Physiotherapy Theory and Practice

An International Journal of Physical Therapy

ISSN: 0959-3985 (Print) 1532-5040 (Online) Journal homepage: https://www.tandfonline.com/loi/iptp20

Miller Fisher syndrome and Guillain-Barré

syndrome: dual intervention rehabilitation of a
complex patient case

Jill E. Mayer, Christine A. McNamara & John Mayer

To cite this article: Jill E. Mayer, Christine A. McNamara & John Mayer (2020): Miller Fisher
syndrome and Guillain-Barré syndrome: dual intervention rehabilitation of a complex patient case,
Physiotherapy Theory and Practice, DOI: 10.1080/09593985.2020.1736221

To link to this article: https://doi.org/10.1080/09593985.2020.1736221

Published online: 09 Mar 2020.

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PHYSIOTHERAPY THEORY AND PRACTICE
https://doi.org/10.1080/09593985.2020.1736221

Miller Fisher syndrome and Guillain-Barré syndrome: dual intervention

rehabilitation of a complex patient case
Jill E. Mayer, PT, DPT, NCSa, Christine A. McNamara, PT, DPTa, and John Mayer, PT, DPT, NCSb
a
Department of Physical Therapy, Ithaca College, Ithaca, NY, USA; bInpatient Therapy Department, Cayuga Medical Center, Ithaca, NY, USA

ABSTRACT ARTICLE HISTORY

Purpose: Guillain-Barré syndrome (GBS) presents with acute peripheral neuropathy leading to Received 19 February 2019
ascending motor and sensory deficits. Miller Fisher syndrome (MFS), a GBS variant, is characterized Revised 4 November 2019
by ophthalmoplegia, ataxia, and areflexia. In unusual cases, MFS and GBS overlap. The purpose of Accepted 23 January 2020
this case report is to illustrate the effects of an aquatic and land-based physiotherapy (PT) KEYWORDS
intervention on a patient with MFS-GBS. Miller Fisher Syndrome;
Case Description: A 57-year-old male physician was diagnosed with complex regional pain guillain-Barré Syndrome;
syndrome following a quadriceps muscle tear. Within 1 month, the patient experienced evolving aquatic therapy; ataxia:
motor, sensory, autonomic, and cranial nerve dysfunction and was diagnosed with MFS-GBS. rehabilitation
Interventions: Five months post-onset, a 7-week intensive PT program was initiated including
aquatic and land-based interventions.
Outcomes: Following completion, functional improvements were demonstrated on the 6 Minute
Walk Test, Timed-Up-and-Go, 10 Meter Walk Test and Short Form-36. However, 6 weeks after
program completion, the patient had a recurrence.
Conclusion: PT intervention demonstrated improvement in functional outcomes for a patient
with a diagnosis of MFS-GBS. Complex patients lacking recovery within 6 months may benefit
from continued rehabilitation. Other intervention approaches may need to be considered, includ-
ing aquatic therapy.

Introduction Ishii et al, 2016). See Table 1 for an outline of the

Guillain-Barré syndrome (GBS) is an autoimmune dis-
ease presenting with acute onset idiopathic peripheral
neuropathy which leads to rapid progression of ascend-
ing motor and sensory deficits with frequently asso-
ciated autonomic dysfunction, cranial nerve (CN)
involvement, and respiratory failure (Hughes and
Rees, 1997; Ropper, 1992; van der Meché et al, 1997).
Miller Fisher syndrome (MFS) is a rare variant of GBS
occurring in approximately one in 1 million or about
5% of persons with GBS (Fisher, 1956; McGrogan,
Madle, Seaman, and de Vries, 2009). Clinical presenta-
tion of MFS is characterized by the presence of
ophthalmoplegia, ataxia, and areflexia with minimal to
no weakness (Fisher, 1956). However, additional symp-
tom presentation may include generalized muscle
weakness, CN involvement, and respiratory failure
(Berlit and Rakicky, 1992; National Institute of
Neurological Disorders and Stroke, 2019b). In rare
cases, MFS and GBS may overlap, reported as 5%-
28% of cases, and indicated by paresis of the extremities
or definite limb weakness (Berlit and Rakicky, 1992;
typical clinical presentation for each diagnosis.
Classification of GBS variants continues to be clinically
challenging and is thought to be on a continuum rather
than discrete disease presentation (Ito et al, 2008;
Wakerley, Uncini, and Yuki, 2014). Although prognosis
is typically favorable, this differentiation among variants
makes it difficult to determine the optimal treatment
protocol. Thus, MFS is treated similarly to GBS, despite
variations in signs and symptoms (Aranyi, Kovacs, Sipos,
and Bereczki, 2012; National Institute of Neurological
Disorders and Stroke, 2019a, 2019b). Intravenous admin-
istration of immunoglobulin (IVIg) or plasmapheresis has
been documented to promote recovery (Aranyi, Kovacs,
Sipos, and Bereczki, 2012; Overell et al, 2007). However,
contradicting research reports similar outcomes with no
treatment (Teener, 2012).
Past research supports early and intensive rehabilita-
tion for patients with GBS to promote functional recov-
ery (Ensrud and Krivickas, 2001; Khan and Amatya,
2012; Ko, Ha, and Kang, 2017; Meythaler, 1997;
Williams and Middleton, 2018). Intensive exercise, in
the inpatient setting, focused on activities of daily living

