Occupational Therapy In Health Care, 27(3):228–237, 2013


C 2013 by Informa Healthcare USA, Inc.
Available online at http://informahealthcare.com/othc
DOI: 10.3109/07380577.2012.755277

The Utilization of Occupational Therapy Services

for Persons with Charcot-Marie-Tooth Disease

Maria Matyjasik-Liggett, MS, OTD, OTR/L1 & Peggy Wittman, EdD, OT/L, FAOTA2
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1
University of St. Augustine for Health Sciences, St. Augustine, Florida, USA, 2 Eastern
Kentucky University, Richmond, Kentucky, USA

ABSTRACT. The purpose of this study was to investigate the utilization of occupa-
tional therapy services in persons with Charcot-Marie-Tooth (CMT) disease, to better
understand if services are being provided, the types of interventions being provided,
and satisfaction of services. Using survey methods, an online questionnaire was made
available to persons with CMT listed on association websites worldwide. One hundred
and fifteen persons with CMT, residing in all geographical regions within the United
States and seven other countries participated in the study. Around half of all partici-
For personal use only.

pants had received occupational therapy services. Twelve interventions were identified
as being provided for persons with CMT. Although the majority of individuals receiv-
ing occupational therapy services did not feel like it improved their quality of life, the
majority of individuals would recommend occupational therapy services to others and
would return to an occupational therapist. Of those participants not receiving occupa-
tional therapy, the majority felt they would benefit from occupational therapy services.
The results suggest that occupational therapy services are not being well utilized for this
population. A low response rate limits generalization, thus further research is necessary.

KEYWORDS. Charcot-Marie-Tooth, occupational therapy

Charcot-Marie-Tooth (CMT) disease is a commonly inherited peripheral neuropa-

thy, affecting one in every 2500 people worldwide (Jani-Acsadi, Krajewski, & Shy,
2008). CMT involves the demyelination of the peripheral nerves in a stocking-glove
distribution due to the mutation of genes that encode nerve proteins. Without intact
axons and myelin sheaths, peripheral nerve cells are unable to communicate with
muscles, or relay sensory information from the periphery back to the central ner-
vous system (Pareyson & Marchesi, 2009). Symptoms commonly found include pe-
ripheral weakness, atrophy, sensory loss, decreased fine motor coordination, hand
tremors, muscle cramps, high arches, hammer toes, restless leg syndrome, scolio-
sis, intrinsic hand muscle weakness, wrist drop, and hip dysplasia. Less common
symptoms include vocal cord paralysis and phrenic nerve involvement, affecting
respiratory function (National Institute of Neurological Disorders and Stroke,

Address correspondence to: Maria Matyjasik-Liggett, 16817 Oak Preserve Drive, Jacksonville, FL 32226,
USA (E-mail: matyjm04@highpoint.edu)
(Received 22 October 2012; accepted 29 November 2012)

228
 The Utilization of Occupational Therapy Services 229

2011). Miller, Williams, Slack, & Nappi (1991) found that the most common com-
plaint was motor deficits, followed by loss of strength, and decreased dexterity.
Hand weakness has been found to be the most significant factor affecting qual-
ity of life in adults with CMT (Redmond, Burns, & Ouvrier, 2008). Two-thirds of
individuals demonstrate hand symptoms, with the majority of strength deteriorat-
ing within the first 5 to 10 years following the onset of the disease (Kuhlenbaumer,
Stogbauer, Ringelstein, & Young, 2005). These symptoms combined, can lead to
great deficits in occupational performance and quality of life.
Diagnosis usually occurs by identifying familial history as well as symptom pre-
sentation. Genetic testing can identify 80% of persons with the disease, however,
due to the cost, it is not commonly used for diagnosis of the 26 known types (Athena
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Diagnostics, 2008). Onset of symptoms can begin as early as infancy to as late as the
4th decade of life depending on the type of CMT. There is no gender predilection
with this disease. The symptoms and characteristics vary from person to person,
even between family members diagnosed with the same type of CMT (National In-
stitute of Neurological Disorders and Stroke, 2011). An occupational therapist can
play an essential role in improving the lives of persons with CMT. In early stages
of the disease an occupational therapist can assist in slowing symptom progression
by providing services to help maintain strength and hand function. As the disease
progresses, individuals with CMT require modified techniques to complete activi-
ties of daily living, maintain their jobs, and complete instrumental activities of daily
For personal use only.

