Disability and Rehabilitation

ISSN: (Print) (Online) Journal homepage: https://www.tandfonline.com/loi/idre20

Assessment and tailored physical rehabilitation

approaches in persons with cerebellar
impairments targeting mobility and walking
according to the International Classification of
Functioning: a systematic review of case-reports
and case-series

Anne Bogaert, Francesco Romanò, Pierre Cabaraux, Peter Feys & Lousin
Moumdjian

To cite this article: Anne Bogaert, Francesco Romanò, Pierre Cabaraux, Peter Feys & Lousin
Moumdjian (2023): Assessment and tailored physical rehabilitation approaches in persons
with cerebellar impairments targeting mobility and walking according to the International
Classification of Functioning: a systematic review of case-reports and case-series, Disability and
Rehabilitation, DOI: 10.1080/09638288.2023.2248886

To link to this article: https://doi.org/10.1080/09638288.2023.2248886

View supplementary material Published online: 28 Aug 2023.

Submit your article to this journal View related articles

View Crossmark data

Full Terms & Conditions of access and use can be found at

https://www.tandfonline.com/action/journalInformation?journalCode=idre20
Disability and Rehabilitation
https://doi.org/10.1080/09638288.2023.2248886

Review Article

Assessment and tailored physical rehabilitation approaches in persons with

cerebellar impairments targeting mobility and walking according to the
International Classification of Functioning: a systematic review of case-reports
and case-series
Anne Bogaerta*, Francesco Romanòb*, Pierre Cabarauxc, Peter Feysa,d and Lousin Moumdjiana,d,e
a
REVAL Rehabilitation Research Center, Faculty of Rehabilitation Sciences, Hasselt University, Diepenbeek, Belgium; bNeuroimaging Research Unit,
Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy; cService de Neurologie, Médiathèque Jean Jacquy, CHU-Charleroi,
Charleroi, Belgium; dUMSC Hasselt, Pelt, Belgium; eIPEM Institute of Psychoacoustics and Electronic Music, Faculty of Arts and Philosophy, Ghent
University, Ghent, Belgium

ABSTRACT ARTICLE HISTORY

Purpose: Cerebellar impairment (CI) manifests from different etiologies resulting in a heterogenic Received 31 March 2023
clinical presentation affecting walking and mobility. Case-reports were reviewed to provide an analytical Revised 9 August 2023
clinical picture of persons with CI (PwCI) to differentiate cerebellar and non-cerebellar impairments Accepted 10 August 2023
and to identify interventions and assessments used to quantify impact on walking and mobility KEYWORDS
according to the International Classification of Functioning, Disability and Health (ICF). Cerebellar impairment;
Materials and Methods: Literature was searched in PubMed, Web Of Science and Scopus. Case-reports assessment; physical
conducting physical rehabilitation and reporting at least one outcome measure of ataxia, gait pattern, rehabilitation; walking;
walking or mobility were included. mobility; International
Results: 28 articles with a total of 38 different patients were included. Etiologies were clustered to: Classification of Functioning;
case reports
spinocerebellar degenerations, traumatic brain injuries, cerebellar tumors, stroke and miscellaneous.
The interventions applied were activity-based, including gait and balance training. Participation based
activities such as tai chi, climbing and dance-based therapy had positive outcomes on mobility.
Outcomes on body function such as ataxia and gait pattern were only reported in 22% of the patients.
Conclusions: A comprehensive test battery to encompass the key features of a PwCI on different
levels of the ICF is needed to manage heterogeneity. Measures on body function level should be
included in interventions.

hhIMPLICATIONS FOR REHABILITATION

• This review reports on 38 cerebellar cases from 14 different aetiologies.
• Distinguishing cerebellar and non-cerebellar symptoms and categorising patients within the three
cerebellar syndromes can assist with heterogeneity.
• Reporting of assessment on the body function level of ICF in terms of ataxia and gait pattern
was only present in a minority of reports and thus increased reporting is encouraged.
• Multimodal and patient-tailored strategies are promising for targeting walking and mobility in
persons with cerebellar impairment.
Abbreviations: Persons with Cerebellar Impairments: PwCI; The International Classification of
Functioning, Disability and Health: ICF; The cerebellar motor syndrome: CMS; The vestibulo-cerebellar
syndrome: VCS; Cerebellar cognitive affective syndrome/Schmahmann’s syndrome: CCAS/SS;
Spinocerebellar ataxia: SCA; Gait training: GT; Scale for assessment and rating of ataxia: SARA;
International Cooperative Ataxia Rating Scale: ICARS; Functional Independence Measure: FIM; Timed Up
and Go: TUG; Berg Balance Scale: BBS.

Introduction states based on contextual (sensory) information and previous

The cerebellum plays an integral role in the control of limb and
ocular movements, balance, and walking [1]. Noteworthy, it is not
merely crucial for perception or action in the narrow sense, but
operates as a forward controller, capable of predicting system
memory [2]. Dysmetria [3] and increased motor variability [4–6]
are observed when affected. A recent study distinguished three
cerebellar syndromes by linking the clinical presentation to neu-
roimaging data [7]: cerebellar motor syndrome encompassing

