Neurology Asia 2020; 25(1) : 75 – 80

Rehabilitating individuals with spinocerebellar ataxia:

Experiences from impairment-based rehabilitation
through multidisciplinary care approach
Fatimah Ahmedy MBBCh MRehabMed, 1Yuen Woei Neoh MBBS, MRehabMed, 1Lydia Abdul
1,2

Latiff MBBS MRehabMed

Department of Rehabilitation Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur;
1

Department of Surgery, Faculty of Medicine & Health Sciences, Universiti Malaysia Sabah, Kota
2

Kinabalu, Sabah, Malaysia

Abstract

Spinocerebellar ataxia (SCA) is a rare neurodegenerative disease with progressive course and poor
expected outcomes. Therefore, rehabilitation remains the principal form of management especially
in advanced disease. Impairment-based rehabilitation through multidisciplinary care approach has
proven benefits for functional improvement in individuals with advancing SCA. This concept is
based on comprehensive assessments of individualised impairments and functional limitations while
exploring contributing environmental and personal factors affecting the person as a whole. From this
assessment, individualised rehabilitation goals can be formulated through a multidisciplinary care
approach. Neurologists, rehabilitation physicians, physiotherapists, occupational therapists and speech
and language pathologists are key individuals involved in the multidisciplinary care for individuals
with SCA rehabilitation. Two cases of individuals at different stages of SCA are presented to highlight
the rehabilitation approach in providing focused interventions based on individualised impairments
through multidisciplinary care. These cases emphasise the importance of understanding the needs
of each individuals with SCA so that the rehabilitative therapies prescribed can be tailored to the
functional achievements desired.

Keywords: Spinocerebellar ataxia, neurorehabilitation, spinocerebellar ataxia rehabilitation, impairment-

based rehabilitation, multidisciplinary care approach

INTRODUCTION Concept of impairment-based rehabilitation

through multidisciplinary care approach
Spinocerebellar ataxia (SCA) is a rare progressive
neurodegenerative disease with heterogenous The lack of evidence and guidelines on
genetic mutations and phenotypes.1 Up to 40 rehabilitative treatment in degenerative ataxia
SCA subtypes have been reported leading to confers further difficulties in formulating a
variable neurological presentations including standard protocol for rehabilitation in SCA. Hence,
incoordination, postural imbalance, unsteady an individualised impairment-based rehabilitation
gait, recurrent falls, oculomotor disturbances and approach could be appropriate.
speech difficulties.2 As curative treatment for SCA Comprehensive assessment on impairments
has yet to be established and the expected outcomes and functional limitations are based on the
are poor with restrictions in functional and social International Classification of Functioning,
activities, rehabilitation remains the mainstay of Disability and Health (ICF) framework, a clinical
management especially in an advancing course of problem-solving tool towards the provision
the disease. Though the primary aim is supportive, of holistic management.3 This framework not
nevertheless, impairment-based rehabilitation only evaluates the functional limitations of
through a multidisciplinary care approach has each individual but also explores contributing
been proven to be beneficial in terms of functional environmental and personal factors. Therefore,
improvement in individuals with advancing SCA. individualised rehabilitation goals can be
formulated through input from a multidisciplinary

Address correspondence to: Fatimah Ahmedy, Department of Surgery, Faculty of Medicine & Health Sciences, Universiti Malaysia Sabah, Jalan UMS,
88400 Kota Kinabalu, Sabah, Malaysia. Tel: +6088 320000 (ext: 611341), +60138805513, Email: fatimahmedy@ums.edu.my

