1. health and fitness
2. disease

Physical Therapy for a Patient Through Six Stages of
Amyotrophic Lateral Sclerosis
Vanina Dal Bello-Haas, Anne D Kloos and Hiroshi
Mitsumoto
PHYS THER. 1998; 78:1312-1324.
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Physical Therapy for a Patient
Through Six Stages of Amyotrophic
Lateral Sclerosis
Background and Purpose. This case report describes the use of Sinaki
and Mulder's approach to staging amyotrophic lateral sclerosis (ALS)
and functional outcome measures in designing a treatment program
for a 59-year-old woman with ALS. Case Description and Outcomes. As
the patient progressed from stage I through stage VI, over 12 months,
the physical therapy goals changed from optimizing remaining function, to maintaining functional mobility, and finally to maximizing
quality of life. Discussion. Disease staging and the use of functional
outcome measures provide a framework for physical therapy evaluation and treatment of patients with ALS throughout the disease
process. Physical therapists can assist patients with ALS through the
provision of education, psychological support, rehabilitation programs, and recommendations for appropriate equipment and community resources. [Dal Bello-Haas V, Kloos AD, Mitsumoto H. Physical
therapy for a patient through six stages of amyotrophic lateral sclerosis.
Phys Ther. 1998;78:1312-1324.1
Key Words: Amyotrophic lateral sclerosis, Physical therapy, Staging of disease.
Vaninu Dal Bellc+Haas
I
Anne D Kloos
Hiroshi Mitsumoto
Physical Therapy. Volume 78 . Number 12 . December 1998
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Identification of
stage of
amyotrophic lateral
myotrophic lateral sclerosis (ALS) is the
most common motoneuron disease among
adults.' The incidence of ALS is 0.4 to 2.4
cases per 100,000 people worldwide, with a
prevalence of 2.5 to 7 cases per 100,000
Amyotrophic lateral sclerosis is slightly more common in
men than in women, and the average age of onset is the
mid 50s. In 5% to 10% of people with ALS, the disease is
inherited as an autosomal dominant trait and is referred
to asfanzilial amyotrophic lateral ~clerosis.'-~
In 90% to 95%
of people with ALS, there is no family history of the
disease, and these people are said to have sporadic
amyotrophic lateral sclerosis.
In people with ALS, motoneurons in the spinal cord,
brain stem, and cerebral motor cortex degenerate and
result in a variety of signs and symptoms (Tab. 1).lr2The
most frequent initial symptom, which occurs in more
than 705%of patients, is focal weakness beginning in the
leg, arm, or bulbar musc1es.l Amyotrophic lateral sclerosis is characterized by the absence of sensory symptoms
and findings, although pathological conditions have
been found in the sensory systems of some patients with
ALS.1 Cognition, extraocular eye movements, and autonornic, bowel, bladder, and sexual functions usually
remain intact.
Muscle weakness progresses over time, and the pattern
and rate of physical deterioration vary widely among
individuals. Patients must cope with continual, multiple
functional losses of speech, swallowing, mobility, and
sclerosis can assist
physical thetwpists
in designing
appropriate
intervention.
activities of daily living
(ADL). Death from
from respiratory failure, with 50% of
patients surviving only
3 to 4 years after the
onset of symptoms
unless mechanical ventilation is used to sustain breathing.l.4
A major breakthrough in ALS research was made in 1993
with the discovery that some forms of familial ALS are
caused by a mutation in the superoxide dismutase-1
(SOD-1) gene located on chromosome 21.5 Superoxide
dismutase eliminates free radicals, which-although
products of normal cell metabolism-are damaging to
~ - ~ suggested that
cells. The results of some s t u d i e ~ have
free-radical injury is also involved in cases of sporadic
ALS. In addition, an unusual accumulation of neurofilaments, which are proteins that usually serve as a supporting structure in neurons, were found in motoneuron
a~ons.~
Other
J ~ researchers have found that levels of
glutamate, an excitatory neurotransmitter, were elevated
in cerebrospinal fluid1' and that glutamate uptake was
decreased in the brain tissues of people with ALS.'2
Neuroscientists speculate that overstimulation of nerve
cells by excessive amounts of glutamate could lead to cell
death.' The immune system may also be involved in ALS
because the serum of individuals with ALS has been
V Dal BelleHaas, PT, is Assistant Professor, Department of Health Sciences, Cleveland State University, Health Science Bldg, Room 122, 2501
Euclid Ave, Cleveland, OH 441 15 (USA) ([email protected]). Address all correspondence to Ms Dal BelleHaas.
AD Kloos, PT, is Research Physical Therapist, Department of Neurology, The Cleveland Clinic Foundation, Cleveland, Ohio.
H Mitsumoto, MD, DSc, is Director of the Amyotrophic Lateral Sclerosis Association Center, Department of Neurology, The Cleveland Clinic
Foundation.
This article was submitted Februaq 28, 1997, and was accepted August 6, 1998,
Physical Therapy . Volume 78 . Number 12 . December 1998
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Dal BelleHaas et al
. 13 13
Table 1.
