Stroke and neurodegenerative disorders. 4. Neurodegenerative disorders.

Abstract

This self-directed learning module highlights diagnosis, treatment, and rehabilitation issues in patients with neurodegenerative disorders, including multiple sclerosis (MS), Parkinson's disease, and amyotrophic lateral sclerosis (ALS). It is part of the study guide on stroke and neurodegenerative disorders in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on the differential diagnosis, diagnostic evaluation, medical management, and rehabilitation issues in MS. Similarly, the differential diagnosis treatment and rehabilitation in Parkinson's disease is discussed. Electrodiagnosis, pharmacologic treatment, and rehabilitation options for ALS are also discussed. OVERALL ARTICLE OBJECTIVES: To review the differential diagnosis, evaluation, medical treatment, and rehabilitation management of patients with MS, Parkinson's disease, and ALS.

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Stroke and Neurodegenerative Disorders. 4. Neurodegenerative
Disorders
Phillip R. Bryant, DO, Carolyn C. Geis, MD, Alex Moroz, MD, Bryan J. O’Neill, MD, Ross A. Bogey, DO
ABSTRACT.
Bogey RA. Stroke and neurodegenerative disorders. 4. Neuro-
degenerative disorders. Arch Phys Med Rehabil 2004;85(3
Suppl 1):S21-33.
Bryant PR, Geis CC, Moroz A, O’Neill BJ,
This self-directed learning module highlights diagnosis,
treatment, and rehabilitation issues in patients with neurode-
generative disorders, including multiple sclerosis (MS), Par-
kinson’s disease, and amyotrophic lateral sclerosis (ALS). It is
part of the study guide on stroke and neurodegenerative disor-
ders in the Self-Directed Physiatric Education Program for
practitioners and trainees in physical medicine and rehabilita-
tion. This article specifically focuses on the differential diag-
nosis, diagnostic evaluation, medical management, and reha-
bilitation issues in MS. Similarly, the differential diagnosis
treatment and rehabilitation in Parkinson’s disease is discussed.
Electrodiagnosis, pharmacologic treatment, and rehabilitation
options for ALS are also discussed.
Overall Article Objective: To review the differential diag-
nosis, evaluation, medical treatment, and rehabilitation man-
agement of patients with MS, Parkinson’s disease, and ALS.
Key Words: Amyotrophic lateral sclerosis; Multiple sclero-
sis; Parkinson’s disease; Rehabilitation.
© 2004 by the American Academy of Physical Medicine and
Rehabilitation
4.1Educational Activity: To discuss the differential diagno-
sis for a 29-year-old woman with intermittent diplopia,
numbness, and tingling involving the right arm and
hand, and frequent tripping during ambulation.
T
of signs and symptoms is extensive. Multiple sclerosis
(MS), central nervous system (CNS) tumor, intracranial arte-
riovenous malformation (AVM) or aneurysm, brainstem
stroke, or infectious process of the CNS all merit inclusion in
the differential. Nevertheless, assuming this young woman has
no history of a stroke or risk factors for stroke, MS is a primary
consideration in the differential.
HE DIFFERENTIAL DIAGNOSIS for this constellation
Description
MS is a chronic inflammatory disease of the CNS. It is
characterized by sclerotic plaques consistent with focal demy-
elination involving the white matter of the brain and spinal
cord.1More recent neuropathologic evidence has confirmed
that some axonal injury also occurs in both the early and late
stages of this illness.2It is thought to be caused, at least in part,
by an autoimmune reaction against myelin antigens, although
environmental factors may also play a role.2The clinical course
is variable and classified into relapsing-remitting, secondary
progressive, primary progressive, and progressive relapsing in
order of frequency, based on the temporal presentation of signs
and symptoms.1The diagnosis of MS is based on a complete
history, clinical examination, neuroimaging results, and se-
lected laboratory testing. This diagnosis is strongly suggested
by a history of neurologic symptoms and documented signs
occurring in a different anatomic distribution at different times.
Early in the course of the disease process, historical and clin-
ical data may be limited, making it difficult to establish a
definitive diagnosis. However, over time, the differential diag-
nosis may become more focused and the actual diagnosis more
evident.
