RY, Figueroa KP, Pulst SM, Kuo PH, Perlman S,
Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG,
Ying SH, Zesiewicz T, Bushara K, Geschwind M, Xia G, Subramony
SH, Ashizawa T, Kuo SH (2017) The initial symptom and motor progression in spinocerebellar ataxias. Cerebellum (London, England)
16(3):615–622. https://doi.org/10.1007/s12311- 016- 0836- 3
Manto M, Ben Taib NO (2008) A novel approach for treating cerebel-
lar ataxias. Med Hypotheses 71(1):58–60. https://doi.org/10.1016/j.
mehy.2008.01.009
Manto M, Habas C (2013) Le cervelet, de l’anatomie et la physiologie
à la clinique humaine. Springer-Verlag France, Paris
Manto M, Shaikh AG, Mitoma H (2021) Identication of the prodro-
mal symptoms and pre-Ataxic stage in cerebellar disorders: the next
challenge. Int J Environ Res Public Health 18(19):10057. https://
doi.org/10.3390/ijerph181910057
Margolesky J, Singer C (2017) How tandem gait stumbled into the
neurological exam: a review. Neurol Sci 39(1):23–29. https://doi.
org/10.1007/s10072- 017- 3108- 1
Margolesky J, Bette S, Shpiner DS, Jordan EA, Dong C, Rundek T,
Singer C (2019) Tandem gait abnormality in parkinson disease: prevalence and implication as a predictor of fall risk. Parkinsonism Rela
Disord 63:83–87. https://doi.org/10.1016/j.parkreldis.2019.02.034
Mari S, Serrao M, Casali C, Conte C, Martino G, Ranavolo A, Coppola
G, Draicchio F, Padua L, Sandrini G, Pierelli F (2014) Lower
limb antagonist muscle co-activation and its relationship with gait
parameters in cerebellar ataxia. Cerebellum (London, England)
13(2):226–236. https://doi.org/10.1007/s12311- 013- 0533- 4
Marquer A, Barbieri G, Pérennou D (2014) The assessment and treat-
ment of postural disorders in cerebellar ataxia: a systematic review.
Ann Phys Rehabil Med 57(2):67–78. https://doi.org/10.1016/j.
rehab.2014.01.002
Martino G, Ivanenko YP, d’Avella A, Serrao M, Ranavolo A, Draicchio F,
Cappellini G, Casali C, Lacquaniti F (2015) Neuromuscular adjustments of gait associated with unstable conditions. J Neurophysiol
114(5):2867–2882. https://doi.org/10.1152/jn.00029.2015
Соседние файлы в папке Библиотека им академика М.И. Перельмана