Clinical Signs and Symptoms of Amyotrophic Lateral Sclerosis
Upper
Motoncuron Signs
Lower
Motoneuron Signs
Spasticity
Hyperreflexia
Pathological reflexes
Muscle weakness
Muscle atrophy
Fasciculations
Hyporeflexia
Hypotonicity
Muscle cramps
Bulbar Signs
Respiratory Signs and
Symptoms
Other Signs and
Symptoms
Dysarthria
Dysphagia
Sialorrhea
Pseudobulbar palsy
Nocturnal respiratory difficulty
Exertional dyspnea
Accessory muscle use
Paradoxical breathing
Fatigue
Weight loss
Cachexia
Tendon shortening
Joint contractures
shown to contain antibodies to motoneurons.13
Although these potential mechanisms are related to
ALS, the pathogenesis of ALS is complex and remains
unknown.
No cure exists for ALS, but medications have beneficial
effects. Riluzol (Rilutec) inhibits glutamate release and
antagonizes the glutamate receptor, which prolongs
survival.14 Myotrophin (insulin-like growth factor-I
[IGF-I]) moderately lessens motor dysfunction, promoting the survival of motoneurons and regeneration of
motor nerves.15 Riluzol is now available by prescription,
and the US Food and Drug Administration has approved
the use of IGF-I as an investigational new drug.
Staging of Patients With ALS
Sinaki and Mulder16J7 described the natural course of
ALS as consisting of 6 stages. The stages are based on the
progressive loss of function in the trunk and extremity
muscles. Identification of a patient's stage can assist
physical therapists in designing intervention throughout
the disease process.
Stage 1
The patient is in the early stages of the disease and is
independent in mobility and ADL. A specific group of
muscles are mildly weak, which may be manifested as
limitations in performance or endurance, or both.16
Therapy consists of patient and caregiver education,
energy conservation training, modification of the home
and workplace, and psychological support. The patient is
advised to continue normal physical activities. General
active range of motion (AROM) and stretching of
affected joints, resistive strengthening exercises of unaffected muscles with low to moderate weights, and aerobic activities (eg, swimming, walking, bicycling) at s u b
maximal levels may be prescribed.
Stage I1
The patient has moderate weakness in groups of muscles. The patient, for example, may have a foot drop on
one or both sides or may have intrinsic muscle weakness
in one hand that interferes with fine motor activities.16
Assessing the need for and providing appropriate equip13 14 . Dal BelleHaas et a1
ment and assistive devices to support weak muscles is the
primary goal of intervention. The patient is encouraged
to continue stretching and AROM exercises, strengthening exercises of unaffected muscles, and aerobic activities, as he or she is able. In addition, the patient and
caregivers are instructed in performing active-assistedrange-of-motion (AAROM) and passive-range-of-motion
(PROM) exercises of affected joints to prevent
contractures.
When designing a strengthening exercise program for a
patient in ALS stages I and 11, the therapist should
consider prevention of overuse fatiguela and disuse
atrophy. Evidence from patients with some other neuromuscular diseases indicates that highly repetitive or
heavy resistance exercise can cause permanent loss of
force in weakened, denervated m u s ~ l e . ~ A
" ~marked
~
reduction in activity level secondary to ALS, however,
can lead to cardiovascular deconditioning and disuse
weakness beyond the amount caused by the disease.22
Sinaki did not advocate any vigorous exercise for individuals with ALS, stating that "in most patients, no
exercise other than that inherent in everyday ambulatory
activities is indicated."l7(p170,Other authors, however,
have reported beneficial effects of specific muscle
strengthening and endurance exercise programs on
patients with ALSZ3jz4 and other neuromuscular
disea~es.Z~-~O
Although functional gains as a result of exercise have not
yet been determined, these st~dies,Z~-~O
along with
reports from our patients, suggest that exercise programs may be physiologically and psychologically beneficial for patients with ALS, especially when implemented before there is a great deal of muscle atrophy.
Therefore, we have modified Sinaki and Mulder's framework to include muscle strengthening and endurance
exercises when tolerated, particularly during the early
stages of the disease. We continuously adjust the intensity of exercise to prevent excessive fatigue, while at the
same time promoting use of intact muscle groups to
perform functional activities. Patients are advised not to
carry out any activities to the point of extreme fatigue
(ie, inability to perform daily activities following exercise
Physical Therapy . Volume 78 . Number 12 . December 1998
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due to exhaustion, pain, fasciculations, or muscle cramping). Some individuals have cramping or fasciculations
because of ALS; an increase in these lower motoneuron
(LMN) symptoms may indicate overuse. Patients are also
advised to exercise for several brief periods throughout
the day, with sufficient rest between exercise sessions.
The total daily exercise time is 30 to 45 minutes. This
total daily exercise time would be divided into 2 or 3
sessions depending on the patient's tolerance, response
to exercise, and daily schedule. The exercises may
include resistive exercises, active exercises, and aerobic
conditioning exercises (eg, cycling, walking, swimming).
Stage 111
The patient remains ambulatory but has severe weakness
in certain muscle groups that may result in severe foot
drop or a markedly weak hand. The patient may be
unable to stand up from a chair without help. Overall,
the patient may exhibit mild to moderate limitation of
function.16In this stage, as with all other stages, the goal
is to keep the patient physically independent. Adaptive
equipment (eg, ankle-foot orthoses [AFOs], splints, electrically powered height-adjustable chairs) may be
needed to support weak muscles, decrease energy
expenditure, and improve the patient's safety and mobility. Patients may begin to report heaviness and fatigue
while holding their head up in this stage, and they may
benefit from a soft collar. In addition, to avoid exhaustion, a wheelchair may become necessary when traveling
long distances.