Symptoms
Because of the scattered sites of focal demyelination, the
clinical presentation can be quite variable among individuals
with MS and within the same individual from 1 point in time
to another. Common sites of demyelination include the white
matter adjacent to the lateral ventricles, the floor of the fourth
ventricle, the corpus callosum, the periacqueductal region, the
optic nerves and chiasm, the corticomedullary junction, and the
white matter tracts of the spinal cord. Visual loss, oculomotor
disturbances, dysarthria, and dysphagia can occur as a conse-
quence of cranial nerve and brainstem lesions.3
People with MS may also manifest scattered weakness,
variable degrees of numbness and tingling, spasticity, fine and
gross motor incoordination, bowel and bladder dysfunction,
impotence, and cognitive dysfunction. The most frequent
symptoms seen at onset of MS include sensory symptoms in
the arms and legs (27%–40%), unilateral visual loss (16%–
29%), motor and gait abnormalities (19%–40%), incoordina-
tion (9%–20%), diplopia (7%–18%), vertigo (4%–7%), bladder
and bowel dysfunction or sexual problems (5%–10%), and
cognitive deficits (3%).1,4Although the differential diagnosis
of many of these individual symptoms is extensive, few disor-
ders present with the array of symptoms and in the temporal
manner often witnessed in patients with MS. Optic neuritis,
internuclear ophthalmoplegia (secondary to a lesion of the
medial longitudinal fasciculus), a positive Lhermitte sign, and
heat intolerance are particularly suggestive of MS. In contrast,
generalized hyporeflexia or areflexia, homonymous hemianop-
sia, hemiparesis, hemisensory deficits, diffuse muscle weak-
ness, and aphasia are less likely to be caused by MS.1
Diagnosis
The Shumacher criteria for MS1are based on identifying
evidence by neurologic examination of 2 or more CNS lesions
and 2 or more distinct patterns of worsening separated in time.
It also includes the stipulation that the CNS pathology be
primarily consistent with white matter disease. Although it can
From the Division of Physical Medicine and Rehabilitation, University of Utah
School of Medicine, Salt Lake City, UT (Bryant); Neuroscience Center, Halifax
Medical Center, Daytona Beach, FL (Geis); Department of Rehabilitation Medicine,
New York University School of Medicine, Rusk Institute of Rehabilitation Medicine,
New York, NY (Moroz); Department of Rehabilitation Medicine, Thomas Jefferson
University, Philadelphia, PA (O’Neill); and Department of Physical Medicine and
Rehabilitation, Rehabilitation Institute of Chicago, Chicago, IL (Bogey).
No commercial party having a direct financial interest in the results of the research
supporting this article has or will confer a benefit upon the authors(s) or upon any
organization with which the author(s) is/are associated.
Reprint request to Phillip R. Bryant, DO, Div of PM&R, R1-3, Univ of Utah
Health Sciences Ctr, 30 North 1900 East, Salt Lake City, UT 84132, e-mail:
phillip.bryant@hsc.utah.edu.
0003-9993/04/8503-8965$30.00/0
doi:10.1053/j.apmr.2003.12.007
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be used to diagnose most patients with MS, the criteria are
limited to patients with onset of symptoms between 10 and 50
years of age. It is known, however, that MS can occur outside
this age range. Also, based on these criteria alone, serial
neurologic evaluations of patients over extended periods of
time may be required to establish a definitive diagnosis. In
addition, patients with documented MS do not necessarily
match all the Schumacher criteria.
The introduction of magnetic resonance imaging (MRI) has
greatly enhanced the ability to document CNS lesions and has
allowed earlier diagnosis of MS. This, in turn, has provided the
opportunity for earlier treatment of MS. As a result, the Inter-
national Panel on Multiple Sclerosis Diagnosis5developed a
revised set of diagnostic criteria for MS in July 2001, which
includes greater emphasis on the role of MRI in diagnosing
MS.
This subject’s history of frequent tripping during walking
can be caused by weakness, spasticity, motor incoordination,
ataxia, vertigo, or sensory and motor polyneuropathy. Given
her history of right arm and hand numbness and tingling and
frequent tripping, cervical myelopathy as well as brainstem or
intracerebral stroke are diagnostic considerations. This patient
could also have cervical myelopathy with concomitant cervical
radiculopathy or brachial plexopathy. The latter disorders,
however, would not explain her diplopia.