CONTACT Jill E. Mayer jmayer@ithaca.edu Department of Physical Therapy, Ithaca College, 953 Danby Rd, Ithaca, NY 14850
© 2020 Taylor & Francis Group, LLC
2 J. E. MAYER ET AL.
Table 1. Clinical presentation associated with GBS and MFS
diagnosis.
Clinical
presentation Other strong indicators
Classic Ophthalmoplegia Minimal or Absent limb weakness
MFS Ataxia Presence of IgG antibodies against
ganglioside GQ1b (anti-GQ1b)
Areflexia
Classic Four limb Rapid, symmetrical symptoms
GBS involvement
Areflexia Sensory, cranial nerve and autonomic
dysfunction
Elevated protein in Cerebrospinal Fluid
(ADL), strengthening, endurance training, and gait
training have proven effective to improve functional
independence. Given the favorable prognosis, less
information is available on the impact of rehabilitation
in the chronic phases of the disease, however, evidence
promotes continued high intensity and progressive
strength, endurance, and functional skill training
(Arsenault et al, 2016; Khan and Amatya, 2012; Khan
et al, 2011). This is suggested to be true for cases of
MFS, however, minimal to no evidence exists on the
impact of rehab interventions for this distinct variant.
A case report by Waheed, Hussain, and Shehzadi
(2018) reported the involvement of a physiotherapist
in the care of one patient with MFS during an acute
care stay while simultaneously receiving plasmapher-
esis. However, no further information was provided on
the frequency, type of intervention or objective func-
tional progress of this patient. The focus of this case
report is to describe the effects of physiotherapy (PT)
intervention, consisting of land-based and aquatic PT
for a patient with a diagnosis of MFS-GBS in an out-
patient setting.
Case description
Written patient consent was received by the authors to
present his case and findings. The patient was a 57-year-
old male, 1.98-m tall and approximately 105 kg in good
physical health who exercised 5 days per week. He was
married, had 3 children in college and worked full time as
an emergency room physician. Past medical history was
significant for migraines, borderline hypertension, shin-
gles, and Gilbert’s syndrome. He sustained a traumatic left
quadriceps tear from a fall and subsequently developed
complex regional pain syndrome (CRPS). Over the next
5 weeks, he experienced evolving sensory, motor and CN
dysfunction with reports of ataxia, difficulty swallowing,
repeated falls, diplopia, areflexia, and respiratory distress.
The rapid symptom progression, along with abnormal
nerve conduction studies, raised concern for possible
demyelination and required emergent hospital admission.
Subsequently, he was diagnosed with a complex presenta-
tion consisting of GBS with overlapping MFS, mild trau-
matic brain injury (TBI) and CRPS. Testing was not
performed for the presence of anti-ganglioside antibodies
which may be present in up to 58% of patients with MFS
(Kaida et al, 2006). Following diagnosis, he underwent
a 5-day course of IVIg treatment with minimal improve-
ment in respiratory function and muscle strength. See
Table 2 for a detailed timeline of inpatient medical events.
Following acute medical stabilization and short inpa-
tient rehabilitation due to financial limitations, the
patient was discharged home approximately 5 weeks
from initial hospitalization. He continued to require
assistance for transfers, ADLs and ambulation while
predominantly utilizing a manual wheelchair for mobi-
lity. Outpatient rehabilitative interventions were
delayed for 3 months due to inadequate insurance
coverage despite persistent and extensive functional
limitations. The patient eventually experienced mild
improvements in mobility and presented to initial eva-
luation, 5 months post-onset of symptoms.
Clinical findings
The International Classification of Functioning was
used to classify the patient’s health-related presenta-
tion (World Health Organization, 2018). Upon phy-
sical examination, he presented with numerous
impairments. Most significant impairments included
LE strength deficits with greater distal muscle invol-
vement than proximal, 6–9/10 pain in the left knee,
impaired proprioception below bilateral knees with
absent deep tendon reflexes, extensive CN involve-
ment, and severe truncal ataxia resulting in poor
standing balance. Abnormal findings were seen on
the following CN tests: ocular muscle impairments
(CN II, III, IV, VI), muscles of facial expression with
right anterior tongue sensation loss (CN VII),
impaired vestibulo-ocular reflex (CN VIII), glosso-
pharyngeal, and vagal nerve with esophageal/palate
partial paralysis (CN IX, X). Dysautonomia was
another concern with blood pressure fluctuations
causing headaches and diaphoresis when elevated as
well as syncope during episodes of hypotension.
Despite being alert and oriented, he reported mild
cognitive system impairments including memory and
executive function particularly related to his work as
a physician, secondary to mild TBI.
Functional assessment revealed activity limitations with
regards to transfers, ambulation, and elevations. He
required varying levels of assistance from close supervision
to minimal assistance and UE support for transfers based
on surface height, fatigue, knee pain, and availability of
 PHYSIOTHERAPY THEORY AND PRACTICE 3