Stage IV
The patient in this stage has severe weakness of the legs
and mild involvement of the arms. Thus, the patient uses
a wheelchair and may be able to perform ADL.16 PROM
and AAROM exercises are recommended to prevent
contraclures. Strengthening exercises and AROM of any
noninvolved muscles should be continued. As general
mobility decreases, the need for instruction to inspect
the skin for pressure areas increases, and sleeping and
sitting systems that allow position changes and pressurerelief surfaces (eg, egg-crate bed cushion, alternatingpressure bed pad) may be recommended.
Stage V
This stage is characterized by progressive weakness and
deterioration of mobility and endurance. The patient
uses a wheelchair when out of bed, and arm muscles may
exhibit moderate or severe weakness. Transferring the
patient to and from a wheelchair becomes a major effort,
and a lift may be necessary.I6 Patients become unable to
move themselves in bed; thus, frequent repositioning
and skin care by the caregiver are necessary. Pain may
become a major problem in immobilized joints and
needs to be addressed in the overall treatment plan. Pain
is addressed according to the pathophysiology of the
Physical Therapy . Volume 78
problem causing the pain. For example, pain due to
spasticity or muscle cramping may be addressed by
stretching and massage; pain due to contractures may be
addressed by the use of thermal modalities, stretching,
splinting, and soft tissue mobilization; pain to due joint
hypomobility or acute injuries (eg, trauma to a shoulder
resulting from a fall) may be addressed by joint mobilization, the use of thermal modalities, and electrical
stimulation; pain due to joint instability may be
addressed by the use of assistive devices, orthoses, slings,
and positioning; and so on. Patients may be unable to
hold their head up for extended periods. Thus, a
semirigid collar (eg, Philadelphia collar,* Newport collart) is appropriate in this stage of the disease. If the
patient has a tracheostomy, a Miamij c o ~ l a ror
, ~a similar
collar that allows for anterior neck access, is prescribed.
By maintaining the head in a neutral position, breathing, eating, and seeing may be facilitated.'
Stage VI
The patient must remain in bed and requires maximal
assistance with ADL. A hospital bed should be prescribed. Frequent repositioning of the body, padding to
prevent uneven pressure, and prevention of venous stasis
in the legs are crucial. Pain management continues to be
important. "Head drop" from weak neck extensor muscles may become a major problem. Progressive respiratory distress develops in this stage, and a suction
machine should be available.16 Cardiopulmonary physical therapy techniques may be required, such as body
positioning to optimize ventilation-perfusion matching
and prevent atelectasis; modified postural drainage positioning to decrease retention of secretions and aid in
mobilization of secretions; self-assisted (if the patient is
able) or manually assisted coughing techniques to compensate for a weak, ineffective cough and to aid in
mobilization of secretions; and airway clearance techniques (ie, vibrations, shaking, percussions) to mobilize
secreti~ns.~
Goals
l
in this stage are similar to those of
hospice care: to address the patient's and caregivers'
needs and to maximize the quality of each day. Nurses,
aides, and caregivers are instructed in home programs
depending on the patient's problems and needs
(eg, PROM, stretching, transfers, massage, airway clearance techniques).
In any stage of the disease, the patient may have respiratory problems or pain, or both. Deep breathing exercises, assisted coughing techniques, and airway clearance
techniques may be recommended to address acute or
chronic respiratory problems. Pain may occur as a result
of previous musculoskeletal impairments (eg, patient
' Armstrong Medical Industries Inc, 575 Knightsbridge Pkwy, I.incolnshire, IL
60069.
'California Medical Products Inc, I901 Obispo Ave, Long Beach, CA 90804.
'Jerome C e ~ c a Spine
l
System, 305 Harper Dr, Moorestown, NJ 08057-3239.
. Number 12 . December 1998
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Dal BelleHaas et al
. 1 3 15
may have concurrent osteoarthritis), spasticity, muscle
cramping, weakness or atrophy, and joint instability
caused by muscle imbalances and should be addressed
accordingly.
Examination and Evaluation for Patients
With ALS
Physical therapist examination and evaluation for
patients with ALS throughout the stages of the disease is
necessary to plan appropriate treatment programs. The
tests we include in our assessment are used, along with
general observation and patient report information, to
determine the stage and rate of progression of the
disease, to uniformly communicate patient status to
other health care professionals, and to evaluate the
effects of medical and physical therapy treatments.
The Amyotrophic Lateral Sclerosis Functional Rating
Scale (ALSFRS)32is used to assess functional status and
changes in patients (Appendix 1). The evaluator asks the
patient (or the caregiver if the patient cannot speak) to
rate his or her function for each of the 10 items of the
ALSFRS on a scale ranging from 0 (unable to attempt
the task) to 4 (normal function). The evaluator questions the patient by asking "How are you doing at..." and
prompts the patient by using one of the available
choices. Each scale item has criteria related to patient
functioning, and the evaluator must have a thorough
understanding of each item's criteria. In a study of 75
patients," the ALSFRS was found to correlate positikely
with measures of upper-extremity and lower-extremity
muscle force ( r = .88 and 3 6 , respectively) and demonstrated good internal consistency (Cronbach a= .815),
test-retest reliability ( r = .88), and sensitivity to change.