The hallmark of MS is the presence of multiple lesions in the
periventricular regions of the brain noted by MRI of the brain.1
An MRI of the brain is the most sensitive diagnostic test for
MS. Typically, MS lesions present as hyperintense white-
matter signals on T2-weighted and fluid-attenuated inversion
recovery images. The MRI may also reveal gadolinium en-
hancement of active lesions, hypointense areas consistent with
gliosis, and brain atrophy on T1-weighted studies. Serial MRI
tests are a sensitive indicator of disease progression.6
Despite the extensive differential diagnosis that may account
for each of this patient’s symptoms, far fewer diagnoses
present with all 4 symptoms simultaneously and no single
anatomic lesion can explain all her symptoms, except perhaps
an isolated brainstem lesion. However, if on further question-
ing this patient reveals a history of additional symptoms attrib-
utable to different anatomic locations, then MS is arguably the
primary diagnostic consideration.
4.2 Educational Activity: The aforementioned patient is
diagnosed with MS. Discuss the pharmacologic treat-
ments currently available.
Treatment of MS can be divided into symptomatic treatment
and treatment to affect the natural course and pattern of the
disease. It should be emphasized that treatment must be indi-
vidualized.
Steroids and immune-modulating agents are designed to
alter the course of MS. Generally, after a clinical relapse of
MS, there will be improvement of a variable degree. Many will
have residual impairment that, over a series of relapses, may
persist. Corticosteroids appear to facilitate more rapid recovery
from an acute relapse and reduce MS plaque enhancement on
MRI. It is unclear, however, if corticosteroids reduce long-term
cumulative disability. The dosing and duration of corticoste-
roid treatment is variable.7,8Optimal duration of treatment is
determined by the clinical response and is generally short term.
The mechanism of action is unknown. Selection of oral and
intravenous steroids is based on previous response and severity
of the symptoms.7,8Appropriate prophylactic measures should
be implemented to prevent potential complications associated
with steroid use, including osteoporosis, peptic ulcer disease,
hyperglycemia, and hypokalemia.8
The National Multiple Sclerosis Society recommends early
initiation of immunomodulating medications in persons who
have a definite diagnosis of MS and evidence of ongoing
disease activity.9Interferon-? (Betaseron, Avonex, or Rebif)
and glatiramer acetate (Copaxone) alter the natural course of
MS. The mechanism of action is different in the 2 groups.
Interferon-? acts at the blood-brain barrier and impairs en-
trance of inflammatory cells into the CNS. Glatiramer acetate
allows T-helper cells to enter the CNS decreasing inflammation
through bystander suppression.10
Immunosuppressive agents, including azathioprine and cy-
closporine, have been used, but significant side effects limit
treatment. Plasmapheresis has shown mixed results.7There is
emerging evidence that statins may have an immunomodula-
tory effect in vitro and further study is underway.11
4.3Educational Activity: Over the next 2 years, the pa-
tient develops worsening spasticity in both legs, blad-
der incontinence, and decreased short-term memory.
Outline treatment options.
Spasticity is a frequent presentation in MS. It has been
identified in up to 75% of patients.12Progressively worsening
spasticity is associated with decreasing motor coordination.
The lower limbs are typically more spastic than the upper limbs
in MS.12If severe, spasticity can limit and sometimes eliminate
the ability to ambulate. It can also be a source of discomfort.
However, some degree of spasticity, such as extensor spasticity
of the quadriceps, may actually enhance an individual’s ability
to transfer, stand, and ambulate. For this reason, it is important
to focus on improving function and ease of care, as well as
alleviating pain, rather than simply eliminating the spasticity.
In addition to judicious treatment with medications, attention
should also be directed toward addressing conditions that may
exacerbate the spasticity, such as bladder infections, bowel
impaction, and ingrown toenails.