Table 2. Timeline of events from chart review.

Days Weeks Event
0 1 Sustained fall, L vastuslateralis and intermedius partial muscle tear, patellar subluxation
2 ER visit due to increased pain; provided knee immobilizer, cane and referral to PT
17 2 Outpatient PT initiated, developed L LE sweating, neuralgia, L LE dysesthesias, diagnosed with complex regional pain syndrome
(CRPS)
20 Patient notes difficulty swallowing, ataxia develops, multiple falls (4+)
23 3 ER visit post- serious fall with head trauma, brief LOC, CT (-), sustained concussion
26 4 Neurologist evaluation for reported R LE dysesthesias, NCS (+) slowing velocity, diminished/abnormal amplitude, concerning for
demyelinating process
37 5 Progressive ascending B dysesthesias up to groin with continued falls, difficulty swallowing, autonomic dysfunction, dyspnea on
exertion
39 Admitted to ICU with worsening symptoms, NCS (+) slowing velocity UE/LE and diminished amplitude consistent with
demyelination; spinal tap (+) increased protein; diplopia with R lateral gaze only, dysphagia: diagnosed with GBS with variant of
MFS, started on 5-day course IVIg
40 Diplopia with lateral gaze bilaterally, worsening cardiovascular and respiratory status requiring supplemental O2 up to 4 liters,
refused endotracheal intubation despite recommendations, ECG (+) L bundle branch block/AV block, transthoracic ECHO (+), EF
40-45%; brain MRI (+) L frontal area involvement attributed to repeated falls, chest CTA (-), head CT (-), spine MRI (-), HIV (-),
Lyme (-),
43 6 Final dose of IVIg; respiratory improvement noted, though continued autonomic instability and cardiac concerns; started use of
eye patch for diplopia; B LE doppler (-), decreased sensation (R: T9-S2, L: T10-S2, patchy throughout B UEs), motor weakness (R
UE 5/5; L UE 4/5; R LE hip flexion 2/5, knee extension 4/5, dorsiflexion 4/5, EHL 4,/5 plantar flexion 4+/5; L LE EHL trace,
dorsiflexion trace, plantar flexion 4/5); absent DTR B LE, intact DTR B UE
44 Improved symptoms, increased functional strength, initiated stand pivot transfer with two person assist, improved respiratory
status though dyspnea on exertion
46 7 Transferred to major acute care hospital as per pt request with expanded services and larger rehabilitation unit; affirmed
diagnosis of GBS with MFS
47–61 9 Continued stabilization of cardiopulmonary systems, initiated inpatient rehabilitation, truncal ataxia identified, neuropsychiatric
testing (+) impairment of executive reasoning secondary to TBI
72 10 Discharge home: continued sensory/motor neuropathy, ataxia, diplopia, dysphagia, dysautonomia, neuralgias, areflexia;
functional status- limited ambulation with minimal assist, transfers with supervision, wheelchair mobility independent
72–185 11–27 Self-directed HEP, no outpatient services, limited to no insurance coverage, slow improvement to mod I transfers, ambulating
household distances with FWW, status monitored by monthly appointment with internist and every 2 months with neurologist
185 27 Start of Outpatient PT, Initial evaluation, aquatic and land-based interventions 4 times per week, 60 min sessions
235 34 Discharged from PT, final evaluation
280 40 New onset of worsening symptoms, signs of recurrence including progressive dizziness, diplopia, ataxia and generalized
weakness; attempt to initiate PT at that time discontinued
294 42 Diagnosed with recurrent GBS
Abbreviations: L, left; R, right; LE, lower extremity; UE, upper extremity; LOC, loss of consciousness; CT, computed tomography; NCV, nerve conduction studies;
hx, history; B, bilateral; ICU, intensive care unit; HIV, human immunodeficiency virus; AV, atrioventricular; ECG, electrocardiogram; ECHO, echocardiogram;
EF, ejection fraction; HEP, home exercise program; mod I, modified independent.