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Neurology Asia March 2020

care team, comprising primarily of neurologists, Sit-to-stand exercises were reinforced on a daily
rehabilitation physicians, physiotherapists (PT), basis in addition to therapeutic flexibility and
occupational therapists (OT) and speech and strengthening exercises of the core trunk muscles
language pathologists.4 (Table 1). To promote the ability for corrective
The progress of an individualised rehabilitation balance from postural sway as a measure of a
programsfor SCA individuals is guided through fall prevention strategy, visual feedback training
outcome measures monitoring; namely Scale using centre of pressure motion signals was used.
of Assessment and Rating of Ataxia (SARA), Gait training was delivered with bodyweight
Berg Balance Scale (BBS), Timed Up and Go supported device on a treadmill. The percentage
(TUG) and Modified Barthel Index (MBI). of supported body weight was gradually reduced
The former two measure body structures and and at the third week of intensive training, over-
function, the latter two measure functional ground gait training was achieved with a walking
domains. Our primary intention of this review frame for minimal distance ambulation. As part
is to highlight impairment-based rehabilitation of environmental adaptation, visual cueing was
through a multidisciplinary care approach,with taught during walking to enhance gait speed and
various instruments adopted throughout two step length. PT delivered this form of training for
different stages of SCA. We emphasise the need an hour a day, for 5 times per week.
to address each patient individually, with the
ultimate goal being to promote a certain degree Assistive and adaptive devices for functional
of self-independence while reducing fall risk and improvement
improving quality of life.
In the compensatory arm, to achieve functional
improvement, the patient was trained by OT to
CASE REPORTS
decompose complex movements into simple
and multiple joint movements. Additional use of
Patient 1
weighted cuffs and largely handled utensils helped
to improve feeding and grooming whereas quad
History and clinical course
cane was used to facilitate safe ambulation and
A 25-year-old lady presented with a history prevent falls. Training on wheelchair propulsion
of progressive worsening of imbalance and was included to enable longer distance mobility
recurrent falls since early 2013 associated with as a measure of energy-conserving techniques.
an abnormal gait. By late 2015, she had to resign The duration of training received was similar to
as a cashier; required walking aids for ambulation the sessions by the PT.
by late 2016, and subsequently was confined to a
wheelchair as her fatigue worsened. Noting that Maintenance exercises for the preservation of
a similar disease progression had affected her communication ability
mother and two siblings, she finally presented
The patient continued to have effective
in early 2017 with head titubation and limbs
communication in addressing her needs. Oral
tremors, dysmetria, nystagmus and dysarthria.
facial muscle training including tongue and jaw
Computed brain tomography showed global
range of motion and strengthening exercises were
cerebellar and brainstem atrophy, with normal
prescribed as part of maintenance exercises to
CSF studies, hormonal studies, connective tissue
delay the deterioration of her speech symptoms
and infective diseases screenings. The diagnosis
(Table 1). The progression of the patient’s
of SCA was made based on the clinical features,
rehabilitation training is summarised in Table 2.
strong family history and exclusion of other
causes. She was then admitted for four weeks of
Patient 2
intensive rehabilitative therapy with the goal of
safe, limited short-distance ambulation within
History and clinical course
the setting of her room to enable her to perform
self-care. A 38-year-old policeman presented with
progressive deterioration in walking function
Balance and coordinative training and recurrent falls since early 2012. He required
support for ambulation by the end of 2015 and
Focused balance training was done in a standing
by mid 2016, the patient started to develop an
position while incorporating functional tasks such
asymmetrical head posture. In early 2017, the
as holding objects and throwing a ball (Table 1).