In this patient’s case, addressing her spasticity with the
lowest effective dose of baclofen or tizanidine would be a
reasonable initial choice. In selected patients, particularly those
unresponsive to oral medications and physical modalities, bot-
ulinum toxin injections may be beneficial in reducing spasticity
(see Study Guide, Activity 3.1).13Intrathecal baclofen is an
effective alternative in patients with moderate to severe spas-
ticity—particularly in those poorly responsive to oral medica-
tions. The baclofen is administered via an implanted infusion
pump and delivered through a catheter into the intrathecal
space. Less than one hundredth of the oral dose is typically
required to obtain an acceptable response. Potential complica-
tions include pump malfunction or displacement, kinking of the
catheter, local inflammation, and, in rare cases, spinal menin-
gitis.12
Ice application to the most affected muscles in MS may
result in appreciable, although transient, decrease in spasticity.
Physical therapy (PT), done with modest intensity to avoid
causing excessive fatigue, can help prevent progressive con-
tractures, maintain or increase strength in affected limbs, and
increase endurance. Periodic rest breaks and a cool environ-
ment may help minimize excessive fatigue. Hot tubs or warm
pools can exacerbate symptoms and should be avoided. How-
ever, water exercises with a pool temperature from 81° to 85°F
(27.2°–29.5°C) are typically well tolerated and beneficial for
patients with MS.14Unfortunately, the fluctuating clinical pic-
ture often seen in MS makes it a challenge to determine how
effective the aforementioned options really are.
Bladder dysfunction develops in 80% to 90% of individuals
with MS.15Brainstem lesions or focal demyelination of the
posterior and lateral columns of the spinal cord can result in
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bladder dysfunction.15Depending on the severity and distribu-
tion of urologic involvement, a person with MS may present
with a wide spectrum of symptoms—from complete urinary
retention to persistent urinary incontinence. In fact, the same
person can present with variable bladder dysfunction at differ-
ent times. Some describe bladder dysfunction in MS as failure
to store, failure to empty, or mixed bladder dysfunction.1The
failure to store is usually caused by detrusor hyperreflexia,
whereas the failure to empty is typically caused by detrusor-
sphincter dyssynergia.12A urodynamic study can help identify
the cause and nature of the urologic dysfunction and the most
appropriate treatment course. A complete investigation should
include a voiding history and any history of recurrent urinary
tract infections, stones, hematuria, urologic tumors, or, in men,
prostatic hypertrophy.
The most common urinary dysfunction in persons with MS
is detrusor hyperreflexia,15-17which results in premature blad-
der contractions at lower bladder volumes. Irritative bladder
symptoms, such as urinary urgency, frequency, nocturia, and
intermittent urge incontinence, characterize of this form of
bladder dysfunction. Detrusor hyperreflexia with detrusor-
sphincter dyssynergia is the second most common bladder
dysfunction in patients with MS.15A weak, intermittent urinary
stream, postvoid dribbling, and hesitancy are typical symptoms
of this obstructive form of bladder dysfunction. Measured
postvoid bladder volumes often reveal incomplete bladder
emptying consistent with urinary retention. These measure-
ments can be obtained with postvoid bladder catheterizations or
scans. Incomplete emptying can be caused by persistent
sphincter contraction, weak detrusor muscle contraction, or
both. The least common presentation of bladder dysfunction in
patients with MS is detrusor areflexia caused by a lack of
adequate detrusor muscle contraction. This voiding pattern
typically results in urinary retention or voiding only with
straining.15Most patients with MS, however, eventually de-
velop a small-capacity, hyperreflexic bladder as the disease
progressively worsens.3Untreated, it can significantly increase
bladder pressure and ureterovesicle reflux, with potential for
renal damage. Treatment, therefore, should facilitate complete
bladder emptying and focus on avoiding excessive bladder
pressures. This, in turn, will help protect the upper urinary tract
and minimize the incidence of urinary tract infections (UTIs).
The most effective treatment of urinary retention experi-
enced by patients with MS is typically clean, intermittent
bladder catheterizations. The frequency of bladder catheteriza-
tions per day is scheduled to optimize bladder storage and
bladder emptying. It is generally desirable to maintain bladder
capacity between 300 and 500mL. Severe cognitive deficits or
impaired hand coordination limits a person’s ability to inde-
pendently perform this function.