The Schwab and England Rating Scale (SERS) is an
1l-point global measure of function that asks the patient
(or caregiver) to report ADL function from 0% (vegetative functions only) to 100% (normal) by circling the
number that best corresponds to the phrase that
describes the patient at the moment (Appendix 2).33
The ALS Ciliary Neurotrophic Factor Treatment Phase
1-11 Study Group found that the test-retest reliability of
the SERS was excellent ( r =.94) and that SERS scores
correlated well with qualitative and quantitative changes
in patients' function.32
Assessment of motor function in individuals with ALS
should include impairment and disability measures that
can detect both upper motoneuron (UMN) and LMN
loss. Quantitative muscle testing for these patients consists of measuring maximal voluntary isometric contractions (MVICs) of the shoulder extensors, elbow flexors,
ankle dorsiflexors, knee extensors, and hip flexors using
a strain gauge tensiometer system.34This method eliminates muscle length and contraction speed as factors in
testing."-" Measurement of MVlCs is currently considered by some experts to be the most direct technique for
investigating motor unit loss.34 Timed 4.6m (15-ft)
walking tests assess the impact of physical impairments,
such as reflex abnormalities, lower-extremity muscle
weakness, and diminished motor control, on ambulation
speed." The "PaTa" test, a test of bulbar function
(oral-labial dexterity), requires the patient to repeat the
syllables "PaTa" as many times as possible for 5 seco n d ~In. ~
patients
~
with ALS, the ability to perform a task
may not change until a critical level of motoneuron loss
is reached. Thus, the timing of motor tasks may be a less
sensitive measure of disease progression than isometric
muscle force testing using a t e n ~ i o m e t e r . ~ ~ , ~ ~
The modified Ashworth Spasticity Scale is a clinical
measure of resistance to passive stretch that has been
shown to produce reliable data.40,41Scores range from 0
(no increase in muscle tone) to 4 (affected parts rigid in
flexion or extension) .40,41The Purdue Pegboard Test4'
involves placing pins in a pegboard to assess right-hand
and left-hand prehension, manual dexterity, and gross
movement of the hands, fingers, and arms. Normative
values are available, and the test has been used in various
patient population^.^^ Pulmonary function has a marked
impact on an individual's comfort, ability to communicate, and quality of life. Thus, forced vital capacity (FVC)
and maximum inspiratory pressure (MIP), which are
sensitive measures of respiratory f u n c t i ~ nare
, ~ evalu~~~~
ated using a desktop spirometer.#
Case Description
Patient
The patient was a 59-year-old woman who was referred to
the multidisciplinary neuromuscular clinic at The Cleveland Clinic Foundation (Cleveland, Ohio). She reported
that, 1 year prior to the initial clinic visit, she experienced cramping in her right hand and, later, slowly
progressive hand weakness, especially with pinching
maneuvers. One to 2 months before the initial visit, she
noted that her feet "slapped" occasionally during walking, and she had painless twitching of the muscles in her
right hand, forearm, and upper arm. She reported no
respiratory or bulbar symptoms.
The patient had been treated for ulcerative colitis for 20
years, and she had bilateral carpal tunnel releases in the
year prior to experiencing cramping in her right hand.
She lived with her husband in a house with 10 stairs to
the basement with a railing on the right. She was not
using any assistive devices at the time of the initial
assessment. The patient and her husband had 4 grown
"uritan-Bennett
Corp, Boston Division, 265 Ballardvalle St, Wilmington. MA
01887.
13 16 . Dal Bello-Haas et al
Physical Therapy . Volume 78 . Number 12 . December 1998
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children, 2 of whom lived in the area. She described her
family as very supportive. She was a homemaker who
enjoyed knitting and golfing. She was right-handed.
Neurological Examination
A clinic:al examination revealed that the patient had a
clonic jaw reflex; a positive nasolabial reflex; weakness,
wasting, resistance to PROM, and pathological reflexes
in both arms (positive Hoffman's sign); weakness and
pathological reflexes in both legs (positive Babinski
sign); and a positive Babinski reflex on the right side.
Based on these findings, "definite ALS" was diagnosed by
the neurologist because, according to the El Escorial
riter ria,"^ the patient had "LMN signs [weakness, wasting] in at least 2 body regions and UMN signs [pathological reflexes, spasticity] in at least 1 body region, with
UMN signs present above the level of LMN signs, and
historical evidence of progressive disease" and all other
disease processes were excluded.
Electrornyographic studies showed (1) low compound
motor action potentials in all extremities, (2) normal
sensory nerve conduction, (3) fibrillations and fasciculations in all extremities, and (4) widespread neurogenic
changes in motor unit action potentials, abnormal
recruitment patterns in the arms, and mild to moderate
changes in the distal leg musculature. These findings
were consistent with an electrodiagnosis of motoneuron
disease.'+(5
The pa~.ientand her family were informed about the
diagnosis and the progressive nature of the disease. We
believe it is important that the patient and family be
given as much information as possible about the disease
process, so that informed decisions about care can be
made. At every stage of the disease, the patient participated in goal setting and treatment planning.
Neurontin, a drug that is used in the treatment of
epilepsy and that is thought to have antiglutamate
properties, was prescribed for the patient. In addition,
she was instructed to take antioxidative vitamins with all
meals.
Physical Therapy Examination
The patient stated that she could perform all functional
activities independently, but she noticed that she
ascended and descended stairs more slowly than usual.
Cutting meat was difficult, and she needed to rock
fo~wardto stand up from a seated position. She also
reported cramping in her calves in the evening.