CONTACT Lousin Moumdjian lousin.moumdjian@uhasselt.be REVAL Rehabilitation Research Center, Faculty of Rehabilitation Sciences, Hasselt University,
Diepenbeek Belgium; UMSC Hasselt, Pelt, Belgium; IPEM Institute of Psychoacoustics and Electronic Music, Faculty of Arts and Philosophy, Ghent University, Ghent,
Belgium
*Shared first authorship.
Supplemental data for this article can be accessed online at https://doi.org/10.1080/09638288.2023.2248886.
© 2023 Informa UK Limited, trading as Taylor & Francis Group
2 A. BOGAERT ET AL.
general motor dysfunction; vestibular cerebellar syndrome encom-
passing oculomotor deficits, vertigo and dizziness and; cerebellar
cognitive affective syndrome or Schmahmann’s syndrome charac-
terized by dysfunction in cognition and affect [8]. The umbrella
term used to characterise these impairments is termed cerebellar
ataxia [9].
The population of persons with Cerebellar Impairments (PwCI)
is characterized by a heterogeneity in clinical presentation which
can be ascribed to a wide range of etiologies, being either genetic
or acquired [10,11]. Thus the clinical picture seen in a PwCI can
be explained by both cerebellar and non-cerebellar pathophysi-
ology. Therefore an emphasis for a full clinical picture of the
patients functioning, is required, with the ambition of separating
cerebellar from non-cerebellar impairments in this heterogenic
population, with the end goal of optimizing effective physical
rehabilitation delivery. A framework to guide clinicians is the
International Classification of Functioning, Disability and Health
(ICF). The ICF was officially endorsed in 2001 by the World Health
Organization as international standard to describe and measure
health and disability, and is a cornerstone in rehabilitation practice
[12]. It promotes a patient-centered approach, since it encom-
passes not only body function, but also activity & participation
functioning and the influence of personal and environmental
factors for particular health conditions. The ICF framework invites
clinicians to disentangle underlying impairments causing the dys-
function. Assessment tools and interventions used should be
matched to these dysfunctions.
Specific to cerebellar dysfunction, impairments in balance and
walking reflect the cerebellum’s proposed role in coordination,
sensory integration, coordinate transformation, motor learning,
and visuomotor adaptation [1] and lead to lower quality of life
in patients [13]. The complexity of the cerebellum as a neurolog-
ical entity demands for novel rehabilitation strategies that can
challenge these functions. There is moderate evidence that phys-
ical therapy may improve postural disorders [14], ataxia and daily
life functions [15] and balance and coordination [16], foremost in
persons with degenerative cerebellar diseases. Most of the research
on PwCI has been conducted on impairment-based rehabilitation
strategies, for example on balance and coordination exercises and
conventional physical therapy. Less often, activity-level rehabilita-
tion, such as gait training and virtual reality-based training has
been investigated [14, 16]. As walking is a highly determinant
factor for mobility and independence, this will be the main focus
of this review.
Another systematic review on exercise and physical therapy
for children with ataxia showed promising results but could make
no firm conclusions due to overall low methodological quality of
studies [17]. Similarly, the review of Winser, Chan, Chen, Hau,
Leung, Leung and Bello [18] on exercise-based approaches in
individuals with cerebellar ataxia outlined positive effects on ataxia
and balance, though reporting high heterogeneity between sam-
ples and low to moderate quality of studies included [18]. Clearly,
there is a lack of high quality evidence for rehabilitation in PwCI
and concrete recommendations for physical therapy practice
are needed.
In this context, we present this work, that is, a systematic
review on case reports and case series of PwCI. In case reports
and case series, clinical information of each patient is reported,
and thus this review aims to separate from the study patients
the cerebellar from non-cerebellar impairments. Thereafter, we
aim to document the information per patient within the ICF
framework and the recently proposed cerebellar syndromes [19]
to provide an overview of the heterogeneity in PwCI with the
intention of bridging the gap between research and practice.
Finally, we aim to systematically identify the physical rehabili-
tation approaches used and the impact of these approaches on
ataxia, gait pattern, walking and mobility. An overview of the
assessments methods used to quantify the impact of these
interventions in all dimensions of the ICF framework will be
reported.
Methods
Database selection and search strategy
The search strategy was developed based on defining the popu-
lation, the intervention type and the outcome measure, and was
carried out in the following three databases: PubMed, Web of
Science -all databases and Scopus on 27th September 2022. The
following search strategy was applied in PubMed: (cerebellar dis-
eases[MeSH Terms]) OR (cerebellar ataxia[MeSH Terms]) OR ("cerebellar
stroke"[Title/Abstract]) OR ("cerebellar infarction"[Title/Abstract]) OR
("degenerative ataxia"[Title/Abstract]) OR ("cerebellar degeneration"[Ti-
tle/Abstract]) OR ("cerebellar degenerative diseases"[Title/Abstract])
OR ("cerebellar dysfunction"[Title/Abstract]) OR ("cerebellar lesion"[Ti-
tle/Abstract]) OR ("cerebellar disease"[Title/Abstract]) OR ("cerebellar
diseases"[Title/Abstract]) OR ("cerebellar ataxia"[Title/Abstract]) OR
("cerebellar ataxias"[Title/Abstract]) AND (rehabilitation[MeSH Terms])
OR (physical therapy modalities[MeSH Terms]) OR ("exercise therapy-
"[MeSH Terms]) OR (rehabilitation[Title/Abstract]) OR ("exercise ther-
apy"[Title/Abstract]) OR ("neurologic rehabilitation"[Title/Abstract]) OR
("task- specific training"[Title/Abstract]) OR ("task-oriented training"[Ti-
tle/Abstract]) OR ("functional task practice"[Title/Abstract]) OR
("Exercise"[Mesh]) AND (gait[MeSH Terms]) OR (walking[MeSH Terms])
OR (gait ataxia[MeSH Terms]) OR (gait analysis[MeSH Terms]) OR
("walking capacity"[Title/Abstract]) OR (gait[Title/Abstract]) OR (walk-
ing[Title/Abstract]) OR ("gait ataxia"[Title/Abstract]) OR ("gait analy-
sis"[Title/Abstract]) OR (mobility[Title/Abstract]). The search strategies
applied in Web of Science and Scopus is found in Supplementary
Table 1. A protocol for this systematic review is not previously
published.
Selection criteria
Published studies were included when following criteria were met:
1) Design: case reports with one (case-study) or multiple cases
(case-series). Case series were included if information was reported
per patient. 2) Population: participants diagnosed with any type
of cerebellar disease or dysfunction 3) Outcome measure: at least
one measure specific for measuring walking on the following
levels of the ICF [12]: Body function level: a) Gait pattern function
(code b770): defined as “Functions of movement patterns associ-
ated with walking, running or other whole body movements.” b).
Ataxia: reflecting a dysfunction in “Control of voluntary movement
functions” (code b760) as this is defined as “Functions associated
with control over and coordination of voluntary movement”.
Activity and participation level: a) Walking (activity level, code
d450): defined as “Moving along a surface on foot, step by step,
so that one foot is always on the ground, such as when strolling,
sauntering, walking forwards, backwards, or sideways.” b) Mobility
(activity level, code d4): the overall term for changing or main-
taining body position or changing from location “by carrying,
moving or manipulating objects, by walking, running or climbing,
and by using various forms of transportation”. 4) Intervention: any
type of physical rehabilitation with active involvement of the
patient. Rehabilitation is defined by the World Health Organization
as “a set of interventions designed to optimize functioning and reduce
 ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
disability in individuals with health conditions in interaction with
their environment” (WHO, int, 2021).
Studies were excluded when the full text was in a language
other than English, Dutch, French or Italian. Cases reports on
participants under 18 years old were excluded as evidence on
rehabilitation in children with ataxia can be found in another
systematic review [17]. All articles were screened by two inde-
pendent reviewers (A.B. and F.R). When comparing the remaining
articles a consensus was reached by deliberation or a third party
was consulted (L.M.).
Quality and risk of bias assessment
The quality and risk of bias assessment of the included articles
was assessed with the CARE checklist [20]. The CARE checklist is
a thirty-criteria checklist recommended to use for case reports by
the EQUATOR Network. The checklist was applied per individual
study, to inform on the overall information presented in the case
reports and studies, and were not used in data synthesis.
Data extraction
The following data was extracted for each article: the etiology
for cerebellar impairment and lesion location, medical findings/
major medical interventions, age and gender, time from diagnosis,
complete clinical presentation and ambulance in daily life.
Additionally, based on the clinical presentation and the MRI
results reported in the case reports, the patients were grouped
in one of the following cerebellar syndromes [7]: 1) The cerebellar
motor syndrome (CMS), characterized by dysfunctions in general
motor behavior resulting in gait and upper limb ataxia, dysmetria,
balance problems and dysarthria. 2) The vestibulo-cerebellar syn-
drome (VCS), characterized by vertigo, dizziness, and oculomotor
deficits like impaired ocular pursuit and vestibulo-ocular reflexes,
dysmetria of saccades, abnormal fixation and errors in vergence.
3) Cerebellar cognitive affective syndrome/Schmahmann’s syn-
drome (CCAS/SS) characterized by dysfunction in executive func-
tioning (planning, abstract reasoning, verbal fluency, working
memory), attentional control, spatial cognition, language produc-
tion and personality changes with dysregulation of affect e.g.,
overfamiliarity, impulsivity and aberrant behavior. The dosage
and the type of intervention was also extracted and therapy
hours were calculated. The outcome measures and effects of the
intervention on the outcomes were extracted in four categories
based on the ICF framework: ataxia, gait pattern (body function
level), walking and mobility (activity/participation level) [12]. Data
integrity of all patient characteristics and outcome measures were
inspected by co-authors A.B. and F.R. Authors of papers were
contacted where data was missing or incomplete.
Results
Initially, a total of 767 publications were identified. After remov-
ing 287 duplicates, 480 records were screened based on title
and abstract. From this list, 83 records were found to be eligible
for further assessment based on the above mentioned selection
criteria. 82 full-text articles were assessed for eligibility (one
full-text could not be retrieved after request from the corre-
sponding author), from which 27 articles in English were included
in this systematic review. In addition, one article was added
from the reference lists of the included articles. The Prisma flow-
chart is presented in Figure 1. The list of references of the
3
excluded studies based on full text is found in supplementary
Table 2.
Data integrity
Data integrity of all patient characteristics and outcome measures
were verified. Discrepancy was found only in one study only [21],
and authors of this study [21] were contacted due to a discrep-
ancy between the description of the results in their manuscript
(implying an improvement) and the pre/post intervention Berg
Balance Scale scores reported in their table (showing a worsening).
The authors complied to the request, and checked their records
and confirmed that the scores had been inverted in the table.
Thus, we have verified this information, and reported it correctly
in the current manuscript.
Quality assessment
Overall, criteria on the abstract, introduction, patient information,
clinical findings and the description of the intervention were
consistently reported. A lack of reporting across studies was
observed in criteria on diagnostic assessment, timeline of import-
ant historical and current information, prognosis, therapy adher-
ence and how this was measured and the patient perspective on
the intervention. The use of an informed consent was reported
in half of the case reports. The results of the quality assessment
can be found in the Supplementary Table 3. Moreover,
Supplementary Table 4 provides statements extracted from the
studies on funding sources and patient informed consent.
Patients in the case reports
A total of 38 patients were included across the 28 reports.
The following six clusters were defined to distinguish the eti-
ologies of cerebellar impairment: Spinocerebellar degenerations,
Traumatic brain injury, Cerebellar tumor/Paraneoplastic cerebellar
degeneration, Cerebellar Stroke, Cerebellar ataxia not specified
and Miscellaneous. A distribution of the patients according to the
etiologies is presented in Figure 2. All features of the clinical
presentation are listed in Table 1.
The cluster “Spinocerebellar degenerations” consisted of two
patients with non-specified spinocerebellar ataxia (SCA) [22,23],
one Spinocerebellar ataxia (SCA) type 7 [24], one SCA type 2 [25],
one SCA type 3 (Machado Joseph’s Disease) [21], two Friedreich’s
ataxia’s [26,27], and one sporadic Olivopontocerebellar atro-
phy [28].
The “Traumatic brain injury” cluster [29–33] consisted of eight
patients. The “Cerebellar tumor/paraneoplastic cerebellar degen-
eration” cluster consisted of three patients with a epidermoid
cysts [34], one with a cerebellar tumor [35] and two with para-
neoplastic cerebellar degeneration which of one was caused by
left breast cancer [36] and the other by ovarian cancer [37].
The cluster “Cerebellar stroke” consisted of one patient with a
cerebellar hemorrhage and obstructive hydrocephalus [38], one
ischemic lacunar stroke [33], one right cerebellar and brainstem
infarction [39] and one with an ischemic stroke in the left and
mainly posterior cerebellum [40].
The cluster “Cerebellar ataxia, not specified” consisted of two
patients with non-specified cerebellar ataxia [41] and one with
progressive cerebellar ataxia [42].
The cluster “Miscellaneous” consisted of the remaining patients
w i t h v a r i o u s o t h e r e t i o l o g i e s : p e r i n a t a l a n ox i c
4 A. BOGAERT ET AL.
Figure 1. PRISMA flowchart Outlining study screening and selection.
encephalopathy [33], metabolic encephalopathy and epilepsy after
stereotactic biopsy of a frontal lobe tumor [33], Cerebrotendinous
xanthomatosis with cerebellar involvement [35], Systematic lupus
erythematosus [43], Pontine cavernoma [44], Charcot-Marie-Tooth
disease with cerebellar involvement [45], cerebellar atrophy asso-
ciated with Primary Sjögren’s Syndrome [46], cerebellar
dysfunction resulting from high levels of drugs after treatment
for acute leukemia [29] and progressive multiple sclerosis with
cerebellar involvement [47,48].
The age of the patients ranged from 18 to 72 years old (mean
37.91) and the time from diagnosis ranged from acute up to
20 years. The level of assistance needed by the patients ranged
 ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
Figure 2. Number of cases according to the etiology for cerebellar impairment presented in 6 clusters.
from ambulant without assistance to totally not able to walk.
The Functional Ambulation Category, which is a mobility score
ranging from 0 (nonfunctional ambulator) to 5 (ambulator, inde-
pendent) [49] was sometimes used to describe the level of
assistance.
Based on the clinical presentation and the MRI results reported
in the studies, each patient was grouped to one or more of the
cerebellar syndromes described by Cabaraux et al. (2021) [19]. As
seen in Table 1, of the included patients, 63% were estimated to
have only cerebellar motor syndrome, 18.5% were estimated to
have a cerebellar motor syndrome in combination with vestibular
cerebellar syndrome, 10.5% were estimated to have a cerebellar
motor syndrome in combination with cognitive affective syn-
drome/Schmahmanns’s syndrome and 8% were estimated to have
all three.
Other non-cerebellar impairments
Apart from features of cerebellar ataxia, deficits not primarily due
to the cerebellar lesion or dysfunction were reported and pre-
sented in Table 1. Almost half of the patients had sensory and/
or strength deficit in combination with their cerebellar impairment.
In Figure 3, the presence of strength and/or sensory deficits is
presented per etiology cluster.
In the cluster “Spinocerebellar degenerations” (N = 8) no sen-
sory or strength deficits (non-cerebellar) were reported in the
SCA’s, but a sensory deficit was present in the patient with
Olivopontocerebellar atrophy (typical hearing loss) and a
moderate-to-severe spastic tetra paresis was present in one of
the two cases with Friedreich ataxia [26]. In the cluster
“Traumatic Brain Injury” (N = 8), five patients reported no
strength or sensory deficits [29, 32,33], one patient had a spas-
tic quadriparesis with retropulsion [29], one had a lower limb
weakness and diplopia [30] and one had lower limb weak-
ness [32].
In the cluster “Cerebellar tumor/paraneoplastic cerebellar
degeneration” (N = 8), three patients with cerebellopontine angle
epidermoid cysts had more than one sensory deficits mainly due
to cranial nerve palsy’s [34]. In the cluster “Cerebellar stroke”
(N = 4), two had a strength deficit [38, 40] and one had a sensory
5
deficit [33]. In the cluster “Cerebellar ataxia, non-specified” (N = 3)
only one patient had a strength deficit [41].
In the cluster "Miscellaneous” (N = 8), one patient with enceph-
alopathy reported visual impairments [33] and one patient
reported several sensory deficits (visual problems, right hearing
loss, mild decreased vibration sense) due to Cerebrotendinous
xanthomatosis [35], which is a neurometabolic disorder of lipid
storage and bile acid synthesis [50]. The patient with Systematic
Lupus Erythematosus reported a strength deficits in all four limbs
(MMT 4/5) [43], the one with a pontine cavernoma had a right
hemiparesis with loss of sensation and proprioception [44] and
the case with a Charcot-marie-tooth disease had plegia of ankle
and foot muscles, hyposensitive feet, impaired vibration sense
overall and blindness [45].
Other non-cerebellar impairments were foot pain when walk-
ing, ankle sprains [27], chronic fatigue and depression [28, 42],
retropulsion in sitting and stance [29], fractures of the right upper
extremity, ribs, clavicle and facial bones [30], clonus of the right
ankle and retraction and external rotation right hip [32], dyspha-
gia, diplopia due to VI cranial nerve palsy and hoarseness [34],
nausea and diaphoresis in a case with paraneoplastic degeneration
[37], xerophthalmia and xerostomia which result from lymphocytic
infiltration of lacrimal and salivary glands in a case with Primary
Sjögren’s Syndrome [46, 51], bradykinesia, attention deficits and
psychomotor retardation [33] and a reduced range of motion in
both lower extremities.
Physical rehabilitation interventions
In Figure 4, all interventions conducted are displayed from most-
to less frequently used.
The most frequently used therapy was gait training (27/38
patients) under diverse formats: gait training (GT) with a cane,
walking sticks, a walker and an intervention with assistance dog
[41]; a GT method that discouraged upper extremity weight
bearing [29]; GT combined with cranial nerve non-invasive neu-
romodulation [22]; GT using body-weight support on a treadmill
and overground (assistance of a person and a walker), GT with
a rolling walker, stepping to targets and stair training [30]; GT
with functional electrical stimulation of left dorsiflexors in
 6

Table 1. Etiology for cerebellar impairment and clinical presentation.