The effectiveness of clean, intermittent catheterizations can
be enhanced by the simultaneous use of an anticholinergic
medication. Anticholingergic medications, such as propanthe-
line bromide, oxybutynin, and tolerodine, have been success-
fully used to improve bladder capacity, decrease detrusor hy-
perreflexia,andlimiturge
chlorohydrate, a tricyclic antidepressant with both anticholin-
ergic and alpha-stimulating effects, may also reduce detrusor
hyperreflexia.18Hyoscyamine sulfate, which has anticholin-
ergic and antispasmodic effects, has been prescribed to de-
crease irritative symptoms during voiding. It is synergistic with
oxybutynin, and the 2 medications can be used concomitantly
for those patients suboptimally responding to a single agent
alone.18Anticholinergics, however, may cause visual blurring,
nausea, constipation, tachycardia, drowsiness, confusion, and
dry mouth and are contraindicated in patients with narrow-
incontinence.18
Imipramine
angle glaucoma. Of the anticholinergics, tolerodine has the
least anticholinergic side effects.18Alpha-antagonists, such as
terazosin, doxazosin, and tamsulosin, block sympathetic recep-
tors in the smooth muscle of the proximal urethra and bladder
neck. As a result, they decrease sphincter tone and may be
beneficial in patients presenting with bladder outlet obstruc-
tion. However, they can cause adverse effects such as ortho-
static hypotension, dizziness, and lassitude.15In patients who
are experiencing nocturia and have not responded to oxybuty-
nin, desmopressin—a synthetic antidiuretic hormone adminis-
tered by a nasal spray—can be effective. This medication
predictably causes hyponatremia, which is rarely symptomatic.
However, headaches or generalized malaise can herald the
presence of significant hyponatremia when using this medica-
tion, particularly in patients over the age of 65 years.12
Unmyelinated C fibers stimulate the detrusor muscle when
normal spinal pathways are interrupted. Capsaicin has been
found to cause neurotoxic effects on these fibers.12When 1 to
2mL of capsaicin are dissolved in alcohol and administered in
the bladder, detrusor hyperreflexia may be reduced. Repeated
bladder instillations may be required for optimal benefit, but no
long-term side effects have been identified to date.12
Behavioral modification, including attention to the amount
and timing of fluid intake (monitoring overall fluid intake and
limiting fluids before bedtime), voiding at regular intervals
during the waking hours, and avoiding alcohol and caffeinated
drinks, may be helpful. Transvaginal or transanal electric or
vibratory stimulation of the pelvic floor musculature may de-
crease detrusor instability and urinary urgency in selected
patients.15Sacral nerve stimulators designed to modulate spinal
reflex responses have also been used for improving inconti-
nence, with some success.15Rarely, surgical intervention, such
as supravesical urinary diversion, augmentation cystoplasty,
ileal conduit supravesical urinary diversion, ileal cystoplasty,
or transurethral external sphincterotomy in men may be indi-
cated.15Although use of a chronic indwelling Foley catheter or
a percutaneously placed suprapubic tube can cause hematuria,
recurrent UTIs, or local skin irritation and breakdown, they
may be acceptable alternatives in patients who are nonambu-
latory and incapable of independently performing intermittent
bladder catheterizations.15This person would benefit from
postvoid bladder scans and urodynamic testing to identify the
type and severity of her bladder dysfunction. The information
derived would help determine her need for 1 or more of the
aforementioned therapeutic interventions.
Multiple compensatory aids are available for those patients
with MS who develop memory deficits. “To do” and “Re-
minder” lists, and regular use of day planners or calendars,
dictaphones, voice organizers, journals, and palm computers
may help the individual not only to remember key events, but
also to organize and prioritize them more efficiently and effec-
tively. Those who have access to a computer and the knowl-
edge to use it may be able to use selected personal information
manager software to help them sort, file, retrieve, and process
pertinent data in an organized and efficient manner. Some
experience difficulty with both concentration and memory
when reading. The “four R’s” method, which involves the
sequence of reading, rereading, reorganizing, and reviewing,
may enhance an individual’s ability to read and recall key
elements of an article or other written work.14For those who
have mild cognitive and language difficulties, as well as mem-
ory deficits, a speech and language pathologist may be able to
help them improve specific word retrieval by using word as-
sociation techniques and self-cueing strategies.14In this per-
son’s case, using such techniques and electronic aids may help
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her compensate for her memory deficits and may even enhance
her residual memory.