She had marked wasting of the interosseous muscles
bilaterally. Range of motion (ROM) was normal for all
joints, except the thumbs. She was only able to oppose
her thumbs to the third digits. Bilateral lower-extremity
strength was assessed as 5/5 on the Medical Research
Council Scale,47except for the hip flexor group (right
side=4/5, left side=4+/5). Shoulder muscle strength
was assessed as 4-/5 (right side) and 4/5 (left side), and
elbow muscle strength was assessed as 4/4 (right side)
and 4+/5 (left side). Ashworth Spasticity Scale scores
were 1+ for both arms and 1 for both legs. Grip strength
was assessed using a Jamar adjustable hydraulic handheld dynamometerli (right hand=6 kg, left hand=12
kg). Balance was assessed by timing the duration of
holding a unilateral stance (right side=l second, left
side=2 seconds). Gait was independent of assistive
devices and was negative for foot drop.
Normal range for thumb opposition is considered the
ability to touch the thumb to the top of the fifth finger.
Some surgeons consider normal opposition as the ability
to touch the base of the fifth digit with the tip of the
t h ~ m b . ~ T hreliability
e
of ROM measurements varies
among different j~ints.~"eliability testing of thumb
ROM-has not been conducted. Using the Medical
Research Council Scale, a grade of 5/5 is considered
normal. The reliability of manual muscle test grades is
low and varies depending on the strength of the musA grade of 0 on the modified Ashworth Spasticity
Scale indicates "no increase in muscle tone" and would
be considered "normal." Intrarater agreement has been
reported as 86.7% agreement among raters, but testing
was performed with the elbow flexors only.41 Normal
values for grip strength using the Jamar dynamometer
for a woman aged 60 years were reported as follows: right
side=24.9?4.5 kg (55+ 10 Ib) , left side=20.9?4.5 kg
(46k 10 Ib) ." Interrater reliability using the Jamar dynamometer was found to be 2.99, and test-retest reliability
(1 week) was found to be 2.88.5"he
duration that an
individual can maintain single-leg stance is related to
age. In individuals aged 50 to 59 years, Bohannon et a154
found that the mean time for one-legged timed balance
was 29.4 seconds (SD= 2.9). Reliability testing results
have not been published.
The patient rated herself as 80% on the SERS." The
FVC and MIP results indicated normal respiratory capacity. Walking speed was 3.4 seconds for the 4.6-m [15-ft]
walk test, which was below the normal value of 5 2
seconds. Bulbar function was considered normal (ie, 15
times or more in 5 seconds)94;she repeated the "PaTa"
test 15 times in 5 seconds. Hand function was severely
impaired, as shown by the Purdue Pegboard Test where
only 3 peg holes were filled in 30 seconds with the right
hand and 2 peg holes were filled in 30 seconds with the
left hand. The quantitative muscle testing and ALSFRS
results are shown in Figure 1 and Table 2, respectively.
We concluded that her arms were more affected than
her legs, with distal muscle groups having greater weak"Therapeutic Equipment Corp. 60 Page Rd,Clifton, NJ 07012.
Physical Therapy . Volume 78 . Number 12 . December 1998
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Dol Bello-Haas et al . 1317
the shoulder and hip flexor muscles
in sitting; self-stretching of thumb
muscles; and aerobic exercise, using
a stationary bicycle, at 65% of maximum heart rate for 10 minutes, 2
times per day. She was told about the
effects of overwork fatigue on muscles, and she was told to avoid activities that required lifting greater
than 6.8 kg (15 lb). The exercises
were written up as a home program.
Exercise sessions were limited to
20-minute sessions twice per day and
to 10 repetitions of each AROM and
resistive exercise.
The use of AFOs and the purchase
of a cane (for future use) were also
Figure 1.
discussed. She was given energy conMuscle force of a 56-year-old woman with amyotrophic lateral sclerosis (ALS) at the time of
servation
information (eg, how to
diagnosis. Distal muscles tended to be weaker than proximal muscles. Asterisk (*) indicates subject
carry
her
laundry basket from the
data from The National Isometric Muscle Strength Database Consortium. Muscular weakness
assessment: use of normal isometric strength data. Arch Phys Med Rehobil. 1 9 9 6 ; 7 7 : 1 251 - 1 2 5 5 . basement), and home modifications
to ease mobility were suggested (eg,
sitting in raised chairs, using a raised
Table 2.
toilet seat). To relieve calf cramping, she was instructed
Initial Amyotrophic Lateral Sclerosis Functional Rating Scale 3 2 Scores
in gastrocnemius muscle massage and stretching.
of a 56-Year-Old W o m a n With Amyotrophic Lateral Sclerosis
Stretching was to be performed in a standing position,
with the stretch held for 20 seconds, and repeated at
Possible
least 5 times in the morning and evening, or as required
1 Function
Score Score
when cramping occurred. She was referred for occupa1 Speech
4
3
tional therapy for a right wrist-thumb splint to be worn
1 Salivation
4
4
during activities, a resting hand splint to prevent claw1 Swallowing
4
4
ing, cylindrical foam for utensils to increase gripping
1 Handwriting (pre-ALS dominant hand)
4
3
ability, and instruction in dressing and bathing. The
1 Cutting food and handling utensils (patients
patient
and her husband were provided with informa4 or NA
without gastrostomy)
3
Cutting food and handling utensils (patients
tion about the local ALS support group.
with gastrostomy)
1 Turning in bed; adjusting bed clothes
Walking
Climbing stairs
Breathing
Total score
NA°
3
3
3
4
30
NA or 4
4
4
4
4
40
" NA=not applicable.
ness than proximal muscle groups. Overall, these impairments appeared to have a mild impact on her ability to
function.