Cerebellar
Age Time from diagnosis syndrome:
Ethiology for cerebellar Neuroimaging and medical (years) in years and Ambulation status and cerebellar CMS VCS Non-cerebellar
Study impairment interventions and sex months impairments CCAS/SS impairments
Spinocerebellar degeneration
Bastani, 2018 Slow progressive MRI: cerebellar atrophy 55 F 20 years Spasticity in lower limbs. Poor balance, CMS NR
A. BOGAERT ET AL.

spinocerebellar ataxia frequent falls. Able to do a gait

Goulipian, 2008 Friedreich ataxia
Im, 2021 Spinocerebellar ataxia (SCA)
Kim, 2021 Spinocerebellar ataxia type 7
Landers, 2009 Sporadic olivopontocerebellar
atrophy
Mendonça, 2021 SCA type III (Machado Joseph’s MRI: cerebellar atrophy
Disease)
Portaro, 2019 Friedreich ataxia
Song, 2019 Spinocerebellar ataxia type 2
Balliet, 1987 (P1-P4) P1: TBI with cerebellar
involvement, in combination
with: P1: right parietal skull
fracture with contracoup
cerebral contusion
P2: right parietal A/V
malformation
right intercerebral hematoma
P3: gunshot wound right
occipitoparietal region
P4: unspecified, diffuse
encephalopathy
Genetic study
Confirmed by CT/MRI scans
NR
MRI: cerebellar atrophy
MRI: cerebellar atrophy and
bilateral hypointense signals
in the globus pallidus, the
putamen and the substantia
nigra.
NR
Traumatic brain injury
NR
assessment.
26 F Onset: 12 years Independent with cane, 1 person’s CMS Foot pain when walking,
Diagnosis: 1 year assistance for stairs, frequent falls, ankle sprains
fatigability (estimated walking distance
400 m). Dysarthria
51 F 19 years Able to walk 10 m without assistance. CMS NR
Wide-based and unsteady gait and
balance, uncoordinated limb movement,
mild postural tremor, slurred speech.
23 F NR Able to walk a short distance with a CMS NR
walker. Postural instability and gait
disturbance.
19 F 3 years Ambulant with support on furniture or CMS, VCS, 30% hearing loss
assistance of 1 person, 1 fall a day. CCAS/SS bilaterally, chronic
Severely ataxic gait, upper extremity fatigue and
ataxia, moderate dysmetria, depression.
dysdiadochokinesia., dysarthria, vertigo
and left beating nystagmus. Mild
short-term memory loss.
34 M 10 years Ambulant without aid. Wide-based gait and CMS, VCS NR
instability. Limb and trunk ataxia,
dysmetria, dysdiadochokinesia and
dysarthria. Rotatory nystagmus.
29 M 19 years Wheelchair dependent, can sit without CMS ,VCS Mild delay in swallowing.
support for >10s. Able to walk for 10 m Moderate-to severe
with walker and assistance of 1 person. spastic tetra paresis
Use of walker or powered wheelchair
for ADL, transfer into a car, and stairs.
Dysmetria, dysdiadochokinesia,
dysarthria, horizontal nystagmus.
39 M 11 years Ambulant with max assistance of 2 CMS NR
persons, Wheelchair-bound in ADL.
Severe balance problems, gait ataxia,
dysmetria and dysdiadochokinesia, mild
dysarthria and writing problems.
P1: 22 M P1: 2 years, P1: ambulant with cane assistance of 1 P1: CMS P3: spastic quadriparesis,
P2: 30 M 6 months person P2: CMS strong backwards
P3: 30 M P2: 12 years, P2: ambulant with wheeled or reciprocal P3: CMS pulsion in sitting and
P4: 42 M 2 months walker, max assistance of 1 person P4: CMS stance
P3: 10 years, P3: ambulant with wheeled walker, max
6 months assistance of 2 persons. Severely
P4: 1 year, 9 months depressed equilibrium reactions, only
gross movements,
P4: ambulant with wheeled or reciprocal
walker, max assistance 1 person
All participants had normal cognition.

(Continued)
Table 1. Continued.
Cerebellar
Age Time from diagnosis syndrome:
Ethiology for cerebellar Neuroimaging and medical (years) in years and Ambulation status and cerebellar CMS VCS Non-cerebellar
Study impairment interventions and sex months impairments CCAS/SS impairments
Freund, 2010 TBI with cerebellar involvement, NR 24 M 13 months Ambulant using a two-wheeled walker with CMS Muscle weakness of
as a result of a motor moderate assistance (max 6-24.4 m), plantar flexors (MMT
vehicle accident using wheelchair in ADL. Truncal and 3/5) and hip
extremity ataxia. abductors (MMT 3/5)
Dysmetria (left > right), diplopia with quick and extensors (MMT
head with movements and slow speech. 4/5). Fractures: right
upper extremity, ribs,
clavicle, facial bones.
Freund, 2013 ** Same person as above NR 28 M 2 years, 5 months Ambulant using a two-wheeled walker with CMS Bilateral weakness
intermittent light touch or supervision plantar flexors (MMT
(1 year after intervention previous 3/5)
study), mild dysmetria on the left side.
Sartor-Glittenberg, 2014 TBI following accident, more P3*: NDI: 30 months after the P1: 22 F P1: 3 years, P1: Ambulant with a front-wheeled walker P1: CMS, P2: Weakness of the
specific: injury revealed prior shear P3: 20 M 4 months or max assistance of 1 person, using CCAS/SS lower extremity,
P1: traumatic subarachnoid injury of the corpus callosum, P2: 6 months also wheelchair in ADL. Severe ataxia of P2: CMS, clonus right ankle
hemorrhage, external injury left superior cerebellar the lower extremity and dysmetria. CCAS/SS and retraction and
of cerebellum and frontal peduncle, left cerebral Impairments in executive and cognitive external rotation right
lobes, diffuse axonal injury, peduncle and the left function. hip.
multifocal shear injuries, cerebellar hemisphere. P2: Ambulant with a front-wheeled walker
mild subdural hematoma, with min assistance. Ataxia, behavioral
cerebellar hemorrhages and issues.
punctate hemorrhages in
the mesencephalon
P2: temporoparietal epidural
hematoma and a subdural
hemorrhage Cerebellar
ataxia due to:
Stephan, 2011 (P1) P1: cranio-cerebral injury. CT-scan: P1: 29 M P1: 2 years P1: Mobility FAC 4/5, upper limb ataxia P1: CMS NR
P1: Diffuse axonal injury with and tremor
bilateral frontal, occipital, and
hippocampal hemorrhagic
lesions and hydrocephalus
Cerebellar tumor/paraneoplastic cerebellar degeneration
Fu, 2014 3 patients with P1-3: MRI/CT scan: CPA P1: 18 F Post-operative P1: Ataxia, vertigo, mild cognitive deficits P1: CMS, P1: Mild dysphagia,
cerebellopontine angle (CPA) epidermoid cyst + surgical P2: 72 M P2: able to walk 3 meters with stick and VCS, diplopia due to left VI
epidermoid cysts. resection. P3: 18 F perform a sit-to-stance with minimal CCAS/SS cranial nerve (CN)
P1: 1.4 cm × 2.7 cm × 2.1 cm, assistance. Unsteady gait P3: Gait P2: CMS palsy, left facial droop
left, with mass effect on instability, dysphagia, mild left upper P3: CMS and numbness
the pons and displacement of extremity dysmetria P2: Dysphagia,
cranial nerves VII and VIII. hoarseness, deafness
P2: 7 cm, right, with significant in 1 ear
brainstem displacement and P3: Diplopia, right cranial
hydrocephalus nerve (CN) IV paresis,
P3: right, with severe brainstem right CN VI palsy, and
compression. right CN V2 and V3
numbness.
Gill-Body, 1997 (P1) P1: Recurrent pilocystic P1: Surgical resection cerebellar P1: 36 F P1: 6 years P1: 7 months post-operative: ambulant, P1: CMS, Left-sided weakness,
cerebellar astrocytoma tumor 6 years ago and outdoors only under supervision. VCS blurred vision and
located in the middle and 7 months ago + 1 month Dizziness, postural unsteadiness, hearing loss after
ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS

superior cerebellar vermis radiation therapy. impaired hand coordination and radiation therapy.
and extending to the floor moderate dysarthria.
of the fourth ventricle.
7

(Continued)

Table 1. Continued.
Ethiology for cerebellar
Study impairment
Kato, 2017 Anti-Yo antibody-positive
paraneoplastic cerebellar
degeneration caused by left Neuroimaging 6 months before
breast cancer
Perlmutter 2003 Paraneoplastic cerebellar
degeneration (PCD)
High, 2020 Cerebellar hemorrhage with
obstructive hydrocephalus.
Stephan, 2011 (P2) Cerebellar ataxia due to:
P2: Ischemic lacunar stroke,
sequelae of left vertebral
aneurysm with
ventriculocardiac derivation
Takimoto, 2021 Right cerebellar and brainstem MRI: infarct in the right
infarction
Wilson, 2017 Ischemic stroke left cerebellum, Complication: hydrocephalus
mainly in the PICA
distribution
Neuroimaging and medical
interventions
Surgery: mastectomy 1 month
after admission
admission: cerebellar
hemisphere atrophy and
inflammation of anti-Yo
antibodies
MRI: cerebellar atrophy
Serology: PCD confirmed by
positive serum for
anti-Purkinje antibodies at
1:30,000
History: hypertension and
metastatic ovarian cancer
status after total abdominal
hysterectomy, colon resection
with subsequent reversal of
colostomy, chemotherapy and
radiation therapy
Cerebellar stroke
Acute: Decompressive
craniotomy, a hematoma
evacuation and an external
ventricular drain. Intubation
for acute respiratory failure,
nasogastric tube for
dysphagia. Hypertension and
prediabetes.
CT-scan:
P2: Sequelae of clipping, no
acute lesion
cerebellum and brainstem
with brainstem compression
ICU: Tracheostomy and
percutaneous endoscopic
gastrostomy.
8
Cerebellar
Age Time from diagnosis syndrome:
(years) in years and Ambulation status and cerebellar CMS VCS Non-cerebellar
and sex months impairments CCAS/SS impairments
42 F 2.5 months Ambulant between parallel bars with CMS, VCS NR
support of one hand. Trunk and limb
ataxia, walking difficulties, dizziness and
A. BOGAERT ET AL.
dysarthria.
75 F NR Severe ataxia, wheelchair dependent for CMS, VCS Nausea and diaphoresis.
mobility (only tolerates 5 min in seated
position). Upper limb dysmetria and
dysdiadochokinesia (not present in
lower limbs). Dysarthria, severe
nystagmus with lateral gaze.
Ambulant with cane before this event.
32 M 1 month Non-ambulant, able to sit at the edge of CMS, CCAS/ Impaired strength and
the bed with bilateral upper extremity SS activity endurance
support and moderate physical
assistance. Impaired coordination and
balance, cognitive deficits, confusion.
Alcohol abuse.
P2: 56 M P2: 2 months P2: Mobility FAC 1/5, wheelchair in ADL, P2: right sensitive-motor
(stroke) 25 years severe balance disturbance and tremor hemi syndrome
(aneurysm)
̴40 M 37 days Able to walk unassisted, requires CMS NR
supervision in walking and transfers,
independent in ADL. Cerebellar ataxia
with rigid gait pattern, risk of falls in
dynamic balance conditions.
51 F Acute ICU: Non ambulatory, minimally responsive. CMS ICU: flaccid in all limbs
flaccid in all limbs. IRU: Reduced strength in
IRU: 6 weeks post: Max assistance of 2-3 all limbs, upper limb
persons needed for transfers. Truncal proximal (MMT 1/5)
ataxia with poor sitting balance (needs distal (2/5, 3/5), lower
2 persons). Stance with max assistance limb hip flex 2-/5,
for 5-10 min. Bilateral dysmetria and rest 3/5. Weakness in
dysdiadochokinesia. neck extensors
OR: 17 weeks post: Moderate assistance requiring collar OR:
needed in ADL, requires special Upper and lower
wheelchair for positioning and stability. extremity weakness
Whole body ataxia. (left side weaker than
the right). Left eye
patch.
(Continued)
Table 1. Continued.
Ethiology for cerebellar
Study impairment
Abbud, 2014 Cerebellar Ataxia, not specified
Pilloni 2019 Progressive cerebellar ataxia
Balliet, 1987 (P5) P5: Acute leukemia with
complete remission.
Cerebellar dysfunction
resulting from high drug
levels during treatment.
Cha 2020 Systemic lupus erythematosus MRI: cerebellar atrophy
(SLE) with cerebellar atrophy PET-CT: markedly decreased
Gill-Body, 1997 (P2) P2: Cerebrotendinous
xanthomatosis (CTX) with
cerebellar involvement
Lo Voi, 2021 Cerebellar Atrophy Associated
with Primary Sjögren’s
Syndrome
Neuroimaging and medical
interventions
Cerebellar ataxia, not specified
NR
NR
Miscellaneous
NR
signal intensity at bilateral
temporal and parietal lobes
and cerebellum and
moderately decreased signal
intensity at the bilateral
frontal lobes
P2: CT scan: cerebellar atrophy,
calcification in the dentate
nuclei of the cerebellum, and
widening of the prepontine
region consistent with
brain-stem atrophy.
Cholesterol lowering
medication (past 4 years)
Cerebrospinal fluid analysis:
oligoclonal bands (<1 cell
mm3, 28 mg/dL of total
proteins)
MRI: reduced cerebellar volume
Treatment: metylprednisolone
(0.5/mg/kg/day),
intravenous immunoglobulins
(IvIG) (1 g/kg/
month on 5 days), and celecoxib
(200 mg/as
needed)
Cerebellar
Age Time from diagnosis syndrome:
(years) in years and Ambulation status and cerebellar CMS VCS Non-cerebellar
and sex months impairments CCAS/SS impairments
P1: 22 F NR P1: ambulant with assistance. Ataxia of all P1: CMS Deficits in strength and
P2: 47 M four limbs, balance impairment (unable P2: CMS endurance of all four
to stand with a normal base of support limbs
without assistance), frequent falls (>3/
day)
P2: ambulant without assistance. Impaired
balance and gait with frequent falls.
71 F 9 years Ambulant with cane. Unsteady gait with CMS Fatigue
increased risk of falls, balance
impairment, reduced manual dexterity,
episodes of speech slurring.
P5: 63 M P5: 1 year, 3 months P5: ambulant with cane, independent P5: CMS NR
Normal cognition
34 F 9 years Poor standing balance and dizziness CMS, VCS Strength deficit (MRC
(Romberg test and tandem gait could 4/5) on all 4 limbs.
not be performed). Bilateral nystagmus,
intention tremor, severe dysmetria and
dysdiadochokinesia.
P2: 48 M P2: 10 years P2: ambulant without an assistive device, P2: CMS, Visual problems, right
wide-based, unsteady and stiff gait. CCAS/SS hearing loss, and
Outdoors independent, except in winter mildly decreased
periods. Progressive gait instability, vibration sense.
forward bent posture, mild dysarthria
and mild cognitive deficit.
43 F 20 years Ambulant without aid, gait problems: Xerophthalmia and
dyssynergy, decomposition of multi-joint xerostomia
movements, incoordination of trunk and
arm swing, unsteadiness during sudden
direction change. Moderate dysmetria,
gaze-evoked horizontal nystagmus on
both sides (more markedly on the left)
and hypermetric saccades.
(Continued)
ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
9
 10
Table 1. Continued.
Cerebellar
Age Time from diagnosis syndrome:
Ethiology for cerebellar Neuroimaging and medical (years) in years and Ambulation status and cerebellar CMS VCS Non-cerebellar
Study impairment interventions and sex months impairments CCAS/SS impairments
Moumdjian, 2021, 2022 Progressive Multiple Sclerosis MRI in 2019: multiple white 55 M 8 years Can walk with walker and 1 person CMS NR
with severe cerebellar ataxia matter lesions in the assistance for short bursts of time but
cerebellum bilaterally. Lesions uses mostly wheelchair (EDSS 6.5).
in bilateral medulla oblongata Severe ataxia without weakness or
A. BOGAERT ET AL.