4.4Educational Activity: The patient is concerned about
her ability to continue her job as a teacher. Discuss
potential problems and solutions.
Because of the complex array of impairments, the variable
severity of involvement, and the sporadic nature of the symp-
toms encountered by patients with MS, this condition presents
a significant challenge from a vocational rehabilitation perspec-
tive. A majority of patients are working at the time of their
diagnosis.19However, a majority are unemployed following
their diagnosis.19,20Older age at the time of diagnosis and
lower educational level are associated with worse prognosis for
return to work.19
A number of programs have been implemented to assist
individuals with MS in obtaining jobs. These programs initially
focused on instruction in job seeking and placement assistance.
More recently, alternative programs have focused on empow-
ering these people to modify their work environment, work
schedules, or accommodations and have provided employment
negotiation guidance in hopes of helping them retain their jobs.
These programs have included MS Back to Work or Operation
Job Match, Job Raising Program, Return to Work Program,
Career Possibilities Program, and Project Alliance.19The
Project Alliance program specifically encourages early inter-
action with the employer to address current and anticipated job
accommodations and other reasonable adjustments to allow the
persons to retain their jobs while simultaneously permitting
them to continue productive service.19
Several strategies may assist this patient in maintaining her
vocation as a teacher. The work schedule could be shortened to
address her decreased endurance or modified to allow her to
teach during the hours she is least likely to experience func-
tionally limiting fatigue. Alternatively, she may have the option
of designing a split work schedule or incorporation of rest
periods during the day. She could use an assistive device for
static and dynamic support and for energy conservation. She
could periodically use a stool during her presentations to min-
imize the amount of time spent standing. Because elevated
temperatures may exacerbate the easy fatigability in patients
with MS, avoidance of overly warm work environments should
also help improve work endurance. Cooling vests with gel pads
or circulating coolant may relieve fatigue when warm work
areas cannot be avoided.21
Selected medications may offer some relief of the fatigue in
MS. CNS stimulants such as caffeine and pemoline (18.75–
112.5mg daily) have been used with modest success, although
both may cause adverse effects, including irritability and pal-
pitations.21Amantadine hydrochloride at a dose of 100mg
twice daily can decrease the diurnal fatigue often found in
patients with MS, although it too can cause adverse side effects
such as irritability, insomnia, disagreeable psychologic effects,
and livedo reticularis.21Selegiline, a monoamine oxidase B
inhibitor, and 4-aminopyridine, a potassium channel blocker,
have also reportedly offered some relief of fatigue in MS.21
Memory book aids, personal digital recording devices (eg,
palm computers), and voice-activated software may be bene-
ficial in organizing her schedules more efficiently and in help-
ing with recall of important events and tasks, assuming she
maintains adequate dexterity to operate these devices. Working
in classrooms closer to parking sites and bathrooms may be
helpful in minimizing fatigue and providing ready access to
bathrooms in the event of symptomatic urologic dysfunction.
Appropriate air conditioning would be helpful in addressing the
adverse effects of excessive heat and humidity. Referral to a
state vocational rehabilitation agency may help the person
secure funding for assistive technology when indicated. Provi-
sion of a teaching assistant may be an option to help share the
teaching tasks. Although home-based work options would not
be practical in her case, this may be a viable alternative to
consider in a variety of other occupations. This patient would
also benefit from referral for vocational rehabilitation services
to explore the aforementioned options to maintain her current
employment or to assist her in placement in another related job
that accommodates her impairments. Additional information
regarding work accommodation options can be obtained from
this source.22
In persons with MS, self-appraisal of their functional abili-
ties and limitations, their work motivation, and their optimism
regarding their treatment and future recovery also appear to
play a significant role in the success of their vocational reha-
bilitation, independent of the severity of their impairments and
associated functional limitations. Rosseler,23therefore, en-
dorses the use of the Goldberg scale, which is designed to
assess a patient’s vocational rehabilitation outlook.19,23,24The
crucial point is that people with MS require an individualized
approach to assist them in maintaining their occupation or in
obtaining an alternate job.
4.5 Educational Activity: Several years later, the patient
informs you that she is 2 months pregnant. Discuss
special considerations during pregnancy and chil-
drearing issues.