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limb tremors worsened the balance and he had to Assistive and adaptive devices for functional
crawl to mobilise. With time, long term crawling improvement
led to neuropathic pain at the lower back area that
Compensatory strategies were targeted towards
was made worse in a supine position and affected
dampening upper limbs tremors to improve self-
his sleep quality. Alongside dysarthria, his work
feeding performance and reduce spillage of food.
performance was severely affected. Investigations
He was taught by the OT on simplification of
did not found any metabolic causes or infective
complex movements in adjunct with weighted
pathologies. Magnetic resonance imaging of the
cuffs placed on the proximal arm and distal
brain showed generalised cerebral atrophy. There
forearm incorporated with dual-task hand function
was a similar history in his paternal grandfather
training largely in handling of utensils. As the use
and younger sister. A diagnosis of SCA was made
of walking frame without assistance was deemed
based on the clinical features, family history
unsafe, a home environmental modification
and exclusion of other causes. For management,
which comprised of wall-mounted hand-railings
the patient desired pain control, ability to stand
extending from the living room to the washroom
supported during shower in the toilet, ability
for safer mobility option to promote sideways
for self-feeding and mobile phone usage for
ambulation was recommended. By adopting these
communication purpose. He was admitted for
strategies, the patient reported more satisfaction
four weeks of intensive inpatient rehabilitative
with the performance of ADLs and mobile phone
therapy aiming for these goals.
usage as shown in Table 2. Duration spent with the
OT was approximately the same as with the PT.
Pain management
To manage the low back pain, regular gabapentin Alternative and augmentative communication tool
and topical analgesic ointment were prescribed
The patient produced a considerable amount of
with transcutaneous electrical nerve stimulation
incomprehensible words necessitating multiple
as a physical modality. With pain being managed
repetitions and body gestures to improve
effectively as shown in Table 2, the patient was
communication. Improved hand function made
able to sit and sleep upright without considerable
it possible for mobile texting as a means of
difficulties.
alternative and augmentative communication tool
in delivering better communication.
Balance and coordinative training
Focused balanced training was initiated with DISCUSSION
sit-to-stand exercise followed by pivot transfer
SCA, being a neurodegenerative disorder, limits
to promote chair usage so that patient would no
further restorative or curative rehabilitation
longer need to crawl on the floor. Therapeutic
interventions. A review article on motor training in
exercises were focused at targeted strengthening
degenerative spinocerebellar disease demonstrated
of the core and proximal limb muscles with
3 different approaches for ataxic specific
concurrent flexibility exercises (Table 1). Mat
improvements; physiotherapy combined with
activities for balance training and whole-body
occupational therapy, coordinative physiotherapy
movement exercises were accomplished in
and exergame-based training. Based on these
quadruped standing and kneeling positions to
approaches, a new concept of rehabilitative
improve trunk-limb coordination. Gradually,
training in degenerative cerebellar ataxia is
balance training was upgraded to standing position
to emphasise on tailored training regime by
and at the third week, the training was further
applying various training strategies according
upgraded to side-to-side stepping balance exercise
to individualised needs.5 Physiotherapy (PT)
at the parallel bars. By the end of his training, he
had better trunk control in maintaining an upright combined with the occupational therapy approach
standing position for showering, had a smoother by Miyai et al. provided equivalent training
stand pivot transfer technique and achieved duration and utilised similar outcome measure
independent sideways ambulation approximately as described in these two cases.6 However, their
30 metres distance while holding onto the parallel subjects had additional hour over the weekends
bar, as demonstrated from the outcome measures (either physiotherapy or occupational therapy)
shown in Table 2. The PT was responsible for and a lower baseline score of SARA (mean score
conducting this training for an hour a day during between 11.0 to 12.2). Hence they demonstrated
weekdays. less severe cerebellar impairments that may have

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Table 1: Rehabilitation strategies through multidisciplinary care approach
Rehabilitation Type of exercises and training
strategies Case 1 Case 2
Flexibility exercises Flexibility exercises
Neurology Asia