We determined that the patient was in stage I or II of
ALS, because of the mild weakness in several muscle
groups; marked weakness in the intrinsic muscles that
interfered with handwriting, cutting foods, and handling
utensils; and full independence in mobility and ADL.
Thus, treatment included instruction in a general exercise program, which included resistive exercises of the
lower-extremity muscles in lying with a 0.97-kg (2-lb)
weight; AROM of hip flexor muscles; AROM exercises of
1318 . Dal Bello-Haas et al
Three months after the initial evaluation (Figs. 2-4), the
patient reported a mild, general increase in muscle
weakness, especially in her right arm. Her speech was
slower and slightly nasal, and she now needed to use the
railing to ascend and descend stairs. She reported that
the ROM exercises relieved the "stiffness" and pain in
her right shoulder. She had fallen 1 week before this
visit. Manual muscle test results and ROM of her arms
and legs were unchanged from the previous visit, but
quantitative muscle testing indicated decreased force in
all muscle groups tested. The percentages of decrease in
muscle force over the 3-month period between the initial
assessment and reassessment were as follows: left shoulder extension = 21.9%, right shoulder extension = 4.8%,
left elbow flexion = 15.2%, right elbow flexion = 19.7%,
left grip force = 33.3%, right grip force = 25.0%, left hip
flexion = 8.6%, right hip flexion = 31.7%, left knee extension =26.3%, right knee extension = 27.4%, left dorsiflexion = 21.4%, and right dorsiflexion = 25.0%. Bulbar func-
Physical Therapy . Volume 7 8 . Number 12 . December 1998
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J
tion and overall general function
had decreased from the previous
visit. During walking, dorsiflexion
was slightly decreased during heelstrike on both sides, although it was
more pronounced on the right side.
On the basis of the moderate weakness in the muscle groups and the
m
a
signs of impending foot drop, we
concluded that she was in stage I1 of
M S . We advised her to continue her
.>
B
exercises and to emphasize ROM
$ 20
exercises in her right shoulder and
9
0
bilateral
gastrocnemius
muscle
LL
0
I
I
I
I
I
I
I
I
I
stretching exercises. Using clasped
hands, she was to raise both arms
3
4
5
6
7
8
9
10
11
12
13
overhead in a lying position and use
Months After Initial Evaluation
the left arm to provide a pain-free
stretch to the right shoulder at the Figure 2Changes in forced vital capacity during the course of amyotrophic lateral sclerosis. The patient
end of range, holding the stretch for reported shortness of b reath
~ on exertion when forced vital capacity was about 70% of normal.
a count of 15. She was instructed in
the use of a straight cane, and she
and adult children were taught PROM and AAROM
was givtsn a prescription for a right AFO.
exercises for the affected muscle groups (10 repetitions
done 2 to 3 times per day), slow stretching (hold-relax)
Six months after the initial evaluation (Figs. 2-4), the
patient reported increased right shoulder pain and
techniques for the right shoulder, and transfer techincreasing muscle weakness of all extremities, especially
niques using a gait belt to prevent pulling on her
on the right side. Her speech was slurred, and she
shoulders during transfers and further aggravate the
right should pain. A left AFO was provided.
occasioilally choked on spicy food and on her saliva. Left
shoulder ROM was normal, but active right shoulder
abduction and flexion were decreased by 20 and 80
Seven months after the initial evaluation (Figs. 2-4), the
degrees, respectively. Ashworth scores were 1 for the legs
patient enrolled in a clinical drug trial of brain-derived
neurotrophic factor, which she said improved her
and 2 for the arms. The decreased ROM and shoulder
morale. She was still able to ambulate with a wheeled
pain were attributed to increased spasticity about the
walker, but she required more assistance with ADL. A
shoulder. She could oppose her thumbs to the index
finger only. Quantitative muscle testing showed
totally electric hospital bed with a gel/foam overlay and
decreases in force in all muscle groups. In addition, her
a power reclining lift chair were ordered in anticipation
2.4m timed walk test result increased to 10.1 seconds.
of future problems. The patient's husband reported that
he was glad that he could assist her with her exercises
There was no change in the "PaTa" test, although FVC
because he felt like he was doing something to help her.
decreased by approximately 22%. There appears to be a
tendency for bulbar and respiratory function to decline
concurrently, although this tendency has not been
Nine months after the initial evaluation (Figs. 2-4), the
proven. Motoneuron dysfunction is thought to spread
patient stated that she was finding it more and more
from one contiguous region to another. Because the
difficult to think about the future and that she was
cervical spinal cord segment innervating the diaphragm
extremely saddened that she could not carry out her
is the closest region of the spinal cord to the brain stem,
traditional homemaking role, but had to rely on her
it is expected that loss of bulbar function and loss of
family to perforni ADL. She was extremely weepy and
upper cervical motoneuron function would parallel each
despondent at this visit. The physician was informed and
other. The SERS and ALSFRS scores indicated a moderreferred her to a psychiatrist, who diagnosed her as
ate decrease in overall function.