armrests. He was independent of household ambulation of
15 m with use of a front-wheeled walker (FWW); however,
reported falls multiple times per week while ambulating
and negotiating stairs while his wife was at work. He
required close supervision for community ambulation up
to 50 m and contact guard to minimal assistance for stair
negotiation with use of bilateral handrails. The patient was
independent for sitting balance however demonstrated sig-
nificant limitations in standing balance secondary to trun-
cal ataxia requiring maximal to total assistance without UE
support within 3 s. He demonstrated signs of impaired
judgment during functional tasks with noted impulsivity
and limited awareness of fatigue. Given the extensive
impairments and functional limitations, he was unable to
work, exercise, or fulfill household responsibilities as
a husband and father.
Diagnostic assessment
Minimal outcome measures have been validated in the
GBS population (Umphred, Lazaro, Roller, and Burton,
2013). Widely used outcome measures in neurologic
populations were selected to measure changes in activ-
ity limitations (Moore et al, 2018). The following out-
come measures were completed to assess the patient’s
balance, gait, functional mobility, and quality of life
(QOL): Berg Balance Scale (BBS); 6 -minute walk test
(6MWT); 10-meter walk test (10MWT); Timed-Up-and
-Go (TUG); and the Short Form-36 (SF-36). See Table
3 for details.
The prognosis for the patient and expected out-
comes were difficult to predict due to the complexity
of the diagnosis including GBS-MFS, quadriceps tear
with resultant CRPS, and mild TBI from repeated falls.
Typical prognosis for persons with GBS and MFS is
favorable overall, ranging from weeks to several years
with the majority of patients ambulatory and near-
complete recovery by 6 months from onset of symp-
toms (Khan and Amatya, 2012; National Institute of
Neurological Disorders and Stroke, 2019a, 2019b). It
has been reported that approximately 20% of patients
with GBS continue to have functional limitations 1 year
later and 33% of patients with MFS report residual
symptoms of ataxia, diplopia, or hyporeflexia which
4 J. E. MAYER ET AL.