Spine – extensors and lateral rotators Spine – extensors and lateral rotators
Shoulder – flexors and extensors Shoulder – flexors and extensors
Targetedstrengthening exercises Targeted strengthening exercises
Therapeutic
Core – spine extensors and lateral rotators, abdominal flexors Core – spine extensors and lateral rotators, abdominal flexors
exercises
Upper limb – elbow flexors and extensors Upper limb – elbow flexors and extensors
Lower limb – hip flexors and extensor, knee extensors Lower limb – hip flexors and extensor, knee extensors
Aerobic exercises Aerobic exercises
Static cycling Static cycling
Static balance
Static balance
Quadruped standing
Standing on one leg
Standing on one leg
Dynamic balance Dynamic balance
Balance and
Standing with one foot performing stepping to the front, side and back Quadruped standing
coordination
Visual feedback training with pressure motion signals Kneeling
training
Whole-body movement
Quadruped standing
Whole-body movement
Kneeling
Sit-to-stand exercise
Sit-to-stand exercise
Side-stepping
Transfer training
Mobility Bodyweight supported treadmill training
Stand pivot transfer training – from bed to chair, chair to bed
training Over-the-ground body weight supported gait training
Transfer training from floor to chair, chair to the floor
Joint movement decomposition to simple one joint movement Joint movement decomposition to simple one joint movement
Functional
Adaptive device – weighted cuff, large handle utensils Adaptive device – weighted cuff, large handle utensils
training
Dual task training Dual-task training
Handling objects while standing – holding and throwing ball Handling objects at seated level – feeding, mobile phone usage
Oral facial muscles exercises
Oral facial muscles exercises
March 2020

Communication Tongue – range of motion and strengthening exercises

Tongue – range of motion and strengthening exercises
training Jaw – strengthening exercises
Jaw – strengthening exercises
AAC tool – using a mobile phone
Table 2: Summary of outcome measures
Case 1 Case 2
Outcome
measure 1st week of 4th week of 1st week of 4th week of
rehabilitation rehabilitation rehabilitation rehabilitation
TUG 1 min 36 secs 1 min 36 secs NA NA
NRS NA NA 9 1
BBS 29/56 29/56 4/56 9/56
SARA 16/40 16/40 24.5/40 22/40
MBI 61/100 66/100 77/100 81/100
TUG = Timed Up and Go; NRS = Numerical Rating Scale; BBS = Berg Balance Scale; SARA = Scale for Assessment
and Rating of Ataxia; MBI = Modified Barthel Index; NA = Not applicable

led to higher score changes at the end of the several movement disorders have demonstrated
intervention period. improvements in the ataxic symptoms, walking
Coordinative physiotherapy is a strategy distance, number of tandem steps and standing
that emphasises activating and demanding the capacities for individuals with degenerative
control mechanism for balance control and spinocerebellar ataxia.9 However, such therapies
multi-joint coordination.8 While using similar are infrequently accessible and highly selective,
exercise principles for conditioning, i.e. range implying the need to select the right type of
of motion, strengthening, balance and mobility individuals for such an expensive treatment mode.
training, this particular approach studied by Ilg Better still, the exponential growth in numbers
et al. highlighted the importance of selecting of devices for promoting self-independency has
compensatory feedback, either in the form of been intensively explored among individuals
somatosensory, visual or vestibular inputs to with neurological disorders, including spinal cord
prevent falls.8 In addition to utilising SARA injury and stroke. However, such applications have
outcome measure as a monitoring tool, they not been widely researched in SCA, possibly due
used the goal attainment score to determine the to its progressive nature requiring frequent change
relevance of this intervention on daily life. The of devices throughout the disease course.
latter assessment would undoubtedly demonstrate In conclusion, formulating rehabilitation
improvement in these two described cases, since goals for individuals with SCA needs to be
both achieved the functional goals despite pre individualised. Impairment-based rehabilitation
and post-intervention SARA scores that were through multidisciplinary care approach is
relatively unchanged or minimally changed. beneficial for functional improvement. Adopting
These two approaches were concurrently mixed rehabilitative strategies with the use of
applied on these two cases based on a standardised appropriate monitoring instruments at different
rehabilitative intervention framework that stages of SCA will lead to achievement of the
focused on five domains; therapeutic exercises, desired goals.
balance and coordination training, mobility
training, functional training and communication DISCLOSURE
training (Table 1). The prescribed interventions
Financial support: None
were tallied with the individual’s impairments,
through multidisciplinary care in accordance to
Conflict of interest: None
the principles of the ICF model for establishing
individualised goals.

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