living. Many individuals struggle with simple tasks such as opening jars or bottles,
turning keys, typing, or holding a pen or pencil. In later stages it is the role of an
occupational therapist to provide compensatory techniques for these individuals
to maintain a quality of life (Charcot-Marie-Tooth Association, 2008). Vinci et al.
(2005) found that quality of life scores for persons with CMT, taking the Outcome
Study Short Form-36 (McHorney, Ware, & Raczek, 1994), were lower than those
for healthy subjects, and within the CMT population, women scored lower than
men, and nonworking individuals scored lower than working individuals.
Persons with CMT, at all stages, present with decreased function on multiple
hand function tests including the Jamar dynamometer, tip to tip pinch, lateral pinch,
three point pinch, and the Purdue pegboard (Miller et al., 1991). Reliable evaluation
tools that can be used with persons with CMT include the 9-hole peg test for fin-
ger dexterity, the Jamar dynamometer for grip strength, the 3 point pinch for finger
strength (Solari, Laura, Salsano, Radice, & Pareyson, 2008), the Semmes-Weinstein
for sensation (Schreuders, Selles, van Ginneken, Janssen, & Stam, 2008), and the
Rotterdam Intrinsic Hand Myometer with detailed measurement of the intrinsic
hand muscles correlating with fine motor skills (Selles, van Ginneken, Schreuders,
Janssen, & Stam, 2006). Outcome measures can be deemed reliable in this popula-
tion as long as the evaluation tool used is sensitive enough to notice small variations
over large amounts of time (Solari et. al, 2008).
Occupational therapists can play a key role in helping this population by pro-
viding home exercise programs, splints to prevent claw hand deformity or support
for wrist drop, adaptive equipment, education on energy conservation and work
simplification, and home modification recommendations (Charcot-Marie-Tooth
Association, 2008). Chetlin, Gutmann, Tarnopolsky, Ullrich, & Yeater (2004)
found that a 12 week modified resistance home exercise program significantly
 230 M. Matyjasik-Liggett and P. Wittman

improved strength and performance of activities of daily living. Another study

looked at the likelihood of overwork weakness in persons with CMT and found
that all participants had greater weakness in their dominant hand compared to
their non-dominant hand which may be a result of overwork weakness from use in
daily activities (Vinci, Esposito, Perelli, Antenor, & Thomas, 2003). In regards to
splinting, a thumb opposition splint was found to increase pinch strength between
the thumb and fingers and decrease claw hand deformity, resulting in a significant
improvement in manual dexterity during the Sollerman Hand Function Test
(Videler, Beelen, van Schai, de Visser, & Nollet, 2008), and increased perceived
functioning during activities of daily living and satisfaction with occupational
performance, using the Canadian Occupational Performance Measure (Videler,
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Eijffinger, Nollet, & Beelen, 2012).

Other medical treatments for CMT focus on surgical treatment of all extremities
with tendon transfers (Pareyson & Marchesi, 2009). Prior to performing tendon
transfers for the hands, an evaluation of nerve conduction velocities, opposition,
pinch strength, and clawing is necessary to ensure the individual is appropriate. The
goal of tendon transfers in the upper extremities is to decrease deformity, increase
pinch strength, and improve opposition to increase overall hand function (Wood,
Huene, & Nguyen, 1995). Physical therapy research has focused on interval train-
ing and functional capacities (Mhandi et al., 2008), foot ailments that affect stance
and gait (Vinci, & Perelli, 2002), and stabilization training for balance and motor
For personal use only.

patterns (Kobesova et al., 2012).

Research on CMT has expanded over recent years to include treatments and
interventions, (e.g. rehabilitation and surgical treatment), symptoms of CMT from
various discipline perspectives, the risks and benefits of interventions, as well as
reliability of outcome measures with a variety of evaluations. Currently, however,
there is no literature that reports how individuals view their occupational therapy
interventions or how many individuals are receiving this necessary care. Although
a utilization rate of 38.2% in aging individuals has been found with multiple scle-
rosis (Finlayson, Garcia, & Cho, 2008), occupational therapy utilization in other
progressive diseases such as amyotrophic lateral sclerosis and Parkinson’s disease
is lacking. Therefore, it is suspected that occupational therapy is not being appropri-
ately utilized in persons with CMT, and those individuals who have or are receiving
occupational therapy services are receiving them too late in the disease process,
thus, feeling that there are no benefits to occupational therapy services. Hence, the
aim of this research study is to determine if occupational therapy is being appro-
priately utilized. More specifically, the goal was to examine if and when persons
with CMT are receiving occupational therapy, what types of interventions are be-
ing used, if those interventions are affecting the individual’s overall quality of life,
and if they are satisfied or would return to occupational therapy, if needed.