Table 3. Initial and final evaluation outcome measure results. effectiveness (Khan and Amatya, 2012; Williams and
Initial Middleton, 2018). His overall prognosis was predicted
evaluation Final evaluation
(23 wks after (30 wks after as fair to good given his former active lifestyle, knowl-
Outcome measure onset) onset) edge of medical care, motivation, and evidence favoring
BERG balance Test 7/56 7/56 early recovery. However, chronic deficits and multi-
6 Minute Walk Test 129.2 m 177.1 ma
10 Meter Walk Test system involvement were considered significant bar-
Self-Selected velocity 0.51 m/s 0.56 m/s riers to achieving full rehabilitation potential.
Fast Velocity 0.74 m/s 0.95 m/sc
Timed Up and Go 48.21 s 21.4 sb Goals were discussed with the patient who
Short Form 36 expressed he wanted to return to work, resume reg-
Physical function 5% 10%
Role limitations due to physical 0% 25% ular exercise, and achieve independence with func-
health tional mobility as his wife was unable to assist due to
Role limitations due to emotional 0% 100%
problems work obligations. The plan of care further empha-
Energy/fatigue 15% 20% sized education on safety and reduction in falls.
Emotional well-being 48% 88%
Social functioning 25% 37.5% Short-term goals (STG) were predicted at 3 weeks
Pain 0% 0% and long-term goals (LTG) were set at the end of
General health 45% 35%
a the 7-week intervention (Table 4).
Suggestive of a clinically important difference (Tang, Eng and Rand, 2012).
b
Smallest real difference (Flansbjer, Holmback, Downhal, Patten, and Lexell,
2005).
c
Substantial meaningful change (Perera, Mody, Woodman and Studenski,
2006).
Therapeutic intervention
PT sessions took place in an on-campus free clinic and
are usually mild and did not interfere with ADLs were scheduled based on the academic calendar for
(Meythaler, DeVivo, and Braswell, 1997; Osterhues, a 7-week period. The treatment schedule was 4 days
2004; Winer, Hughes, and Osmond, 1988). per week for 60 min each, with conventional land-
Upon initiation of outpatient treatment, however, based sessions on Monday/Thursday, and aquatic ses-
this patient was 5 months post-initial onset of symp- sions on Tuesday/Friday. Two licensed physical thera-
toms with persistent functional deficits and frequent pists were the primary care providers; one on land, and
falls. The patient reported refusal of endotracheal intu- one in the aquatic setting. Two students assisted in
bation upon acute care admission, presented with CN sessions primarily as an educational opportunity to
involvement and autonomic dysfunction; all negative learn about a unique diagnosis. The students’ role con-
prognostic indicators signifying longer lengths of hos- sisted of guiding interventions that were outlined by the
pital stay in classic GBS (Meythaler, 1997). Limited primary therapists, guarding, and handling equipment
information is available on prognostic factors specifi- needs, however skilled hands-on facilitation was pri-
cally for MFS. marily provided by the licensed therapist.
Other considerations leading to a poorer prognosis The frequency of intervention was selected given the
include shortened multidisciplinary inpatient rehabili- lapse in medical care following hospital discharge and
tation and further lapse in PT intervention following evidence supporting the importance of high-intensity
discharge, despite strong evidence to support its intervention (Khan et al, 2011). This setting permitted
the patient to receive needed care without financial
burden or insurance limitations.
Table 4. Short-term and long-term goals for physical therapy.
Evidence-based interventions were implemented
Short-term In 3 weeks the patient will: with an emphasis on addressing impairments and
goals activity limitations found in the examination.
(1) Independently perform transfers with FWW.
(2) Tolerate static standing without UE support for
Principles of experience-dependent neural plasticity
10 sec with close supervision. were utilized including concepts of task specificity,
(3) Independently ambulate community distances of 150 repetition, transference of skill, and providing suffi-
feet or greater with FWW.
Long-term In 7 weeks the patient will: cient intensity (Kleim and Jones, 2008). This included
goals balance training to reduce fall risk, transfers and gait
(1) Independently ambulate in his household with the
least restrictive device. training, and improving motor control and stability
(2) Safely and independently negotiate three 6 inch during standing movements in both environments
stairs with unilateral handrail.
(3) Independently and safely perform sit to stand trans-
(Khan and Amatya, 2012). Table 5 presents a typical
fers without UE assistance. session for each environment.
 PHYSIOTHERAPY THEORY AND PRACTICE 5

Table 5. Sample land-based/aquatic interventions and home exercise program.

Land 60 min Transfer training, postural control and gait training
● Aerobic warm up: recumbent stepper for 5 min
● LiteGait® body-weight support system incorporated for improved stability and safety during standing tasks ambulation
● Exercises included static standing with head turns, weight-shifting, partial sit to stand, forward stepping, ambulating
4 × 15 m.
● Overground walking with rolling walker and manual cues for technique: minimal assistance provided for gait technique.
● Balance:
○ Repetition of limb movements with gradual reduction of external supports in weight-bearing positions (hip flexion, hip
abduction, UE diagonal reaching)
○ Proximal and distal fixation techniques to manage ataxia (UE support on counter, or trunk or pelvic support on wall or
counter).
● Transfers: Repetitive sit to stand transfer training from elevated surface with rolling walker

Aquatic 60 min Postural control, gait, strength and endurance training in shallow and deep water
● Aerobic warm-up: 5 min guided walking in shallow end at T10 level
● Postural Control, righting reactions, body awareness (deep water)
○ Repetitive (B) LE limb movements while maintaining upright posture (Hip/knee flexion to extension; hip abduction/
adduction)
○ Flotation devices utilized to decrease ataxia

● Gait training (shallow and deep water)

○ Forward, backward, lateral walking
○ Gradual reduction in use of flotation device
● LE and general endurance and strength exercises (shallow and deep water)
○ LE kicking, UE abduction/adduction, swimming with pelvic support
○ Squats with UE support on side of pool or flotation device
Home Exercise Program
• Static standing balance;
• At kitchen counter with pelvis supported and reducing UE support;
• Core and LE strengthening;
• Anterior and posterior trunk motions in sitting;
• Mini-squats in standing with UE support;
• Ocular muscle strengthening exercises;
• Visual tracking while keeping his head stabilized.