(level of the mesencephalon spasticity.

and the brain stem)
multiple periventricular black
holes and atrophy of the
corpus collosum
advanced to moderate cortical
and subcortical atrophy with
suggestion of microvascular
impairment.
Stanley, 2021 Pontine cavernoma MRI: Well lobulated lesion 19 M 10 months Ataxia, dysmetria, dysdiadochokinesia, CMS Right hemiparesis with
measuring 1.7 × 2.4 × 2.0 cm at post-operative dysarthria, bilateral intention tremor loss of sensation and
the level of mid-pons proprioception, left VI
extending superiorly to the CN palsy
left superior cerebellar
peduncle and cerebral
aqueduct and inferiorly to the
left side of 4th ventricle.
Surgery: craniotomy and excision
of the tumor
Stephan, 2011 (P3 and P4) Cerebellar ataxia due to: CT-scans: P3: 22 M P3: 22 years P3: Mobility FAC 4/5, severe balance P3: CMS, P3 : bradykinesia,
P3: Perinatal anoxic P3: Intraventricular haemorrhage P4: 42 M P4: 8 months disturbance, dysmetria, oculomotor signs VCS, attention deficits ,
encephalopathy P4: Left frontal expansive lesion P4: Mobility FAC 5/5, hypokinetic CCAS/SS psychomotor
P4: Metabolic encephalopathy of corpus callosum with movement bilateral tremor P4: CMS retardation and visual
with epilepsy after extension to the left temporal impairments
stereotactic biopsy of a lobe
frontal lobe tumor (3.5 years
before study onset) and
subsequent chemotherapy
Vinci, 2003 Charcot-Marie-Tooth (CMT) MRI: severe cerebellar and mild 38 M 15 years Ambulant with mild assistance of 1 person, CMS Plegia of ankle and foot
disease cortical atrophy. unassisted stance for 5s max. Unsteady muscles, except right
gait with frequent trips (10/day) and tibialis anterior (MMT
falls (2/month). Refuses cane or his 2/5). Reduced range
prescribed plastic ankle-foot orthoses. of motion in both
lower extremities,
hyposensitive on feet
and toes, impaired
vibration sense overall
and blindness.
Abbreviations: ADL: activities of daily living, A/V malformation: arteriovenous malformation, CCAS/SS: cerebellar cognitive affective syndrome/Schmahmann’s syndrome, CMS: cerebellar motor syndrome, CN: cranial
nerve, CMT: Charcot-Marie-Tooth disease, CPA: cerebellopontine angle, CT-scan: computed tomography scan, CTX: cerebrotendinous xanthomatosis, EDSS: expanded disability status scale, F: female, FAC: functional
ambulation category, ICU: Intensive Care Unit, IPR: Inpatient Rehabilitation, IRU: inpatient rehabilitation unit, M: male, MRC: Medical Research Council scale, MMT: manual muscle testing, MRI: magnetic resonance
imaging, NDI: neurodiagnostic imaging, NR: not reported, OR: outpatient rehabilitation, OT: occupational therapy, P1: patient 1, PCD: paraneoplastic cerebellar degeneration, PET: positron emission tomography, PICA:
posterior inferior cerebellar artery, PT: physical therapy, SCA: spinocerebellar ataxia, TBI: traumatic brain injury, VCS: vestibulo-cerebellar syndrome.
 ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS 11

Figure 3. Non-cerebellar impairments presented per etiology cluster.

Figure 4. Intervention type per etiology cluster. The numbers stand for the number of patients who underwent this intervention.
12 A. BOGAERT ET AL.
combination with resisted walking, treadmill and overground
walking with walking aids [31]; GT on different surfaces com-
bined with head movements [35]; backward walking [23]; GT
between parallel bars [36]; analytic GT that progressed to func-
tional GT outdoors [38, 45]; GT with an exoskeleton whose speed
was adjusted to the patient’s natural pace [24]; dynamic gait
tasks and GT with an overground harness system and a gait belt
[28]; Robotic GT with a Lokomat-pro [26]; GT using a robotic
end-effector machine [43,44] and ground walking with compres-
sive garments and a weighted sandbag [44]; multi-modal walking
programs [32, 40] and other GT [39]. Two experimental studies
employed auditory rhythmic stimuli (metronomes and music)
during walking at tempi calculated from the patient’s preferred
walking cadence [47,48].
The multimodal physical therapy consisted of the following
multi-modal walking program [32]: walking with and without
various assistive devices, walking on various surfaces and in the
community, curb and obstacle negotiation, carrying objects while
walking, walking at various speeds and in varying directions,
walking with light weights on extremities, tandem gait and toe
walking, stair climbing and treadmill training. The patients had
individual sessions which were tailored to their preferences and
needs, as well as group sessions, which consisted of a balance
group and a motor group. Another example of where progression
was made from the inpatient rehabilitation to outpatient rehabil-
itation with the use of several assistive walking devices is given
in Wilson, Mitchell and Hebert [40], starting with a mechanical
ceiling-mounted gait harness, a LiteGait assisted gait system, a
platform rolling walker, a SARA Plus walking sling and finally one
person assistance. During outpatient rehabilitation, GT consisted
of pre-gait education, seated stepping exercises, gait training
using a rolling walker progressing to outdoor, uneven surfaces,
treadmill and elliptical machine training, and gait training with
U-step walker.
The second most used intervention modality was balance train-
ing (25/38 patients). It ranged from static and dynamic balance
exercises [25, 29, 31, 41,42] to sitting and standing balance exer-
cises [43, 45,46], weight bearing and shifting exercises [24] and
non-specified balance exercises [35,36, 39]. Perturbation balance
training was conducted as being part of a perturbation-based
backward balance training [23] and as being part of a
challenge-oriented gait and balance training program [28].
Different surfaces were integrated in functional balance training
[21] and a robotic end-effector machine was used [44].
Further, functional strength training was used with 12/38
patients [29, 31,32, 36, 40, 44, 46], occupational therapy or ADL
training with 9/38 patients [31, 34, 36, 38, 40, 43,44], speech
therapy with 7/38 patients [34, 37,38, 44], visual therapy with
6/38 patients [34,35, 40, 44] and climbing therapy with 6/38
patients [32,33].
Other therapies used less frequently were Frenkel’s exercises
performed lying down, sitting and standing [43], transcranial direct
current stimulation, always in combination with active therapy
[21, 42], endurance training [31,32, 44], coordination training [21,
28,29, 40], aquatic therapy [28, 32, 43], trunk stabilization exercises
[30,31, 37], vestibular rehabilitation, tai chi [32], stepping exercises,
stretching [27, 45], orthotic management [27, 45], dance based
therapy [25], mediation/relaxation [22], sensory re-education train-
ing [44], computer-based cognitive training games [42], lokomat
virtual reality games [26] and virtual rehabilitation of the upper
limb [39].
The intervention was reported to be progressively applied and
individually tailored to the patient in 22 of the 28 case reports
[21, 23–33, 35–41, 44–46].
The therapy dosage, intervention type and impact on outcome
measures based on the ICF framework can be found in Table 2
and varied across the studies. It was reported in terms of duration
of the therapy in years/months, frequency of sessions/week, dura-
tion of the session and the total amount of sessions completed.
Not all components of therapy dosage were reported in each
article. Based on the pre and post intervention values, the results
are labeled as improved (↑), remained stable (=) or deterio-
rated (↓).
Impact of the intervention on outcome measures based on the
ICF framework
The outcome measures were categorized in four categories based
on the ICF framework, which are presented in Figure 5a and b.
These figures were based on the detailed raw outcome scores
which can be found in the Supplementary Table 5. The total
number stands for the number of patients (N = 40). This time, the
two patients who were reported twice in different reports were
counted double, because of the different outcomes on the pre-
post experimental sessions [47,48] or pre-post interventions [30,31]
in both studies.
Below, the categories are outlined based on the ICF framework.
The first category considered was ataxia (code b760, body
function level), which included the Scale for the Assessment and
Rating of Ataxia (SARA), reported eight times, and the International
Cooperative Ataxia Rating Scale (ICARS), reported three times. In
this category, 7/9 patients improved on at least one of the two
scales [21, 24, 26, 36, 39, 43,44] and 2/9 patient remained stable
[23, 25] For the SARA scale, 3/8 patients [21, 26, 39] exceeded
the Minimal Clinically Important Difference of 4 points [52].
The second category consisted of measurements for gait pat-
tern (code b770, body function level). Gait speed improved in
9/11 patients [24, 27, 35, 41, 46–48] and remained stable in two
patients [22, 41]. In Bastani, Cofré Lizama, Zoghi, Blashki, Davis,
Kaye, Khan and Galea [22] the patient’s stride length asymmetry
improved after intervention, while other gait parameters remained
the stable. The study of Song, Ryu, Im, Lee and Park [25] reported
no gait speed but the patient’s step length was longer, step width
narrower and the percentage double limb support decreased
firmly after the intervention.
The third category included measurements for walking (code,
d450, activity level): the 6-min-walking test, the 10-meter walking
test, the 25-foot-walking-test, the maximum walking distance, the
ICARS-posture and gait subscale with a score ranging from 0-37
(a higher score indicating worse ataxia) [53], the functional ambu-
lation category, the Functional Independence Measure - locomo-
tion (FIM) with a 7- point ordinal scale ranging from fully
dependent (score of 1) to independent with no aids (score of 7)
[54], the level of assistance, the Dynamic gait index (DGI) with a
score ranging from 0-24 (a higher score indicating better dynamic
gait performance) [55], the SARA-gait subscale with a score rang-
ing from 0-8 (a higher score indication worse ataxia) and the
Continuity Assessment Record and Evaluation (CARE)- gait, which
the score ranges from 6 (independent) to 1 (dependent) and 0
(activity not attempted) [40]. 24/25 patients improved on at least
one of these measurements after the intervention [24, 26–32,
34–36, 38, 40–45]. One patient remained the stable pre-post inter-
vention on the FIM-locomotion score [34].
The fourth category was mobility (code, d4, activity and par-
ticipation level). Mobility covers walking (code, d450) but also
encompasses aspects of balance, carrying or moving objects and
moving around using transportation. Mainly measurements for
Table 2. Intervention type, therapy dosage and impact on outcome measures.
Intervention Outcome measures pre-post (improvement)
Total Treatment Gait pattern Activity level: Activity level:
Dosage Intervention Type Time Ataxia functions Walking mobility
Spinocerebellar degenerations
Bastani, 2018 2 weeks, 18 sessions, 2x/day, 1,5h/session Cranial-nerve non-invasive 27 h (9 h gait NR Stride length NR MiniBESTest (↑)
neuromodulation in combination with training) (↓)
three blocks of 20 min, each focusing Stride velocity
on gait performance, balance control (=)
and a relaxation/meditation period. Cadence (=)
During each block the patient used the Double support
PoNS™ device at a comfortable (=)
intensity. Stride length
asymmetry
(↑)
Goulipian, 2008 NR. Physical therapy to maintain the range of NR NR Gait speed Max walking Number of falls
Reevaluated 1 month after wearing orthopedic ankle motion and improve balance. during 10 m distance (↑) (↑)
shoes Orthopedic shoes to improve stability and walk (↑)
hold feet deformities.
Im, 2021 30 min sessions, 3 times per week for 8 weeks Perturbation-based backward balance 12 h ICARS (=) NR NR BBS (↑)
(24 total) training: walking backwards, manual SARA (=) FES (↑)
perturbation balance training (lean
back with sudden release, increasing
lean angle over time)
Kim, 2021 Three 30-min sessions/week for 8 weeks (24 Standing training (5 min) 12 h SARA (↑) Gait speed (↑) 10MWT (↑) BBS (↑)
sessions) weight bearing and shifting exercises Step length
(5 min) (↑)
overground walking in a hallway with the Cadence (↑)
exoskeleton (speed adjusted to Step width (↓)
patient’s natural pace).
Landers, 2009 Gait and balance training program: Challenge-oriented gait and balance 105 h (clinic, 42 h NR NR Level of BBS (↑)
12 weeks, 5x/week, 1.5-2 h. training program: dynamic gait tasks gait training) assistance Number of falls
Home exercise program: (30-45 min), static balance tasks with (↑) (↑)
6 days/week eyes open/closed (30-45 min), static 10MWT (↑)
and dynamic balance with external DGI (↑)
perturbation (20-30 min). First 8 weeks
with harness to prevent falls, then
minimal to moderate assistance with
gait belt.
Home exercise program: gait, balance and
coordination exercises + aquatic
exercises
Mendonça, 2021 10 sessions, 5/week for 2 weeks, 40 min each Progressive functional exercises focused 6.67 h SARA (↑) NR NR BBS (↑)
on balance (stable/unstable surfaces,
dynamic), gait training and interlimb
coordination. First 20 min cerebellar
tDCS.
Portaro, 2019 Stand-alone locomotion training: Robotic gait training with Lokomat-pro + 13 h x 3 (clinic) + SARA (↑) NR SARA gait (↑) NR
2 months, 3x/week (24 sessions) 5 min overground gait training outside 8 h x 2 (home)
walking duration: 15-50 min. of Lokomat. Lokomat virtual reality = 45 h
1 month break. games with provision of visual
Then, a-tDCS + Lokomat training: 2 months, 3x/ biofeedback. Cerebellar anodal tDCS
week (24 sessions). (a-tDCS) versus cathodal-tDCS (c-tDCS)
ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS

1 month break. Conventional home-based physiotherapy

Then, c-tDCS + Lokomat training: 2 months, 3x/ 2x/week (60 min) during the first 2
week (24 sessions) breaks.
13

(Continued)
 14
Table 2. Continued.
Intervention Outcome measures pre-post (improvement)
Total Treatment Gait pattern Activity level: Activity level:
Dosage Intervention Type Time Ataxia functions Walking mobility
Song, 2019 3/week for 8 weeks, 24 sessions Static balance, weight shifting exercises 20 h SARA (=) Step length NR BBS (↑)
(20 min). ICARS (=) (↑) FES (↑)
Dance-based therapy (modified tango, Step width (↑)
basic steps and simplest movements, Double support
A. BOGAERT ET AL.

30 min). (↑)
Traumatic brain injury
Balliet, 1987 (P1-P4) 3 months, 2x/week, 1h/session. Gait training method that discourages P1: 26 h NR NR Max walking NR
After that, self-training at home and a session upper extremity weight bearing. P2: 27 h distance (All
once every 2 months in the clinic. Stage 1: Lower extremity coordination P3: 36 h ↑)
exercise while seated. P4: 29 h Type of assistive
Stage 2: Static and dynamic balance device (All
exercises. ↑)
Stage 3: Gait training with progressively
decreasing assistance
Freund, 2010 10 weeks, 2-3 x/week, 28 sessions: Locomotor training on a BWST (assistance 37 h (inpatient) NR NR FAC (↑) BBS (↑)
- First 6 weeks: 18 sessions, 90 min of 3 persons) and overground (with + 16 h (outpatient) Max walking
- Last 4 weeks: 10 sessions, 60 min walker and assistance of 1 person). = 53 h distance (↑)
+ 12 weeks, 16 sessions, 1-2/week outpatient Trunk stabilization training 10MWT (↑)
PT (= pre-intervention period + first 6 weeks Outpatient PT: gait training with a rolling
of intervention) walker, stepping to targets, balance
training, transfers, sit to stance
training, and stair training
Freund, 2013 ** Additional 20 months, 2-3x/week, 184 sessions Locomotor training. 184 h NR NR FAC (↑) BBS (↑)
Functional electrical stimulation of his left 6MWT (↑)
dorsiflexors using the WalkAide during
gait. 10MWT (↑)
Resisted walking, treadmill and
overground walking with walking aids.
Trunk stabilization, balance training,
strength training, aerobic training.
Occupational therapy
Sartor-Glittenberg, 2014 P1: 12 months, 127 individual sessions + 61 Exercises focused on balance, P1: 95 h (ind) + NR NR 6MWT (↑) BBS (↑)
group sessions. coordination, strength, and gait. 45 h (group) = FIM locomotion FES (↑)
P3: 23 months, 89 individual sessions + 84 P1: more balance and gait training. 140 h (↑)
group sessions P3: more dynamic balance, speed, dual (12 months)
40-45 min/session task training. P3: 67 h (ind) +
All received a home exercise program of Balance group: Tai Chi and balance 63 h (group) =
flexibility and coordination exercises activities in various positions. 130 h
Motor group: aerobic exercise with bike, (23 months)
arm ergometer, and treadmill.
Also pool therapy and rock-climbing
sessions.
Stephan, 2011 (P1) 6 weeks, 2-3x per week, 40-60 min per session Climbing Training: 2 climbing walls, which P1: 13.5 h NR NR NR BBS (P1=)
were 2.5 meters high. One wall was
adjustable from 0° to 45°, the second
wall was almost vertical.
Standard climbing equipment was
available to secure the patients. The
frequency and duration of training
sessions were scheduled taking into
consideration the condition of each
patient.

(Continued)
Table 2. Continued.
Intervention Outcome measures pre-post (improvement)
Total Treatment Gait pattern Activity level: Activity level:
Dosage Intervention Type Time Ataxia functions Walking mobility
Cerebellar tumor/Paraneoplastic cerebellar degeneration
Fu, 2014 P1: 5 days (5 days post-op) Physical, occupational, and speech therapy NR NR NR FIM locomotion FIM transfers
P2: 8 days (13 days post-op) for all, with emphasis on visual and (P1 and P3 (P1 and P3
P3: 7 days (12 days post-op) vestibular rehabilitation. ↑, P2 =) ↑, P2 =)
Gill-Body, 1997 (P1) 6 week, 30- to 45-minute sessions + 30-40 min Gait exercises on different surfaces and P1: 4 h (clinic) + NR Gait speed (↑) P1: level of TUG (P1 =)
daily home program combination with head movements. 17.5 (home) = Step width (↑) assistance
VOR and balance exercises 21.5 h (↑)
Kato, 2017 21 weeks: 2 weeks before surgery, 10 weeks Gait training between parallel bars. 140 h SARA (↑) NR Level of BBS (↓)
after surgery and 9 weeks at readmission. Strength training, balance exercises, assistance
5 days/week, 80 min/session ADL training (↑)
Perlmutter, 2003 3 weeks of inpatient rehab with daily Seating in a tilt-in-space wheelchair (to NR NR NR NR FIM transfers
treatments (duration not specified). improve out-of-bed tolerance). Training (↑)
Started 2 weeks after acute phase. with weighted vest and wrist cuffs.
Proprioceptive neural facilitation for
neck and trunk stabilization on a
tilt-table. Speech therapy with a
communication board.
Cerebellar stroke
High, 2020 47 days, at least 3h/day Physical therapy, occupational therapy, 141 h NR NR FIM locomotion FIM transfers
speech therapy. Gait training: from (↑) (↑)
analytic training with assistance of BBS (↑)
multiple people to functional training
outdoor, and in the community area
(in combination with dual task).
Emphasis on catching exercises.
Family was incorporated towards the end.
Stephan, 2011 (P2) 6 weeks, 2-3x per week, 40-60 min per session Climbing Training: 2 climbing walls, which P2: 9 h NR NR NR BBS (P2 ↑)
were 2.5 meters high. One wall was
adjustable from 0° to 45°, the second
wall was almost vertical.
Standard climbing equipment was
available to secure the patients. The
frequency and duration of training
sessions were scheduled taking into
consideration the condition of each
patient.
Takimoto, 2021 7 weeks in inpatient rehab + 2 weeks (from 3 Rehabilitation: balance training (unassisted Rehab time NR SARA (↑) NR NR FBS (↑)
to 5) of VR rehab (20 min 2x/day, 5 days a step ups) and functional gait training VR: 6.66 h Mini-BESTest
week) (direction changes), which led to not (↑)
enough improvement in balance and
coordination issues at week 3.
VR rehab: Catching falling objects with
upper limbs while seated in a chair
all parameters adjustable.
(Continued)
ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
15
 16
Table 2. Continued.
Intervention Outcome measures pre-post (improvement)
Total Treatment Gait pattern Activity level: Activity level:
Dosage Intervention Type Time Ataxia functions Walking mobility
Wilson, 2017 14 months: Intensive Care Unit: ICU: NR NR NR CARE gait (↑) TUG (↑)
6 weeks in the ICU (19 OT, 12 PT, 17 SLP), OT: Multi-sensory stimulation therapy and CARE bed
10 weeks of IPR (54 OT, 51 PT, 47 SLP, 22 neuromuscular re-education mobility (↑)
recreational therapy) 3 h/day, 5-7 days/ techniques. CARE lateral
A. BOGAERT ET AL.

week. PT: Passive, then active assistive transfers (↑)