METHODS

Design
A survey was chosen as the research method due to low cost, time constraints,
standardization, simple analysis, and availability of resources which are known
advantages of a questionnaire (Ballinger & Davey, 1998). The questionnaire was
 The Utilization of Occupational Therapy Services 231

reviewed with an occupational therapy advisory board member from the Charcot-
Marie-Tooth Association and altered to include more common language and first
person questions. Participants were found using association websites for those with
CMT. The questionnaire was made available, online to over 1000 persons with
CMT, residing anywhere in the world. Participants were allowed to have assistance
in answering questions if symptoms were too severe for computer responses and
parents were allowed to complete the questionnaire for their children, if applica-
ble. Study procedures were approved by the Institutional Review Board at the Uni-
versity of St. Augustine for Health Sciences. Informed consent was received by all
participants by clicking on the online survey link after reading the consent form.
There were no conflicts of interests, outside funding, sponsors, or editorial assis-
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tance for this research study.

Participants
All participants were required to have a formal diagnosis of CMT. The only exclu-
sion criterion was that the survey had to be complete. Participants were encouraged
to complete the questionnaire whether they had or had not received occupational
therapy services previously.
For personal use only.

Instrument
The questionnaire was designed specifically for this study and was reviewed by
faculty and an occupational therapist on the advisory board for the Charcot-
Marie-Tooth Association. The questionnaire included branching, meaning that the
remaining questions changed based on the participants’ response to whether they
have or have not received occupational therapy services. Many questions allowed
marking of all answers that were relevant to the participant. The purpose of the
questionnaire was to provide insight to whether those persons with CMT requiring
occupational therapy are receiving services, are they receiving occupational ther-
apy at the appropriate stage of the disease process, as well as if these individuals
are satisfied with the interventions received.
The questionnaire consisted of 14 questions split into three sections. Section one
consisted of demographic information including age, gender, age at diagnosis, and
symptoms related to upper extremity function and ADL participation. Section two
consisted of questions focusing on the participant’s awareness of the services the
occupational therapists provide, if they had received occupational therapy services,
when in the disease process were services received, and what types of interventions
were used. Section three varied based on responses in section two. For those who
have received occupational therapy services, a Likert scale was used to determine
subject satisfaction with the interventions used, if they would return to an occupa-
tional therapist if needed, and if they would recommend occupational therapy to
others. For those who have not received occupational therapy services, questions
were asked in regards to whether the individual felt like they needed any of the
services provided by occupational therapists. A place for comments was included
at the end of the questionnaire as well as a place to inquire about more information
on occupational therapy services.
 232 M. Matyjasik-Liggett and P. Wittman

Data Analysis
Data was collected from September 15th to October 10th , 2012. A survey reminder
was sent out half way through the collection process. Descriptive statistical analysis
was completed using Microsoft Excel and the survey website.

RESULTS
Of the 124 responses, 115 were used for data analysis constituting an 11.5% re-
sponse rate. Nine questionnaires were not complete beyond section one, thus were
excluded during analysis. Participants represented 31 of the 50 United States (101
of the participants), and 7 additional countries including Australia, Canada, Ger-
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many, India, South Africa, Sudan, and the United Kingdom (14 of the participants).
Gender demographics included 63.5% female and 36.5% male respondents. The
participants’ current ages ranged from 4–84 years old, with an average age of 48.
Age at diagnosis of CMT ranged from 2–70 years old, with an average age of 33.
Data regarding knowledge of the profession of occupational therapy resulted in
96.5% of participants having heard of the profession, and 3.5% having never heard
of the profession.
The data regarding symptom presentation in participants is shown in Figure 1.
Hand weakness was the most common symptom reported (94.4%), followed by
difficulty opening containers/turning keys (87%), decreased sensation in fingers or
For personal use only.

hands (73.2%), difficulty writing or typing (66.7%), arm weakness (65.7%), and
finger and hand deformities (28.7%).
Data regarding occupational therapy services includes 47.8% of participants
having received occupational therapy services, and 52.2% of the participants
never having received occupational therapy services. Of the 60 respondents who
reported they have never received occupational therapy services, 75.9% reported
they feel they would benefit from the services, and 24.1% reported they do not feel
they require occupational therapy services. Of the respondents who reported that
they felt they would benefit from occupational therapy services, the most common

FIGURE 1. I experience the following symptoms as a result of CMT.