Land-based intervention support and cues for safety. Repetition, performing

Land-based sessions utilized proprioceptive feedback
consisting of distal fixation and trunk wrapping with
an ace wrap, similarly to an abdominal binder, was
utilized during interventions in an effort to minimize
truncal ataxia, a primary limiting factor on examination
for the maintenance of standing balance. Research has
supported the effectiveness of trunk wraps in patients
with cerebellar ataxia (Marsden and Harris, 2011;
Umphred, Lazaro, Roller, and Burton, 2013). Positions
focusing on joint approximation and facilitating the
timing of appropriate muscle activation, consistent
with aspects of the neurodevelopmental treatment
approach, were incorporated (Diaz-Arribas, Martin-
Casas, Cano-de-la-cuerda, and Plaza-Manzano, 2019).
Standing balance was challenged by gradually removing
UE and external supports as long as trunk excursions
were minimal and postural control was maintained.
Quadruped and tall kneel exercises with UE support
were trialed, however, limited by knee pain (Umphred,
Lazaro, Roller, and Burton, 2013). Floor and stand
pivot transfers were practiced with reliance on UE
movements slowly, and using a mirror or external
cues for visual feedback were emphasized across all
interventions as these strategies have proven beneficial
in patients with ataxia (Marsden and Harris, 2011).
A body-weight support (BWS) system was also
incorporated into sessions for functional over-ground
training of balance, transfers, and ambulation to
increase challenge while maintaining safety. In a case
report by Tuckey and Greenwood (2004), a BWS sys-
tem was utilized over the course of a 4-month stay in
an inpatient setting with a patient 6 months post-GBS
diagnosis. The system was used to increase standing
tolerance and ambulation capacity both on and off the
treadmill with functional improvement reported.
Ambulation trials were also performed outside of the
harness system with use of an FWW for distances
ranging between 10 and 50 meters. Intermittent facil-
itation at the pelvis and LEs was provided to improve
gait pattern. The patient wore a right LE ankle-foot
orthosis to accommodate for the lack of dorsiflexion
strength in 50% of sessions. Kinesio Taping® was
applied at the left knee to promote neutral patellar
6 J. E. MAYER ET AL.
tracking secondary to the quadriceps injury as this has
been shown to reduce pain (Osterhues, 2004). An eye
patch was worn to accommodate for diplopia. Trunk
wraps, as described previously, were also utilized dur-
ing ambulation and LE wrapping for flexion during
swing phase was used to improve proprioceptive input
in the LEs; all suggested strategies to implement in
patients with ataxia, and ocular and proprioceptive
deficits (Marsden and Harris, 2011; Umphred, Lazaro,
Roller, and Burton, 2013).
Aquatic-based interventions
Aquatic sessions focused on postural control, gait train-
ing, functional strengthening, and endurance with shal-
low and deep water interventions. Given water’s
properties of hydrostatic pressure, viscosity, and hydro-
mechanics, this setting permitted a safe environment to
work within while simultaneously providing a level of
external feedback and resistance for the patient to move
within (Kisner, Colby, and Borstad, 2018).
Deep-water exercises focused on improving postural
control, an abnormal examination finding limited by
ataxia. Due to buoyancy, these exercises required the
use of external flotation by the patient, such as
a kickboard and noodles, and initially required thera-
pist assistance at the patient’s trunk. Over the course of
the 7 weeks, therapist assistance was gradually reduced
with the patient achieving an upright standing position
with use of external flotation only.
Balance was also a focus in shallow water at a height of
T4-T10 spinal levels with an emphasis on reducing external
supports from flotation devices or therapist. Furthermore,
in the water, the patient was able to move his LEs with
a reduction in pain, a limiting factor for progression, allow-
ing improved ROM (Kisner, Colby, and Borstad, 2018).
Given the reduction in joint compression, resistance in all
directions, and dampening of perceived LE knee pain, the
aquatic environment was ideal for implementing strength-
ening exercises. This included squats, marching, and hip
abduction exercises. Interventions were then progressed to
land, based on patient tolerance.
Gait training was also emphasized in shallow water,
permitting adequate joint loading for movement control
while simultaneously providing external pressure with an
emphasis on improving proprioceptive input. The resis-
tance of the water served as a stabilizing force to the
patient, permitting a decrease in support by the therapist,
particularly in chest height water. Subjective reports by
the patient noted he felt safer and more stable in the
water. To the author’s knowledge, no studies have exam-
ined the effects of aquatic therapy in patients with GBS or
MFS, however, improvements have been seen in other
neurologic populations. Salem et al. (2011) found that
patients with multiple sclerosis (MS) who participated in
2 days per week of comparable exercises to this report
(i.e., walking, strengthening, and balance training) saw
improvements in BBS scores, gait speed, and TUG
(Salem et al, 2011).
Both land-based and aquatic PT sessions were gra-
dually progressed in regards to increasing distance
walked, increasing repetitions, or reducing external
supports as tolerated by the patient. Intervention
bouts were modified or discontinued based on indi-
cators that the patient had reached his optimal per-
formance for a given exercise. These indicators
included a sudden rise in BP, and signs of fatigue
such as shortness of breath, diaphoresis, or failure to
maintain optimal kinematics. Attention was paid to
avoid over-fatigue of muscles as this has been sug-
gested as a possible impediment to recovery in
patients with GBS (Meythaler, 1997). Patient educa-
tion on safety with functional mobility was incorpo-
rated into every session. A land-based home exercise
program was provided to enhance carryover of treat-
ment (Table 5).
Outcomes
The patient attended a total of 19 one-hour sessions over
the course of 7 weeks including initial and final evalua-
tions. Following 7 weeks of intervention, the patient
demonstrated improvements on the following outcome
measures: 10MWT (fast velocity); 6MWT; TUG; and SF-
36 (all sub-scales excluding general health and pain). He
did not demonstrate change on the BBS. Minimal detect-
able change (MDC) and minimally clinical important
differences (MCID) have not been established for GBS
or MFS populations. MDCs and MCIDs formerly deter-
mined in persons with stroke were utilized for gait mea-
sures to help determine extent of recovery (Flansbjer et al,
2005; Perera, Mody, Woodman, and Studenski, 2006;
Tang, Eng, and Rand, 2012). A more in-depth assessment
of the patient’s score on the outcome measures is pre-
sented in Table 3.
Results indicated that the patient achieved all STG,
but did not achieve LTG (Table 4). He was performing
transfers and ambulation up to 61 m independently
with an FWW. He was able to maintain static standing
balance without support for 11 s prior to loss of balance
due to ataxia. Stair negotiation was not a focus of
intervention due to increased left knee pain with this
activity. The patient demonstrated improved activity
tolerance with decreased episodes of dysautonomia.
Complications affecting the patient’s therapy sessions
included a fall at home resulting in a minor wrist injury