In women with MS, becoming pregnant is a personal deci-
sion. The literature does not support avoidance of pregnancy
and parenting solely on the basis of the diagnosis of MS.
Therefore, the current consensus is that a woman with MS need
not be discouraged from becoming pregnant.25The decision
should consider the desire of the parents to have and raise
children along with a realistic appraisal of existing and antic-
ipated functional limitations and support system. This specifi-
cally includes the anticipated assistance of the spouse and other
family members or friends.
Although the relapse or exacerbation risk of MS is not
increased during pregnancy and may actually decrease in the
third trimester, it may increase up to 2 to 3 times above the
nonpregnant rate in the postpartum period. Relapses during
pregnancy are typically mild, whereas those occurring in the
postpartum period are more severe.26-28
Relapse or exacerbation of MS typically occurs within the
fourth to eighth week after delivery. As a consequence, it may
be wise for a pregnant woman with MS who works to have a
longer leave time than the typical 4 to 6 weeks, if the option is
available. This would permit additional time for the patient to
recover while still allowing her to provide ongoing care for her
child.25Also, the father, if employed, may wish to consider
taking time off from work under the Family Medical Leave
Act. This would permit him to assist actively the mother at a
time when she may be experiencing increased fatigue and a
possible flare-up in symptoms during the postpartum period. In
the latter period, careful planning of the child’s care on a daily
basis and energy conservation measures should help address
the mother’s easy fatigability.
Daughters of mothers with MS have a 5% risk of developing
MS, which represents a 50-fold increase over the general
population.25However, women with MS who choose to be-
come pregnant do not experience greater disability than other
women with MS who never become pregnant. Also, the course
of the pregnancy or the labor does not appear to be significantly
affected by the underlying MS. At times, hip adductor or flexor
spasticity may make vaginal deliveries more challenging. De-
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liveries under such circumstances, however, are typically man-
ageable with use of epidural anesthesia and assisted deliveries
with forceps or vacuum devices. General anesthesia, when
indicated, is also considered a reasonable and safe option for
patients with MS. There is no evidence of threat to the fetus by
MS. Also, there is no literature to support an increase in the
frequency of miscarriages in patients with MS.25
Among the medications used to treat MS, the immunosup-
pressive medications, in particular, have significant adverse
effects, including potential harm to the fetus. Methotrexate is
contraindicated because of its high potential for fetal harm.28
Azathioprine and cladribine are also known to cause fetal
harm.28Interferon-?, baclofen, amantadine, tizanidine, and car-
bamazepine have all caused adverse effects in animal studies.28
Copolymer, pemoline, and oxybutynin do not appear to cause
harm in animals, but their safety in humans has not been
formally clarified to date.28Therefore, not only are these med-
ications not recommended during pregnancy, they are also not
recommended for a woman who breastfeeds because they may
be secreted in breast milk. A woman who chooses not to
breastfeed can safely resume any of the latter medications after
delivery of her child.
Steroid medications are often used to treat exacerbations of
MS, even during pregnancy. Although there may be some
potential for danger to the fetus in doing so, selected patients
with MS may require continuation of the steroids during preg-
nancy. If done, particular attention to providing the steroids at
a high dose during the labor process may be indicated because
the stress on the adrenal gland at that time may exceed the
intrinsic capacity for the adrenal gland to respond.25
Depending on the severity of functional disability in a
mother with MS, childrearing can be a significant challenge or
even prohibitive if anticipated support is limited or absent.
Although mothers with MS in general are not discouraged from
having and raising children, careful assessment of the mother’s
current and anticipated functional status, her available re-
sources, and her expected support should help guide whether
she will be capable of fully or partially participating in the
childrearing process. The variability of the disease course,
however, makes it difficult to accurately predict a woman’s
long-term functional status, including her childrearing capabil-
ities.
4.6Educational Activity: Five years later, the patient has
an exacerbation and is now a short distance ambula-
tor and is having difficulty with activities of daily
living. Discuss adaptive equipment or assistive devices
that may be beneficial.
This person has developed spasticity, visual impairment,
memory loss, bladder incontinence, and both sensory and mo-
tor deficits. Her ambulation is now limited to short distances.