41 weeks of outpatient rehabilitation (117 OT,
115 PT, 110 SLP) 3 days/week, 45-60 min/
session.
Cerebellar ataxia, not specified
Abbud, 2014 P1: 6 months, 2x/week
P2: 8 months, 2x/week
Pilloni, 2019 8 weeks, 5x/week, daily, 20 min (60 sessions)
tDCS with 20 min simultaneous cognitive
training, followed by 20 min of physical
exercises
mobilization, education to caregivers, CARE sit to
early mobility in bed and with tilt stand (↑)
table.
Inpatient Rehabilitation: IPR: 180 h
OT: upper limb passive and active (10 weeks,
mobilization, training of ADLs, visual 50-60 h of PT)
rehabilitation (eye patch for diplopia,
gaze stabilization, hand-eye
coordination with Wii), functional
reaching and strengthening tasks in
wheelchair and standing.
PT: Open chain active movements (w/ and
w/o weights) progressed to closed
chain strength exercises. Pre-gait
reconditioning with standing frame
and SARA Plus: Then, ceiling-mounted
harness with max assist of 2 persons.
Lastly, gait training with LiteGait
system and unsupported gait training
with walker. Stair training with 2
persons. Use of a recumbent elliptical
machine.
Outpatient Rehabilitation: OR: 108 h
OT: Functional core strengthening, upper (41 weeks, 35 h
limb strength and dexterity exercises of PT)
(also writing), balance and coordination
tasks, basic and advanced ADL and
transfers training.
PT: dynamic and static sitting and
standing balance activities, pre-gait
and family education. Core stability
and fall prevention. Gait training
indoor and outdoor. Advanced strength
and balance activities. LiteGait and
treadmill. Use of U-step with
supervision/min assist.
Cane, walking poles, walker for both P1: 52 sessions NR Gait speed with P1: 6MWT (↑) P2: BBS (↑)
patients: no improvement. P2: 68 sessions different aids
P1: Gait training, static balance, stair Time NR (P1 ↑ and
climbing, transfers P2 =)
all with use of an intervention dog
P2: Gait and balance treatment using an
assistance dog
Exercise program: warm-up, core 60 h (20 h exercise) NR NR 25FWT (↑) TUG (↓)
strengthening, static and dynamic
balance. Computer-based cognitive
training games. tDCS.

(Continued)
 (Continued)

Table 2. Continued.
Intervention Outcome measures pre-post (improvement)
Total Treatment Gait pattern Activity level: Activity level:
Dosage Intervention Type Time Ataxia functions Walking mobility
Miscellaneous
Balliet, 1987 (P5) 3 months, 2x/week, 1h/session. Gait training method that discourages P5: 26 h NR NR Max walking
After that, self-training at home and a session upper extremity weight bearing. distance (↑)
once every 2 months in the clinic. Stage 1: Lower extremity coordination Type of assistive
exercise while seated. device (↑)
Stage 2: Static and dynamic balance
exercises.
Stage 3: Gait training with progressively
decreasing assistance
Cha 2020 2 months, 5x/week, 30 min/day + Frenkel’s Rehabilitation program comprising 22 h ICARS (↑) NR ICARS posture BBS (↑)
exercises 2x/day for 30 min each physical therapy, occupational therapy, + 44 h of Frenkel’s and gait (↑)
whole body therapeutic pool therapy, exercises Walking
and robotic-assisted (end-effector type) = 66 h distance (↑)
gait training. Sitting balance, standing
balance and gait training with anterior
walker.
Frenkel’s exercises: performed lying down,
sitting and standing.
Training in using electric wheelchair in
the last 2 weeks.
Gill-Body, 1997 (P2) 6 week, 30- to 45-minute sessions + 30-40 min Gait exercises on different surfaces and P2: 3 h (clinic) + 12 NR Gait speed (↑) NR TUG (P2 ↑)
daily home program combination with head movements. (home) = 15 h Step width (↑)
VOR and balance exercises
Lo Voi, 2021 20-sessions of 60 min over a 10 week period Physiotherapy: Warming up on treadmill, 20 h NR Speed (↑) NR BBS (↑)
passive stretching and mobilization, Cadence (↑)
coordination exercises (standing Gait cycle
balance (i.e., before on two and one duration (↑)
leg), stair climbing, standing heel-to- Double limb
toe balance, kneeling, hip support (↑)
adduction-abduction, hip
intra-extrarotation, and sitting to Step length (=)
standing) Stride length
Virtual reality exergames with biofeedback (↑)
Moumdjian, 2021 1 session, 3 bouts of 2 min for each walking 3 walking conditions: silence, 6 min NR Gait speed (↑) NR NR
condition non-adaptive music (constant beat of Cadence (↑)
−12% from baseline cadence), and Stride length
adaptive music (adapt the music (↑)
tempo to the cadence, speedup and
slow down were induced when off
from −12% of the baseline cadence)
Moumdjian, 2022 1 session, 14 bouts of 1 min for each walking Walking while synchronizing with 2 14 min NR Gait speed (↑) NR NR
condition different stimuli (metronome and Cadence (↑)
music) at 7 tempi (-12%, − 8%, −4%, Stride length
0%, +4% +8%, +12% of the baseline (↑)
cadence), resulting in a total of 14
different walking conditions.

(Continued)
ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
17
 18
Table 2. Continued.
Intervention Outcome measures pre-post (improvement)
Total Treatment Gait pattern Activity level: Activity level:
Dosage Intervention Type Time Ataxia functions Walking mobility
Stanley, 2021 Inpatient rehab for 3 months. Then visual Rehabilitation: strength exercises, Rehabilitation time SARA (↑) NR 6MWT (↑) BBS (↑)
rehab (45 min every two weeks, 24 endurance training, balance and gait NR TUG (↑)
sessions) and rehab 2/week for 48 weeks training using a robotic end-effector Visual rehab: 18 h
machine, and ground walking with (supervised) +
A. BOGAERT ET AL.