 The Utilization of Occupational Therapy Services 233

reported reasons for why they are not receiving services include, their doctor has
not mentioned anything about occupational therapy or the services they provide
(17.7%); they are unable to find a good therapist or a therapist that knows about
CMT (13.3%); insurance related issues (11.1%); and they have found how to adapt
on their own (8.8%).
The types of services provided by occupational therapists for the participants
included a home exercise program for hand strengthening (58.8%), adaptive equip-
ment (35.3%), hand splints (33.3%), home exercise program for arm strengthening
(29.4%), home modification recommendations (29.4%), education on energy
conservation or work simplification (23.5%), and home safety evaluation (15.7%).
Forty five percent reported other interventions including power wheelchair fitting,
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driver rehabilitation, occupational training, range of motion exercises, and home

management techniques with not all the participants identifying specifically.
Of the 55 participants who reported they have received occupational therapy
services, the range of when those services were received was currently to 20 years
ago, with an average of 3 years ago. Of those respondents, 14.2% are currently re-
ceiving occupational therapy services, and 26.5% have ceased the services within
the past year. When asked why occupational therapy services were ceased, 28.9%
reported that all their goals were met, 28.9% indicated insurance only paid for a
certain number of treatments, and 17.8% were not satisfied with the services they
were receiving. Forty four percent reported other or that they were dissatisfied with
For personal use only.

the services, with 14.5% specifically stating it was related to the fact that the oc-
cupational therapist was unfamiliar with CMT, not educated on current research
regarding treatment, or thought a person with CMT would make a full recovery.
The other most common responses by those reporting ‘other’ included that they
felt like there was no change (9%), and the cost of services was too high (7.2%).
A Likert scale was used to determine the level of satisfaction with the occupa-
tional therapy services received (see Figure 2). In response to feeling as if occupa-
tional therapy improved overall quality of life, 46.2% strongly agreed or agreed,

FIGURE 2. Satisfaction with Occupational Therapy Services.

 234 M. Matyjasik-Liggett and P. Wittman

36.5% were neutral, and 17.3% disagreed or strongly disagreed. In response to if

the participant would recommend occupational therapy to others, 73.6% agreed
or strongly agreed, 22.6% were neutral, and 3.8% disagreed or strongly disagreed.
When asked about returning to an occupational therapist if symptoms progressed
further, 69.8% agreed or strongly agreed, 18.9% were neutral, and 11.3% disagreed
or strongly disagreed.

DISCUSSION
The results of this survey raise interesting points to discuss further. First, for this
population, occupational therapy services may be received too late to slow symp-
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tom progression but are appropriate in providing compensatory strategies. Second,

education of health professionals, consumers, and occupational therapists is essen-
tial to ensure that patients are receiving the most appropriate services at the most
appropriate time. Additionally, occupational therapists need to remain at the high-
est level of competency when it comes to continuing education and interventions
being provided to patients. Although participants did not feel that occupational
therapy improved their overall quality of life, they did feel that techniques were
provided to assist with daily activities.
In this survey of persons with CMT, the majority (94.4%) reported hand weak-
ness as a symptom that affected activities such as opening containers, turning keys,
For personal use only.

typing, and writing. However, when comparing hand weakness to interventions re-
ceived by an occupational therapist, only just over half of those individuals who
received occupational therapy were provided with a home exercise program for
hand strengthening, and only one-third were provided with adaptive equipment.
This may be due to the fact that symptoms were too advanced and hand strength-
ening was no longer an appropriate intervention. However, this would not explain
why compensatory techniques were not being provided. When looking at average
ages, the average current age of the participants was 48 years, the average age at
diagnosis of CMT was 33 years, and the average time since receiving occupational
therapy services was 3 years ago. Thus, on average, participants received occupa-
tional therapy services 12 years after diagnosis. This is significant since the research
indicates that the majority of strength deteriorates within the first 5 to 10 years af-
ter the onset of the disease (Kuhlenbaumer et al., 2005); thus, it is essential that
individuals are provided with services soon after diagnosis to slow symptom pro-
gression.
Almost all participants reported that they were aware of the profession of
occupational therapy, however, only about half of the participants had seen an
occupational therapist. Interestingly, of the half not receiving occupational therapy,
three-quarters reported they thought they would benefit from occupational ther-
apy services. When asked why they felt they had not received services, the majority
of respondents reported that their primary care physician had not recommended
it or is unaware of the interventions provided by occupational therapists. Based on
this finding, it would appear that it is important to not only educate consumers but
also other health care professionals to ensure that patients are receiving the most
appropriate care from the most qualified individual. Education for consumers
might be done via support groups and websites. Education for health professionals,
 The Utilization of Occupational Therapy Services 235