Mobility problems are among the most common complaints of
patients with MS. In a National Multiple Sclerosis Society
survey, 60% of patients reported the need for some assistance
with mobility.29The goal of rehabilitation treatment in MS is to
preserve functional independence and to maintain or enhance
mobility, while increasing safety and reducing energy expen-
diture. Prescription of a proper wheelchair (manual or motor-
ized) or scooter allows the patient to maintain or even enhance
his/her functional independence. Persons who do not have the
strength or endurance to propel a manual wheelchair usually
need a motorized wheelchair or scooter.30This assistive device
may be particularly applicable for persons with MS because
early fatigability is quite common in this disorder.
Safety precautions are a priority to prevent falls. A thorough
evaluation of her strength, muscle tone, balance, coordination,
and sensory perception helps to determine if she will require an
orthosis or assistive device for safe ambulation. If weakness or
spasticity causes her to trip frequently during ambulation, she
may benefit from an ankle-foot orthosis (AFO). Plastic AFOs
are the most commonly used AFOs because of their low cost,
cosmetic acceptability, light weight, interchangeability among
shoes, and ability to neutrally align and support the ankle and
foot, particularly if custom molded. Double-metal upright
AFOs may be appropriate when in people cannot tolerate
plastic orthotic inserts. In selected patients who have some
volitional ankle plantarflexion and dorsiflexion, the plastic
AFO should have a hinged ankle joint to allow full or partial
ankle motion because it facilitates a more natural gait. Addition
of a dorsiflexion assist (if there is weakness of ankle dorsiflex-
ors) or plantarflexion stop (if there is spasticity of ankle plan-
tarflexors) may result in a more secure and stable gait pattern.31
Ambulation aids represent extensions of the upper limb and
are technically upper-limb orthoses.31One of the purposes of
using ambulation aids is to increase the base of support and
stability. Ambulation aids improve balance, redistribute and
extend the weight-bearing area, reduce lower-limb pain, pro-
vide small propulsive forces, and offer sensory feedback.31
This person’s sensory impairment involves her right arm and
hand. As a result, she may not be able to use a cane in the right
hand safely. She could use a cane in the left hand, but this may
not be the most functionally useful hand in which to hold the
cane. Axillary crutches would require weight bearing through
the hands and axillary areas. Although useful in some, others
may experience difficulty coordinating use of the crutches
because of motor incoordination and spasticity. A forearm
orthosis may be more beneficial because weight would be
borne by both the hand and forearm, thus offering added
stability and proprioceptive feedback. A walker with a forearm
platform on the right may also be beneficial, depending on her
motor coordination and whether her home could accommodate
it. Physical therapists can, when requested, try various assistive
devices with patients to help clarify whether they can tolerate
and practically use the devices before one actually orders them.
Therapists also provide training on the devices when it is
identified that patients are capable of using them.
This person is also having difficulty performing her activities
of daily living (ADLs). Ideally, an occupational therapy (OT)
evaluation should be performed with her in her home. If this is
not possible, the patient should have an outpatient OT evalu-
ation. Depending on which activities she is having difficulty
performing, different types of adaptive equipment are available
to assist her. If she has difficulty eating because of impaired
hand coordination or weakness, she may benefit from adaptive
utensils to improve her ability to pick up and direct food to her
mouth, a scoop dish or plate to allow easier pickup of food, or
an adaptive cup to minimize potential for spills. If meal prep-
aration is an issue, there are adaptive utensils and cutting
boards, and she might benefit from cooking in a seated posi-
tion, either from a stool or from her wheelchair. This may
require home modifications to make counter or stove surfaces
accessible and more visible from a wheelchair.
If dressing is difficult, she may benefit from fastener adap-
tations such as a button aid, zipper pull, and elastic laces.
Loops can be sewn to her socks to ease donning, and Velcro
used to close her pants to facilitate ease when toileting. Shirts
can remain buttoned except for the top buttons. This will
permit the patient to slip the shirt over her head. Another
technique is to remove the buttons from a shirt or blouse, sew
the buttonholes closed, and reattach the buttons on top of the
buttonholes. Velcro pieces are then sewn behind the former
buttonholes and on the original button sites, which permits easy
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NEURODEGENERATIVE DISORDERS, Bryant
Arch Phys Med Rehabil Vol 85, Suppl 1, March 2004