having clinical depression and prescribed antidepressants. Her husband expressed difficulty in dealing with
her disabilities; thus, a referral to a home care agency
Because of severe weakness in several muscle groups and
was made. A home health aide was provided, and a
moderate limitation of overall function, she was deterniined to be in stage I11 of ALS. She started to use a
referral to a home care physical therapist was made for
wheeled walker for ambulation and a manual wheelchair
PROM exercises and caregiver instruction on patient
transferring and bed positioning techniques. The home
for traveling long distances because of the muscle weakness and decreased respiratory function. Her husband
care physical therapist initially performed the PROM
C
C
Physical Therapy . Volume 78
. Number
12
. December
1998
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Dal Bello-Haas et al . 13 19
a
b
12
100
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.-
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9 1 0 1 1 1 2 1 3
0
3
I
4
Months After Initial Evaluation
-E
,
5
I
6
l
7
I
8
I
,
I
'
I
9 1 0 1 1 1 2 1 3
Months After Initial Evaluation
c
d
40
7i 100
Stages V and VI
In
':g
35
: ,--------------------- - ------
$4
4
A
i 4
Stage III
0
1'01'1
11213
Months After Initial Evaluation
I
3
4
I
5
I
6
I
7
I
8
I
9
10
I
I
11
12
I
13
14
Months After Initial Evaluation
Figure 3.
(a) Changes in "PaTa" test scores during the course of amyotrophic lateral sclerosis (ALS]. Six months after the initial evaluation, the patient first
reported that her speech was becoming slurred. (b) Changes in global functional ability (Schwab and England Rating Scale33 scores) during the
course of ALS. Her scores declined almost 70% in 10 months. (c) Changes in the 6.8-m ( 1 5 4 ) timed-walk test scores during the course of ALS. Six
months after the initiol evaluation, she began using a wheelchair when traveling long distances (30 m). Four months later, she became
wheelchair-bound. (dl Changes in Amyotrophic Lateral Sclerosis Functional Rating Scale32 scores during the courseof ALS. Between the 8th and 14th
months after the initial evaluation, her scores declined by almost 80%.
exercises with the patient's husband observing and
instructed the husband in performing the PROM exercises to ensure the correct technique.
Ten months after the initial evaluation (Figs. 2-4), a
wheelchair was the patient's primary means of mobility.
She had shortness of breath on exertion, especially after
moving short distances, but not when lying supine. In
patients with ALS, in a seated or standing position,
gravity assists the weakened diaphragm in depressing the
abdominal contents during respiration. In a supine
position, however, the abdominal contents are elevated,
and the weakened diaphragm may be unable to depress
the contents to allow for maximum inspiration, leading
to shortness of breath. This was not the case for this
patient. Her W C was 50% of predicted capacity, and she
was now in stage IV of the disease. Because her W C had
decreased to this level, she and her husband were shown
assisted coughing techniques to compensate for the
decreased force available for effective coughing. We
have found that patients with an FVC of 50% or less of
predicted capacity tend to decline rapidly. Thus, she was
1320 . Dal BelleHaas et al
referred to a pulmonologist, who found her to be
hypoxic. She declined noninvasive ventilation. Noninvasive ventilation (as opposed to tracheostomy) provides
ventilatory support through oral or nasal masks and
interfaces using portable ventilators.' Her main problem
at this time was marked weight loss caused by eating
difficulties secondary to spasticity in the jaw. We discussed inserting a percutaneous endoscopic gastrostomy
(PEG) tube if weight loss continued. She had discontinued using antidepressants because she was unable to
tolerate the side effects and rehsed to try a different type.
One year after the initial evaluation (Figs. 2-4), she
could ambulate only 4.5 to 6.8 m (10-15 ft) before
becoming fatigued. Although the patient's primary
means of mobility was a wheelchair, she continued to
ambulate short distances about the house. Her caregivers stated that transfers were now difficult because her
knees would buckle and her legs became twisted. They
continued the PROM program and assisted her in
performing all of her self-care activities and ADL. She
continued to lose weight, but declined a PEG tube. She
Physical Therapy . Volume 78
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. Number 12 . December 1998
Figure 4.
Changes in muscle force of the legs and arms during the course of amyotrophic lateral sclerosis. The increase in force of some muscles between the
sixth and ninth months after the initial evaluation may be related to the increased spasticity noted at that time. DF=dorsiflexion.
was referred for hospice care, and physical therapy
recommendations (ie, caregiver instruction regarding
Hoyer lift and sit-pivot transfers and positioning the
patient in the wheelchair with a lap tray) were arranged
with the coordinator. A Hoyer lift with a commode sling
was ordered. A lap tray was recommended for her
wheelchair to provide arm and trunk support. Her
caregivers were instructed in sit-pivot transfers.
By 14 months after the initial evaluation (Figs. 2-4), no
new problems requiring physical therapy had emerged.
She continued to decline noninvasive ventilation and a
feeding tube. Fifteen months after the initial evaluation,
she accepted noninvasive ventilation. Sixteen months
after the initial evaluation, her family informed us of her
death.
Discussion
Despite the poor prognosis of ALS, we believe that
physical therapy is an important component of the
overall care of patients with this disease. We believe that
patients with ALS may exhibit a variety of emotions
related to the changes and functional losses that they
experience. Depression, hostility, anger, denial, and
despair are responses with which they may need to
dea1.l~
Through
~~
active participation in goal setting and
treatment planning, patients may gain some sense of
control over what is happening to their bodies, enabling
them to better cope with functional losses. Throughout
all stages of the disease, therapists must assess the
patients' coping mechanisms, watch for signs of depression, refer the patients to other health care professionals
as necessary, assist them in making life choices, and
provide psychological support to the patients and caregivers. The patient and her husband, for example, often
told us how beneficial physical therapy was to them,
especially the exercise prescription and the information
about mobility and assistive devices. Physical therapists
call assist patients with ALS throughout all stages of the
disease through the provision of education, psychological support, rehabilitation programs, and recommendations for appropriate equipment and community
resources.