compressive garments and weighted 15-20 min/day

sandbag (home)
also, sensory re-education training,
oromotor exercises and speech therapy.
Functional writing, typing, and
computer handling training. Given aids
and adaptations for ADL.
VR: basic visual motor skills, eye
stretching and ROM, laterality training,
central-peripheral awareness,
stimulation of left lateral rectus with
post-VOR, selective occlusion, yoked
prism and relieve prism. Home
activities for 15-20 min daily.
Stephan, 2011 (P3 and 6 weeks, 2-3x per week, 40-60 min per session Climbing Training: 2 climbing walls, which P3: 13.5 h NR NR NR BBS (P3 ↑ and
P4) were 2.5 m high. One wall was P4: 18 h P4 =)
adjustable from 0° to 45°, the second
wall was almost vertical.
Standard climbing equipment was
available to secure the patients. The
frequency and duration of training
sessions were scheduled taking into
consideration the condition of each
patient.
Vinci, 2003 Dosage during study NR. Stretching to recover ROM (mainly knee NR NR NR Level of Number of falls
After discharge he continued the exercises extension). Use of an acceptable assistance (↑)
with his physiotherapist for 6 months, 3x/ orthotic device for foot drop. Standing (↑)
week balance exercises with cues to correct
for compensations. Gait training: first
in a narrow corridor and then uneven
path in a courtyard (using bright
targets to guide the patient).
Abbreviations: 6MWT: six-minute walking test, 10MWT: 10-meter walking test, 25FWT: 25-feet walking test, ADL: activities of daily living, BBS: Berg balance scale, BWST: body weight support treadmill, CARE:
Continuity Assessment Record and Evaluation, DGI: Dynamic gait index, FAC: functional ambulation category, FES: falls efficacy scale, FIM: functional independence measure, ICARS: International Cooperative Ataxia
Rating Scale, ICU: intensive care unit, Ind: individual, IPR: inpatient rehabilitation, Mini-BESTest: mini-Balance Evaluation Systems Test, NR: not reported, OR: outpatient rehabilitation, OT: occupational therapy, P1:
patient 1, PNF: proprioceptive neuromuscular facilitation, PoNS™: portable neuromodulation stimulator, PT: physical therapy, ROM: range of motion, SARA: Scale for the Assessment and Rating of Ataxia, SLP: speech
language pathology, tDCS: transcranial direct current stimulation, TUG: timed up-and-go test, UWC: usual walking cadence, VOR: vestibulo-ocular reflex, VR: virtual reality.
 ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
assessing balance were used: the Berg Balance Scale (BBS) which
is scored on a 0-56 scale, with a < 45/56 being a cut off point
for considering an assistive device or supervision in elderly [56],
the Mini Balance-Evaluation-Systems-Test whose score ranges
from 0-28 (a higher score indicating better balance), FIM-transfers,
Timed Up and Go (TUG) with a time of less than 10 s indicates
independent mobility in frail elderly [57], Number of falls, Falls
efficacy scale which measures fear of falling with a total score
ranging from 0-100 (100= high self-efficacy) [58], and the CARE-
bed mobility, CARE- lateral transfers and the CARE-sit to
stand [40].
After the intervention, 25/31 patients improved on at least one
of these measurements [21–25, 27,28, 30–35, 37–41, 43–46], 4/30
patients remained stable [33–35], one patient deteriorated of 1
point on the BBS [36] and one patient deteriorated on 0.5 s on
the TUG [42].
Discussion
In this work, we systematically reviewed case reports and case
series of PwCI and reported on the aetiologies and clinical pre-
sentation with the attempt to separate cerebellar and
non-cerebellar impairments of the included patients. In addition,
physical rehabilitation approaches applied on the included patients
and their impact on ataxia, gait pattern, walking and mobility
according to the ICF framework were systematically reviewed and
reported.
As anticipated, the etiology for the cerebellar impairments
varied across case reports. The main clusters identified were spi-
nocerebellar degenerations (21.6%), traumatic brain injury (21.6%),
lesions or degeneration related to (para)neoplasms (16.2%), cer-
ebellar strokes (11%), non-specified cerebellar ataxia (8%) and a
miscellaneous group of varied etiologies (21.6%). Based on the
clinical presentation, all patients had a cerebellar motor syndrome
(CMS), as each presented with dysfunctions in general motor
behavior. Of all patients, 37% had this syndrome in combination
with a vestibular cerebellar syndrome (VCS) and/or a cerebellar
cognitive affective syndrome/Schmahmann’s syndrome (CCAS/SS).
We acknowledge that this is an estimation, thus to be interpreted
with caution as not all dysfunctions linked to the cerebellar syn-
dromes may be exclusively due to cerebellar damage. In fact,
dysmetria can also be observed when lesions are present in the
brainstem, basal ganglia or anatomical loops encompassing the
cerebellum [19] as for example in the case reported in Cha and
Kim [43], who had severe dysmetria which could be linked to
both cerebellar atrophy and decreased signal intensity at bilateral
temporal and parietal lobes observed on a PET-CT scan. Similarly,
in Sartor-Glittenberg and Brickner [32], the cognitive deficits of
the two patients with traumatic brain injury could be due to the
accompanying cerebral damage and thus may not be purely of
cerebellar origin. Further evidence of the heterogeneity in clinical
presentation of PwCI can be found in the vast range of
non-cerebellar symptoms that were identified.
The patient profiles described above confirm that disentangling
cerebellar and non-cerebellar impairments is challenging. In order
to document heterogeneity, one solution is to assess PwCI within
the scope of a comprehensive test battery, similarly what has
been done for other neurological conditions, such as Multiple
Sclerosis and stroke [59,60]. This would allow to characterize
patients not only based on the diagnosis or etiology, but also on
their functional status. Thereafter, defining a personalized reha-
bilitation strategy within the framework of the ICF could increase
effectiveness of the rehabilitation program.
19
In this context, we categorized the outcome measures used in
the included case reports into the ICF framework as the following:
ataxia, gait pattern, walking and mobility. The outcome measures
were almost all improved after the physical rehabilitation across
the case reports, with no observable differences in improvement
between levels of the ICF. However, a lack of comprehensive
assessment on function level of the ICF was seen (Figure 5), as
assessments of ataxia and gait pattern were reported in only a
few patients (in 22% of the total patients included in this review).
This could be due to the fact that the interventions that were
included in these studies were task-oriented and activity level
based interventions, and therefore the outcome measures selected
were at activity level of the ICF such as walking and mobility. Yet,
this is problematic as evidence is not gathered on the impact of
the interventions on ataxia and gait pattern itself. In other words,
the impact of the intervention on the cerebellar impairments
cannot be deduced. This was most true for the clusters traumatic
brain injury and cerebellar ataxia of unspecified origins where
these outcomes were not reported at all. To assess ataxia, the
SARA and the ICARS were the most commonly used scales. The
SARA is shown to be reliable and valid in spinocerebellar ataxia
(SCA) patients [61] and in non-SCA patients [62] and thus should
be used routinely in the assessment of PwCI.
In regards to interventions, studies mostly applied multimodal
exercise programs which reflect an integrated approach that tack-
les the impairments on different levels of the ICF. There was
merely a combination of function and activity level, such as gait
training (conducted by 71% of the patients in this review), balance
training (66%) and strength training (32%). The predominant use
of these types of treatments was also observed in a recent sys-
tematic review and meta-analysis [18], who included studies inves-
tigating exercise-based interventions in PwCI. They concluded that
the interventions resulted in improvements in balance (i.e., func-
tion and activity level of the ICF) and partially in severity of ataxia
(i.e., function level of the ICF), but not in functional independence
(i.e., activity and participation level of the ICF) [18].
A total of 24% of the patients included in this review under-
went occupational therapy (activity and participation level) and
made an overall improvement in functional independence after
the intervention [31, 34, 38, 40, 43]. These results are in agreement
with a review [15] stating that performing occupational therapy
in conjunction with physical therapy might improve global func-
tional status in patients with ataxia. An example of a multimodal
approach tailored to the patient on all levels of the ICF can be
found in the case reported in Wilson, Mitchell and Hebert [40],
where the authors applied: on function level, multi-sensory stim-
ulation therapy, neuromuscular re-education techniques, stretch-
ing, active/passive mobilization and visual rehabilitation; on activity
level, task-oriented gait training, functional reaching and strength-
ening exercises; on participation level, gait training in the commu-
nity. In addition, involvement of the family (environmental levels)
was also promoted.
In alignment with the idea of using more participation based
interventions, within this review, a variety of therapies were used
alongside multimodal training program and often resulted in
improvement of mobility. These therapies were climbing,
dance-based therapy, walking to music, tai chi, whole body pool
therapy, mediation/relaxation, sensory re-education training and
computer-based cognitive training. These types of training can
open avenues for varying training and inclusion of community-based
training [63] for this population.
In PwCI and especially those with CMS, deficits stemming from
impaired coordination should be assessed and addressed appro-
priately. As previously mentioned, given the lack of quantification
20 A. BOGAERT ET AL.
Figure 5. Impact of the intervention on outcome measures according to the ICF framework. a. Overview and b. Classified by etiology cluster.
on ataxia and gait pattern in the studies included in this review,
it is not possible to conclude on the impact of interventions on
impairments of coordination, which can be seen as an essential
component of a cerebellar dysfunction. The interventions that
were applied to target ataxia in this review (on 16% of the total
number of patients) was coordination training. Previously, a study
on 16 patients with progressive cerebellar ataxia reported that
an intensive coordination training of 4 weeks reduced ataxia [64].
Therefore the inclusion of coordination training may have a ben-
eficial effect on ataxia, and should be verified within larger scale
studies which include quantification of the impairments of
coordination. In addition, studies may consider the use of rhythm
based intervention as a method of coordination training, as evi-
dence is now emerging for the feasibility of using such applica-
tions in PwCI with different neurological etiologies, and its impact
on motor timing and gait pattern [48, 65].
Another relevant strategy is oculomotor training [66]. Four of
the case-reports included in this review applied some form of
visual rehabilitation (the training was not specified) which con-
tributed, in almost all of the patients, to achieving improvements
in mobility [34,35, 40, 44]. Noteworthy, is that oculomotor control,
which has been shown to promote important repercussions on
 ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
balance and gait impairments [66], is a key feature of cerebellar
ataxia, and is not assessed with the SARA.
Moreover, factors such as emotional wellbeing, including fear
of conducting activities were reported in this review [22, 35]. This
is relevant given as evidence exists that persons with cerebellar
ataxia show emotional health issues [13]. Given these results we
also advocate to integrate both environmental and personal fac-
tors when personalizing a physical rehabilitation program.
Further, a crucial concept worthy of discussion in relation to
individualized targeting of rehabilitation in PwCI is cognitive
reserve, defined as the capacity of the cerebellum to compensate
for tissue damage or loss of function resulting from many different
etiologies [67]. Structural cerebellar reserve is present when other
(extra) cerebellar structures compensate for acute focal damage.
In the case report of Takimoto et al. [39] this could be a mech-
anism that explains the successful treatment outcome of a patient
with an acute cerebellar/brainstem infarction with virtual reality
guided rehabilitation. However, even in case of cerebellar degen-
eration, the affected area itself can compensate for the slowly
evolving cerebellar lesion. This is called functional cerebellar reserve
[67]. The assessment of cerebellar reserve is challenging but has
been shown possible by measuring the preservation of predictive
control in a smooth pursuit task [68,69]. The question remains if
this is an applicable method in daily practice, as it has been
advocated that the therapeutic strategy of PwCI should be based
on the assessment of cerebellar motor reserve before administra-
tion of treatment [67].
Limitations of this systematic review is that only data from
published studies were included, which may have implications to
potential selection bias. Another limitation is the selective sam-
pling process of enrollment of participants in case reports and
case series, so that the sample collected in this review are not
representative for the whole population of PwCI. The question
rises if generalization is even possible in this population when
taking into account the heterogeneity that has become clear in
this systematic review. This raises challenges to pool and compare
data across studies. However, exploration of variation of effects
of rehabilitation intervention by study or participant level char-
acteristic (such as estimation of intervention between effect and
covariates) was not conducted in this review.
In this work, a disentanglement of individual cases has led us
towards a clearer view on the functional status and clinical pre-
sentation with both cerebellar and non-cerebellar impairments.
The results of this review provides evidence that distinguishing
cerebellar and non-cerebellar symptoms and categorizing patients
within the three cerebellar syndromes can assist to understand
heterogeneity, and in turn may guide clinical practice. Another
result identified in this review is the fewer reporting on the body
function level of ICF in terms of ataxia and gait pattern. Thus an
advocacy is made to include assessments on the body function
level of the ICF in clinical practice as well. Finally, the results also
support that multimodal and patient-tailored strategies are prom-
ising for targeting walking and mobility in persons with cerebellar
impairments.
Conclusion
This systematic review provides an overview and a synthetic