and hypertensive bouts during therapy sessions as high as
210/115 mmHg requiring 5–15 min of rest to stabilize to
within physician-approved range. The patient continued
to demonstrate elevated BP with increased exercise inten-
sities (rating of perceived exertion between 7–9/10) and
pain reported as 8/10.
A follow-up phone call was made at 4 and 6 weeks
post-discharge. The 4-week phone call revealed
a carryover of therapeutic gains as well as continual
functional recovery with intent on resuming care in the
coming weeks. At 6 weeks, 8.5 months since initial
symptom onset, the patient reported signs of relapse
resulting in worsening ataxia, diplopia, pain, and gen-
eral weakness. Recurrent GBS was confirmed by the
patient’s neurologist who reported onset may have
been related to a recent immunization, thus preventing
the continuation of PT.
Discussion
From the authors’ knowledge, no research exists to
guide PT interventions for patients with MFS specifi-
cally, thus typical rehabilitation utilizes what has been
implemented in the GBS population. The patient’s
recovery in this case report was further complicated
by a rare symptom presentation and an atypical
mechanism of injury that has not been previously iden-
tified in the literature. Typical precedent events in
persons with MFS include infections, particularly
respiratory (83%) and gastroenteritis (6.9%) (Mori,
Kuwabara, and Yuki, 2012). Gastroenteritis is a more
common precedent event in GBS (32%) and more
recently cases of GBS have been found following bar-
iatric surgery due to possible nutritional deficiencies
(Jacobs et al, 1998; Sunbol et al, 2018). The mechanism
of injury in this case was a quadriceps muscle tear
which triggered CRPS. The diagnosis of CRPS occurred
immediately prior to the development of patient ataxia,
difficulty swallowing, and multiple falls. In rare cases,
stressful events may lead to the development of GBS
(van Doorn, Ruts, and Jacobs, 2008). However, current
literature does not correlate CRPS as a preceding event.
Evidence on recovery of patients with GBS is more
heavily focused in the inpatient setting, however, a few
studies have demonstrated significant improvements in
function with outpatient care (Khan and Amatya, 2012).
In a randomized controlled trial by Khan et al. (2011)
a multidisciplinary, individualized care plan was imple-
mented in patients with chronic GBS 3 days a week for
one-hour sessions over the course of 12 weeks. Emphasis
was placed on strengthening, endurance, and gait train-
ing as well as everyday functional tasks and psychologi-
cal counseling. Compared to a less intensive home
PHYSIOTHERAPY THEORY AND PRACTICE 7
exercise control group, significant improvements in
function were demonstrated. Further studies support
the use of cycling and walking as well as individualized
PT programs to improve physical functioning, cardio-
pulmonary function, increased strength, QOL, and
reduce fatigue. However, confident conclusions are dif-
ficult to derive given many low-quality study designs
(Arsenault et al, 2016; Khan et al, 2011).
The dual intervention approach was selected as both
environments have demonstrated functional improve-
ments in neurologic populations, with similar benefits
found in comparative studies of both settings
(Marinho-Buzelli, Bonnyman, and Verrier, 2015).
Although evidence is not known as to the effects of
aquatic exercise on patients with GBS, it has been
shown to demonstrate improvements in gait speed
and kinematics, postural control, and LE strengthening
in patients with stroke, MS, and other neurologic con-
ditions (Marinho-Buzelli, Bonnyman, and Verrier,
2015; Morris, 2010; Noh, Lim, Shin, and Paik, 2008;
Salem et al, 2011). Zivi et al. (2018) looked at the effects
of aquatic versus land-based therapy in patients with
peripheral neuropathies and found each setting simi-
larly beneficial in improving gait and balance outcomes.
The aquatic environment has also been considered
a beneficial and safe initial setting for weaker patients