Research is needed to determine whether physical therapy beginning during the early stages of ALS can reduce
the incidence of falls and the development of severe
contractures, painful joints, and skin breakdown. Avoidance of these complications might maintain the patient's
mobility and function for a longer duration, thereby
decreasing the patient's need for assistance with ADL
and transfers from caregivers. More research on exercise
and rehabilitation programs is also needed if therapists
are to make evidence-based decisions regarding other
therapeutic intelventions. Randomized studies that compare the effects of different exercise programs in terms
of type, frequency, duration, and intensity of exercises,
for example, are needed. Outcome measures should
include not only force measurements but, more importantly, functional and quality-of-life measures. As new
medications that slow the progression of the disease
become available, studies are needed to assess the combined effects of medications and exercise on the functional abilities and quality of life of patients.
We believe that our expanded version of Sinaki and
Mulder's approach to staging patients with ALS and the
use of functional outcome measures aid in planning
appropriate treatment programs that not only address
the patient's current physical and psychological problems, but also take into account the patient's future
needs. We also believe that physical therapists' care and
compassion can have an impact on the well-being of
people living with this disease.
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of
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Appendix 1.
Amyotrophic Lateral Sclerosis Functional Rating Scale32
1. Speech
4 Normal speech processes
3
2
1
0
Detectable speech disturbance
Intelligible with repeating
Speech combined with nonvocal communication
Loss of useful speech
2. Salivation
4 Normal
3
2
1
0
2
1
0
Slight but definite excess of saliva in mouth; may have
nighttime drooling
Moderately excessive saliva; may have minimal drooling
Marked excess of saliva with some drooling
Marked drooling; requires constant tissue or handkerchief
Normal eating habits
Early eating problems; occasional choking
Dietary consistency changes
Needs supplemental tube feeding
Nothing by mouth (exclusively parenteral or enteral feeding]
0
Slow or sloppy; all words are legible
Not all words are legible
Able to grip pen, but unable to write
Unable to grip pen
2
1
0
3
2
1
0
1
0
0
5b.Cutting food and handling utensils (alternate scale for
patients with gastrostomy)
4 Normal
Early ambulation difficulties
Walks with assistance
Nonambulatory functional movement only
N o purposeful leg movement
9. Climbing stairs
4 Normal
3
2
0
Somewhat slow and clumsy, but no help needed
Can turn alone or adjust sheets, but with great difficulty
Can initiate, but not turn or adjust, sheets alone
Helpless
8. Walking
4 Normal
3
1
lnde endent and complete selfcare with effort or decreased
ePciency
lntermittent assistance or substitute methods
Needs attendant for selfcare
Total dependence
7. Turning in bed and adjusting bed clothes
4 Normal
5a.Cutting food and handling utensils (patients without
gastrostomy)
4 Normal
Somewhat slow and clumsy, but no help needed
Can cut most foods, although clumsy and slow; some help
needed
Food must be cut by someone, but can still feed self slowly
Needs to be fed
Clumsy, but able to perform all manipulations independently
Some help needed with closures and fasteners
Provides minimal assistance to caregiver
Unable to perform any aspect of task
6. Dressing and hygiene
4 Normal function
3
2
4. Handwriting
4 Normal
3
2
1
0
3
3. Swallowing
4
3
3
2
1
2
1
Slow
Mild unsteadiness or fatigue
Needs assistance
Cannot do
10. Breathing
4 Normal
3
2
1
0
Shortness of breath with minimal exertion (eg, walking,
talking)
Shortness of breath at rest
lntermittent (eg, nocturnal) ventilatory assistance
Ventilatordeoendent
-
Physical Therapy . Volume 78 . Number 12 . December 1998
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Dal Bello-Haas et al
. 1323
A pendix 2.
Scffwab and England Rating Scale33
1OO%=Completely independent; able to do all chores without slowness, difficulty, or impairment; essentially normal; unaware of any difficulty
90%=Completely independent; able to do all chores with some degree of slowness, difficulty, and impairment; may take twice as long as usual;
beginning to be aware of difficulty
80%=Completely independent in most chores; takes twice as long as normal; conscious of difficulty and slowness
70%=Not completely independent; more difficulty with some chores; takes 3-4 times as long as normal in some chores, must spend a large part of
the day with some chores
60%=Some dependency; can do most chores, but exceedingly slowly and with considerable effort and errors; some chores impossible
50%=More dependent; needs help with half the chores, slower, and so on; difficulty with everything
40%=Very dependent; can assist with all chores, but does few alone
30%=With effort, now and then does a few chores alone or begins chores alone; much help needed
20%=Does nothing alone; can be a slight help with some chores; severe invalidity
1 O%=Totally dependent and helpless; complete invalidity
O%=Vegetative functions such as swallowing, bladder, and bowels are not functioning; bedridden
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1324 . Dal BelleHaas et al
Physical Therapy . Volume 78
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. Number 12 . December 1998
Physical Therapy for a Patient Through Six Stages of
Amyotrophic Lateral Sclerosis
Vanina Dal Bello-Haas, Anne D Kloos and Hiroshi
Mitsumoto
PHYS THER. 1998; 78:1312-1324.
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