analysis of the heterogeneity of etiology and clinical presenta-
tion in PwCI, and provides evidence for the need of a compre-
hensive test battery to encompass the key features of a PwCI
on the different levels of the ICF. The multimodal and
patient-tailored physical rehabilitation strategies used in the
21
case reports had an overall positive impact on walking and
mobility. Yet, the impact of the interventions on ataxia and gait
pattern were underreported, and thus we advocate for including
outcome measures to assess the body function level of the ICF
in PwCI within physical rehabilitation interventions.
Disclosure statement
No potential conflict of interest was reported by the authors.
Author contributions
A.B., F.R, P.C, P.F., and L.M. contributed to the conceptualization of
aims of the systematic review and defining the search strategy.
A.B. and F.R. screened articles for inclusion and completed the
data extraction L.M. contributed as the third reviewer and resolved
discrepancies. A.B., F.R, P.C, P.F., and L.M. contributed to the writing
and revising the manuscript.
Funding
Fonds Wetenschappelijk Onderzoek (FWO) project obtained by
Prof. Peter Feys, grant number G082021N; Fonds Wetenschappelijk
Onderzoek (FWO) project obtained by dr. Lousin Moumdjian, grant
number 1295923 N; Stiftelsen Promobilia grant obtained by dr.
Lousin Moumdjian, grant number 20110.
References
[1] Marsden JF. Cerebellar ataxia. In: Day BL, Lord SR, editors.
Balance, gait, and falls. Elsevier; 2018. p. 261–281.
[2] D’Angelo E. Physiology of the cerebellum. Handb Clin Neurol.
2018;154:85–108.
[3] Manto M. Mechanisms of human cerebellar dysmetria: ex-
perimental evidence and current conceptual bases. J
Neuroeng Rehab. 2009;6:10.
[4] Stolze H, Klebe S, Petersen G, et al. Typical features of cer-
ebellar ataxic gait. J Neurol Neurosurg Psychiatry.
2002;73(3):310–312. doi: 10.1136/jnnp.73.3.310.
[5] Ilg W, Golla H, Thier P, et al. Specific influences of cerebellar
dysfunctions on gait. Brain. 2007;130(Pt 3):786–798. doi:
10.1093/brain/awl376.
[6] Marsden J, Harris C. Cerebellar ataxia: pathophysiology and
rehabilitation. Clin Rehabil. 2011;25(3):195–216. doi:
10.1177/0269215510382495.
[7] Cabaraux P, Gandini J, Manto M. The three cornerstones of
cerebellar ataxia: closing the loop of 200 years of cerebellar
research. In: Cerebellum as a CNS hub contemporary clinical.
Neuroscience. Cham: Springer; 2021. p. 459–478.
[8] Schmahmann JD, Sherman JC. The cerebellar cognitive af-
fective syndrome. Brain. 1998;121(Pt 4):561–579. doi: 10.1093/
brain/121.4.561.
[9] Marsden JF. Cerebellar ataxia. Handb Clin Neurol.
2018;159:261–281.
[10] Hadjivassiliou M, Martindale J, Shanmugarajah P, et al. Causes
of progressive cerebellar ataxia: prospective evaluation of
1500 patients. J Neurol Neurosurg Psychiatry. 2017;88(4):301–
309. doi: 10.1136/jnnp-2016-314863.
[11] Manto M, Gandini J, Feil K, et al. Cerebellar ataxias: an up-
date. Curr Opin Neurol. 2020;33(1):150–160. doi: 10.1097/
WCO.0000000000000774.
22 A. BOGAERT ET AL.
[12] World Health O. International classification of functioning,
disability and health: ICF. Geneva: World Health Organization;
2001.
[13] Joyce MR, Nadkarni PA, Kronemer SI, et al. Quality of life
changes following the onset of cerebellar ataxia: symptoms
and concerns self-reported by ataxia patients and informants.
Cerebellum. 2022;21(4):592–605. doi: 10.1007/
s12311-022-01393-5.
[14] Marquer A, Barbieri G, Pérennou D. The assessment and
treatment of postural disorders in cerebellar ataxia: a sys-
tematic review. Ann Phys Rehabil Med. 2014;57(2):67–78. doi:
10.1016/j.rehab.2014.01.002.
[15] Fonteyn EM, Keus SH, Verstappen CC, et al. The effectiveness
of allied health care in patients with ataxia: a systematic
review. J Neurol. 2014;261(2):251–258. doi: 10.1007/s00415-
013-6910-6.
[16] He M, Zhang HN, Tang ZC, et al. Balance and coordination
training for patients with genetic degenerative ataxia: a sys-
tematic review. J Neurol. 2021;268(10):3690–3705. doi:
10.1007/s00415-020-09938-6.
[17] Hartley H, Cassidy E, Bunn L, et al. Exercise and physical
therapy interventions for children with ataxia: a systematic
review. Cerebellum. 2019;18(5):951–968. doi: 10.1007/
s12311-019-01063-z.
[18] Winser S, Chan HK, Chen WK, et al. Effects of therapeutic
exercise on disease severity, balance, and functional inde-
pendence among individuals with cerebellar ataxia: a sys-
tematic review with meta-analysis. Physiother Theory Pract.
2023;39(7):1355–1375.
[19] Cabaraux P, Gandini J, Manto M. The three cornerstones of
cerebellar ataxia: closing the loop of 200 years of cerebellar
research. In: Mizusawa H, Kakei S, editors. Cerebellum as a
CNS Hub. Contemporary Clinical Neuroscience. Cham:
Springer; 2021. p. 459–478. doi: 10.1007/978-3-030-75817-
2_23.
[20] Riley DS, Barber MS, Kienle GS, et al. CARE guidelines for
case reports: explanation and elaboration document. J Clin
Epidemiol. 2017;89:218–235. doi: 10.1016/j.jcline -
pi.2017.04.026.
[21] Mendonça MS, Goulardins JB, Souza CO, et al. Application
of add-on anodal cerebellar direct current stimulation for
balance rehabilitation in cerebellar ataxia: a case report. Rev
Pesq Fisio. 2021;11(2):427–434. doi: 10.17267/2238-2704rpf.
v11i2.3636.
[22] Bastani A, Cofré Lizama LE, Zoghi M, et al. The combined
effect of cranial-nerve non-invasive neuromodulation with
high-intensity physiotherapy on gait and balance in a patient
with cerebellar degeneration: a case report. Cerebellum
Ataxias. 2018;5:6. doi: 10.1186/s40673-018-0084-z.
[23] Im C-H, Park J-H. Effects of a Perturbation-Based balance
training on compensatory postural responses to backward
loss of balance in patients with cerebellar disease: a case
study. Asian J Kinesiol. 2021;23(1):53–61. doi: 10.15758/
ajk.2021.23.1.53.
[24] Kim SH, Han JY, Song MK, et al. Effectiveness of robotic
exoskeleton-assisted gait training in spinocerebellar ataxia:
a case report. Sensors (Basel). 2021;21(14):4874. doi: 10.3390/
s21144874.
[25] Song Y-G, Ryu Y-U, Im S-J, et al. Effects of dance-based move-
ment therapy on balance, gait, and psychological functions in
severe cerebellar ataxia: a case study. Physiother Theory Pract.
2019;35(8):756–763. doi: 10.1080/09593985.2018.1457119.
[26] Portaro S, Russo M, Bramanti A, et al. The role of robotic
gait training and tDCS in Friedrich ataxia rehabilitation: a
case report. Medicine (Baltimore). 2019;98(8):e14447. doi:
10.1097/MD.0000000000014447.
[27] Goulipian C, Bensoussan L, Viton JM, et al. Orthopedic shoes
improve gait in Friedreich’s ataxia: a clinical and quantified
case study. Eur J Phys Rehabil Med. 2008;44:93–98.
[28] Landers M, Adams M, Acosta K, et al. Challenge-oriented
gait and balance training in sporadic olivopontocerebellar
atrophy: a case study. J Neurol Phys Ther. 2009;33(3):160–168.
doi: 10.1097/NPT.0b013e3181b511f4.
[29] Balliet R, Harbst KB, Kim D, et al. Retraining of functional
gait through the reduction of upper extremity weight-bearing
in chronic cerebellar ataxia. Disabil Rehabil. 1986;8:148–153.
[30] Freund JE, Stetts DM. Use of trunk stabilization and locomo-
tor training in an adult with cerebellar ataxia: a single system
design. Physiother Theory Pract. 2010;26(7):447–458. doi:
10.3109/09593980903532234.
[31] Freund JE, Stetts DM. Continued recovery in an adult with
cerebellar ataxia. Physiother Theory Pract. 2013;29(2):150–158.
doi: 10.3109/09593985.2012.699605.
[32] Sartor-Glittenberg C, Brickner L. A multidimensional physical
therapy program for individuals with cerebellar ataxia sec-
ondary to traumatic brain injury: a case series. Physiother
Theor y Pract. 2014;30(2):138–148. doi: 10.3109/
09593985.2013.819952.
[33] Stephan MA, Krattinger S, Pasquier J, et al. Effect of long-term
climbing training on cerebellar ataxia: a case series. Rehabil
Res Pract. 2011;2011:525879.
[34] Fu JB, Nelson MB, Bruera E. Inpatient rehabilitation of cere-
bellopontine angle epidermoid cysts: report of 3 cases.
Neurosurg Q. 2014;24(3):197–202. doi: 10.1097/WNQ.
0b013e3182a2fac7.
[35] Gill-Body KM, Popat RA, Parker SW, et al. Rehabilitation of
balance in two patients with cerebellar dysfunction. Phys
Ther. 1997;77(5):534–552. doi: 10.1093/ptj/77.5.534.
[36] Kato N, Hashida G, Konaka K. Rehabilitation for a patient
with anti-Yo antibody-positive paraneoplastic cerebellar de-
generation caused by breast cancer: a case report and lit-
erature review. Medicine (Baltimore). 2017;96(44):e8468. doi:
10.1097/MD.0000000000008468.
[37] Perlmutter E, Gregory PC. Rehabilitation treatment options
for a patient with paraneoplastic cerebellar degeneration. Am
J Phys Med Rehabil. 2003;82(2):158–162. doi: 10.1097/
00002060-200302000-00014.
[38] High CM, Andrews AW. Rehabilitation for a complex patient
following cerebellar hemorrhage and obstructive hydroceph-
alus: a case report. Physiother Theory Pract. 2020;36(8):965–
971. doi: 10.1080/09593985.2018.1519007.
[39] Takimoto K, Omon K, Murakawa Y, et al. Case of cerebellar
ataxia successfully treated by virtual reality-guided rehabil-
itation. BMJ Case Rep. 2021;14(5):e242287. doi: 10.1136/
bcr-2021-242287.
[40] Wilson CM, Mitchell CL, Hebert KM. Cerebellar stroke occu-
pational therapy and physical therapy management from
intensive care unit to outpatient: a case report. Cureus.
2017;9(12):e1949. doi: 10.7759/cureus.1949.
[41] Abbud G, Janelle C, Vocos M. The use of a trained dog as
a gait aid for clients with ataxia: a case report. Physiother
Can. 2014;66(1):33–35. doi: 10.3138/ptc.2013-17.
[42] Pilloni G, Shaw M, Feinberg C. Long term at-home treatment
with transcranial direct current stimulation (tDCS) improves
symptoms of cerebellar ataxia: a case report. J Neuroeng
Rehab. 2019;16:41.
[43] Cha JM, Kim HS. Functional improvement after taking reha-
bilitation program in cerebellar ataxia in a patient with sys-
 ASSESSMENT AND PHYSICAL REHABILITATION IN PERSONS WITH CEREBELLAR IMPAIRMENTS
temic lupus erythematosus: a case report. Brain Neurorehabil.
2020;13(2):e11. doi: 10.12786/bn.2020.13.e11.
[44] Stanley T, Ng CY, Mazlan M. Neuro-optometric vision reha-
bilitation after pontine cavernoma: a case report. JUMMEC.
2021;24(2):15–20. doi: 10.22452/jummec.vol24no2.3.
[45] Vinci P. Gait rehabilitation in a patient affected with
Charcot-Marie-Tooth disease associated with pyramidal and
cerebellar features and blindness. Arch Phys Med Rehabil.
2003;84(5):762–765. doi: 10.1016/S0003-9993(03)04907-9.
[46] Lo Voi E, Basile GC, Bramanti A, et al. Cerebellar atrophy
associated with primary Sjögren’s syndrome: diagnosis, ther-
apy, and virtual reality rehabilitation: a case report. Innov
Clin Neurosci. 2021;18:11–17.
[47] Moumdjian L, Rosso M, Moens B, et al. A case-study of a
person with multiple sclerosis and cerebellar ataxia synchro-
nizing finger-taps and foot-steps to music and metronomes.
Neuroimmunol Rep. 2022;2:100101. doi: 10.1016/j.
nerep.2022.100101.
[48] Moumdjian L, Moens B, Van Wijmeersh B, et al. Application
of step and beat alignment approaches and its effect on
gait in progressive multiple sclerosis with severe cerebellar
ataxia: a proof of concept case study. Mult Scler.
2022;28(3):492–495. doi: 10.1177/13524585211054000.
[49] Mehrholz J, Wagner K, Rutte K, et al. Predictive validity and
responsiveness of the functional ambulation category in
hemiparetic patients after stroke. Arch Phys Med Rehabil.
2007;88(10):1314–1319. doi: 10.1016/j.apmr.2007.06.764.
[50] Islam M, Hoggard N, Hadjivassiliou M. Cerebrotendinous xan-
thomatosis: diversity of presentation and refining treatment
with chenodeoxycholic acid. Cerebellum Ataxias. 2021;8(1):5.
doi: 10.1186/s40673-021-00128-2.
[51] Fox RI, Howell FV, Bone RC, et al. Primary sjogren syndrome:
clinical and immunopathologic features. Semin Arthritis Rheum.
1984;14(2):77–105. doi: 10.1016/0049-0172(84)90001-5.
[52] Schmitz-Hübsch T, Fimmers R, Rakowicz M, et al.
Responsiveness of different rating instruments in spinocer-
ebellar ataxia patients. Neurology. 2010;74(8):678–684. doi:
10.1212/WNL.0b013e3181d1a6c9.
[53] Trouillas P, Takayanagi T, Hallett M, et al. 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.
1997;145(2):205–211. doi: 10.1016/s0022-510x(96)00231-6.
[54] Dakin L, Peel N. Effect of accelerometry on the functional
mobility of older rehabilitation inpatients as measured by
functional independence measure–locomotion (FIM) gain: a
retrospective matched cohort study. J Nutr Health Aging.
2011;15(5):382–386. doi: 10.1007/s12603-010-0129-3.
[55] Shumway-Cook A, Woollacott MH. Motor control: theory and
practical applications. Baltimore (MD): Williams and Wilkins;
1995.
23
[56] Berg KO, Wood-Dauphinee SL, Williams JI, et al. Measuring
balance in the elderly: validation of an instrument. Can J
Public Health/Rev Can Sante’e Publique. 1992;83: S7–S11.
[57] Podsiadlo D, Richardson S. The timed "up & go": a test of
basic functional mobility for frail elderly persons. J Am
Geriatr Soc. 1991;39(2):142–148. doi: 10.1111/j.1532-5415.1991.
tb01616.x.
[58] Tinetti ME, Richman D, Powell L. Falls efficacy as a measure
of fear of falling. J Gerontol. 1990;45(6):P239–243. doi:
10.1093/geronj/45.6.p239.
[59] Tiftikçioğlu B. Multiple sclerosis functional composite (MSFC):
scoring instructions. Noro Psikiyatr Ars. 2018;55: s 46–s48.
[60] Spilker J, Kongable G, Barch C, et al. Using the NIH stroke
scale to assess stroke patients. The NINDS rt-PA stroke study
group. J Neurosci Nurs. 1997;29(6):384–392. doi:
10.1097/01376517-199712000-00008.
[61] Schmitz-Hubsch T, Du Montcel ST, Baliko L, et al. Scale for
the assessment and rating of ataxia: development of a new
clinical scale. Neurology. 2006;66(11):1717–1720. doi:
10.1212/01.wnl.0000219042.60538.92.
[62] Weyer A, Abele M, Schmitz-Hübsch T, et al. Reliability and
validity of the scale for the assessment and rating of ataxia:
a study in 64 ataxia patients. Mov Disord. 2007;22(11):1633–
1637. doi: 10.1002/mds.21544.
[63] Chard SE. Community neurorehabilitation: a synthesis of
current evidence and future research directions. NeuroRx.
2006;3(4):525–534. doi: 10.1016/j.nurx.2006.07.002.
[64] Ilg W, Synofzik M, Brotz D, et al. Intensive coordinative train-
ing improves motor performance in degenerative cerebellar
disease. Neurology. 2009;73(22):1823–1830. doi: 10.1212/
WNL.0b013e3181c33adf.
[65] Kobinata N, Ueno M, Imanishi Y, et al. Immediate effects of
rhythmic auditory stimulation on gait in stroke patients in
relation to the lesion site. J Phys Ther Sci. 2016;28(9):2441–
2444. doi: 10.1589/jpts.28.2441.
[66] Crowdy KA, Kaur-Mann D, Cooper HL, et al. Rehearsal by eye
movement improves visuomotor performance in cerebellar
patients. Exp Brain Res. 2002;146(2):244–247. doi: 10.1007/
s00221-002-1171-0.
[67] Mitoma H, Buffo A, Gelfo F, et al. Consensus paper. Cerebellar
reserve: from cerebellar physiology to cerebellar disorders.
Cerebellum. 2020;19(1):131–153. doi: 10.1007/s12311-019-
01091-9.
[68] Lee J, Kagamihara Y, Kakei S. A new method for functional
evaluation of motor commands in patients with cerebellar
ataxia. PLoS One. 2015;10(7):e0132983. doi: 10.1371/journal.
pone.0132983.
[69] Kakei S, Lee J, Mitoma H, et al. Contribution of the cerebel-
lum to predictive motor control and its evaluation in ataxic
patients. Front Hum Neurosci. 2019;13:216. doi: 10.3389/fn-
hum.2019.00216.