including occupational therapists, should be imparted during their academic

curricula, as well as during continuing education for practitioners. Education may
also play a key role when looking at the one-quarter of respondents who had
not received occupational therapy and did not feel that they would benefit from
it, even though all respondents reported at least one symptom that is within the
occupational therapy scope of practice.
In regards to the cessation of occupational therapy services, just over one-quarter
of respondents reported they had met all their goals or they were limited by the
number of insurance payments. The majority of the respondents reported ‘other’,
with the majority indicating the reason as their treating occupational therapist. Par-
ticipants reported that their occupational therapist was unfamiliar with CMT, was
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not up to date on current research, or thought they would make a full recovery,
which was a stunning result. As an occupational therapist it is essential to be knowl-
edgeable about the diagnosis and treatment for conditions that may be unfamiliar.
Treatment of a recoverable disease is extremely different from that of a progressive
disease when considering remedial versus compensatory strategies for both inter-
ventions and patient education. It is key to remain up to date on current research
or investigate successful treatment interventions. Not only are patients not receiv-
ing the services they have the right to get, occupational therapy’s reputation as a
profession can be damaged if patients perceive their therapist as incompetent.
The last important finding is related to satisfaction with occupational therapy ser-
For personal use only.

vices. Although over half of all respondents who have received occupational ther-
apy services were neutral, or disagreed that occupational therapy improved their
overall quality of life, three-quarters of these participants reported they would rec-
ommend occupational therapy to others and would return to an occupational ther-
apist if their symptoms progressed further. The majority of individuals receiving
occupational therapy services are able to see the benefits even if they do not feel
that their quality of life has improved. Thus, although quality of life is seen as an
important outcome of occupational therapy services, it is also important to realize
that subjects in this study believed they received help with everyday life activities
from their involvement with occupational therapy.
Further research is clearly needed. It is possible that individuals with CMT are
not receiving services at the appropriate time, as exemplified by the hand function
findings. However, this finding is not conclusive due to the sample size. Research
on the outcomes of specific interventions used in this population would be bene-
ficial. It can also not be concluded that individuals feel that they did not benefit
from occupational therapy. Even though some individuals reported they were not
satisfied or did not see results from the services provided, the majority of the indi-
viduals who have received occupational therapy services would recommend them
to others. Further research should also be geared towards CMT awareness among
health professionals, especially in the area of rehabilitation.

LIMITATIONS
The low response rate presents the greatest limitation of this study. Even though
participants were located in a variety of states and countries, it is difficult to gener-
alize the findings to the occupational therapy profession and to persons with CMT
 236 M. Matyjasik-Liggett and P. Wittman

as a whole. A low response rate is the greatest disadvantage of using a questionnaire

for research. The low response rate may have be due to the fact that the survey was
online and many individuals with CMT in later stages have difficulty typing, as well
as the fact that not all individuals have computer access on a regular basis. Further
research using questionnaires with this population should include options for in
person or phone responses. Length of time the questionnaire was available online
and knowledge of occupational therapy prior to the study might have also led to a
lower response rate.
The results may also be biased in that participants for the study were found on
websites geared towards those looking for more information about CMT and treat-
ments for it. Thus, results may overestimate the number of individuals with CMT
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actually receiving occupational therapy services or having more difficulty than oth-
ers with CMT.

CONCLUSION
Based on the results of this study, occupational therapy would appear to be not well
utilized for people with CMT. Since results of those individuals not receiving occu-
pational therapy services reported that their primary care physicians were unaware
of the services provided by occupational therapists, further research should look
For personal use only.

at the awareness of occupational therapy among other medical professionals. The

greatest area of need, however, appears to be education for occupational therapists,
not just regarding CMT, but the treatment of all progressive neurological diseases
and the importance of staying current on new interventions, being creative when
treating patients, and self-education on unfamiliar diagnoses patients present with.

ACKNOWLEDGEMENTS
The authors thank the participants who took time to complete the questionnaire.
The authors would also like to thank Susan Salzberg on the advisory board for the
Charcot-Marie-Tooth Association for her valuable insight.

Declaration of interest: The authors report no conflict of interest. The authors

alone are responsible for the content and writing of this paper.

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