similar to those with demyelinating conditions (Ensrud
and Krivickas, 2001). The frequency of this case report
protocol (2 days/week for 60 min) is consistent with
other aquatic therapy research, however, the duration
varies between studies (Marinho-Buzelli, Bonnyman,
and Verrier, 2015; Salem et al, 2011).
The impact of aquatic therapy in reducing or
controlling for truncal ataxia has not been studied.
However, given the properties of water, particularly
hydrostatic pressure and viscosity, this environment
may be beneficial in improving functional outcomes
in the presence of ataxia (Becker, 2009). Outcome
measures that measure ataxia and consider its effects
on function and balance could have been included.
The Scale for the Assessment and Rating of Ataxia is
a valid and reliable measure of ataxia (Schmitz-
Hübsch et al, 2006). In addition, the International
Cooperative Ataxia Rating Scale quantifies the impact
of ataxia in functional activities such as walking and
standing (Schmitz-Hübsch et al, 2006; Trouillas et al,
1997). Utilizing these measures would have likely
been more appropriate than the BBS to measure
potential changes in postural control. More clinical
trials are needed to determine if the combination of
interventions can demonstrate similar or even greater
effectiveness in patients with complex symptom
presentations.
8 J. E. MAYER ET AL.
Although positive outcomes have been demonstrated
with rehabilitation in the GBS population, MFS-GBS
presents very clinically different and includes truncal
ataxia and ocular motor deficits. The use of a BWS
system may be beneficial to improve the safety and
independence of functional tasks in patients with
ataxia. A systematic review by Marquer, Barbieri, and
Pérennou (2014) found that BWS gait training may
demonstrate improvement in walking abilities in per-
sons with ataxia over-ground and on a treadmill, how-
ever, this was only found in two case studies where the
diagnosis was stroke and brain injury, respectively.
Further evidence on the use of BWS systems in over-
ground and treadmill settings is warranted in the MFS,
GBS, and ataxic populations as it has shown positive
outcomes in patients with other neurologic conditions
(Leech et al, 2016).
Current evidence in the outpatient setting supports
supervised exercise in persons with GBS for 3,
1-h sessions per week for 12 weeks (Arsenault et al,
2016). This case report included 4, 1-h sessions per
week, however, given the complexity of this case, it
likely would have been beneficial to increase the dura-
tion of care to achieve LTGs. According to the 2019
Medicare standards, these therapy parameters are per-
mitted if deemed medically necessary (American
Physical Therapy Association, 2019). Unfortunately,
given the age of this patient and time of initial injury,
he did not qualify for Medicare and had a health
insurance plan with restricted and expensive inpatient
and outpatient coverage. Barriers that impede access
to adequate care can include lack of health insurance,
high co-payments, and limited transportation, thus
the free campus clinic was ideal for this patient. In
a survey study completed by Davidson, Wilson,
Walton, and Brissenden (2009) 25% of patients with
GBS reported not receiving PT intervention
following hospital discharge despite relatively high
levels of disability. Further research is needed to sup-
port high-intensity long duration rehabilitation in
complex diagnoses without financial barriers.
This clinical case report illustrates the impact PT
interventions may have on promoting functional recov-
ery in persons with MFS-GBS. It highlights the magni-
tude and chronicity of symptoms which can complicate
a traditional plan of care, warranting more research on
the effects of novel interventions in promoting recovery.
Disclosure of interest
The authors explicitly state that there is no conflict of interest
relevant to any of this work.
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