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Focal Hand Dystonia Affecting Musicians.

Part I: An Overview Of
Epidemiology, PathoPhysiology And Medical Treatments

Focal Hand Dystonia Affecting Musicians. Part I:


An Overview Of Epidemiology, PathoPhysiology And
Medical Treatments
Katherine Butler BAp(Sc) Occupational Therapy, AHT (BAHT), A MusA(Flute) The Princess Grace Hospital, London, UK
Dr Karin Rosenkranz MD Sobell Department, Institute of Neurology, Queen’s Square, London, UK

In 1911, Oppenheim coined the term ‘dystonia’ to describe disordered motor


control, characterised by an association of hypotonia and tonic muscle spasm.
Focal hand dystonia is one form of this disorder, in which symptoms are often
task-specific and occur during skilled movements such as writing (writer’s
cramp) or playing a musical instrument (musician’s cramp). Much research
has been conducted on the pathophysiology of dystonia, but the underlying
mechanisms still remain unclear. Hypotheseses about functional central
nervous system alterations continue to gain more support. Scientific treatment-
based publications on focal dystonia are sparse, and progress in evidence-
based treatment options are necessary in order to assist this patient group.
This paper will review the literature, documenting dystonia classification
criteria, manifestations, pathophysiology and medical treatment techniques for
musicians affected by focal hand dystonia.

INTRODUCTION

ystonia is a syndrome legs, trunk, neck, face, eyelids or vocal


D characterised by involuntary, cords (Berardelli et al 1998; Deuschl and
prolonged muscle contractions that Hallett 1998). Focal dystonia tends to
can lead to sustained twisting postures be named according to the affected
region: such as blepharospasm (eyelids), Figures 1a and 1b: Task-specific,
(Fahn 1998, Fahn et al 1998, Fahn et al 1987).
spastic torticollis (cervical dystonia), action-induced Focal Hand Dystonia
Three criteria can be utilised to assist in
lingual dystonia, spastic dystonia and has different forms, including writer’s
classifying this syndrome: age at onset,
oromandibular dystonia. This paper will cramp and musician’s dystonia (hand
aetiology and distribution of symptoms
focus on focal hand dystonia, a late- and embouchure)
(Fahn et al 1987 and 1998). An onset before
28 years of age is classified as early onset primary dystonia that is often
onset dystonia, and after this age as late- task-specific and includes writer’s or fourth and fifth fingers of the right
onset dystonia. Aetiology can be divided musician’s cramp. The symptoms may hand, whereas guitarists often show
into either idiopathic (no obvious effects affect a single finger up to the hand/ a curling in of the third finger of the
on the brain) or symptomatic (often the wrist; they do not tend to generalise and right hand. Flautists tend to be affected
basal ganglia are affected, resulting in remain fairly task-specific (Bressman et al in the left hand, whereas violinists
more generalised symptoms). Some 1998) (Figures 1a and 1b). and clarinettists can have involuntary
literature uses the term primary or contractions in either hand (Altenmüller
secondary for the same classification 1998; Brandfonbrener 1995; Hochberg et al
(Fahn 1998). Thirdly, dystonia can be FOCAL HAND DYSTONIA IN 1983). Although focal dystonia manifests
classified according to its distribution of MUSICIANS as a motor problem, interestingly this
symptom manifestation: in generalised can be influenced by a ‘sensory trick’ (eg
dystonia, symptoms may manifest in Musicians with focal hand dystonia playing the musical instrument while
all extremities including the trunk; in display varying symptoms which tend to wearing a latex glove). Many entities
hemidystonia, the symptoms are focused be specific to that individual person and appear to ‘trigger’ the manifestation of
on one side of the body; segmental can include incoordination, cramping focal hand dystonia in musicians, such as
dystonia affects a segment of the body or and tremor (Jankovic and Shale 1989). The a sudden increase in playing or practice
adjacent body parts; and focal dystonia symptoms musicians display depend time; a dramatic change in technique; a
affects a single body part. upon the instrument played, rather than return to studies after a long break from
Any part of the body can be affected hand dominance. Dystonia in pianists the instrument; a trauma (not necessarily
by focal dystonia, including the arms, often manifests as a curling in of the recent); current or a history of nerve

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Focal Hand Dystonia Affecting Musicians. Part I: An Overview Of
Epidemiology, PathoPhysiology And Medical Treatments

entrapment; psychological trauma and b; Leijnse et al 1992 and 1993; Wilson et


or a change of instrument al 1993). However, these studies do not
(Brandfonbrener 1995). focus specifically on musician’s cramp
but also include other types of focal
hand dystonia, which may influence
EPIDEMIOLOGY the findings.

Dystonia in musicians might be


considered as a more common problem FOCAL HAND DYSTONIA IN
than is often realised. The prevelance MUSICIANS – PATHOPHYSIOLOGY
among professional musicians can
be estimated as 2-10% (Jabusch 2006; It is thought that focal task-specific
Branfonbrener 1995; Lim et al 2001), which is hand dystonia develops due to a
higher than that of writer’s cramp (0.1%) functional disturbance at several levels
in the general population (Nutt et al 1988). of the central nervous system. This
This high number certainly reflects the is mainly expressed as an imbalance
specific demands made upon musicians. between inhibition and excitation
More male than female musicians are of neurons. There is evidence for
affected with ratios ranging from 2:1 to alteration in sensory and motor
6:1 (Lederman 1991; Brandfonbrener 1995; cortical representation, which may
Lim et al 2001), with only 6% reporting result in altered integration of sensory
a history of either writer’s cramp or information from the periphery
musician’s dystonia in their families. into adjacent and required motor
A recent survey of 116 patients with focal programmes.
hand dystonia, the largest series to be There follow some of the theories
published, showed that the mean age postulated for the development of
at onset of symptoms is about 33 years this functional disturbance. Due to
(range 17-63 years) (Jabusch 2006). About overlapping, it is difficult to identify if
half the patients were in professional focal hand dystonia is due to a sensory,
soloist positions, 17% were tutti players central or motor disturbance. However,
in orchestras, 17% held teaching the points will be outlined following the
positions and 15% were students. These sensory motor loop to allow a clearer
results showed that classical musicians overview.
are predominately affected by musician’s
focal hand dystonia (95%) with only a
Figures 2a and 2b: Focal Hand Dystonia
minor number of jazz and pop musicians SENSORY ALTERATION
in musicians showing motor
affected. In this series, 28% were
inco-ordination or loss of voluntary
keyboard instrumentalists, 26% were Animal studies showed that repetitive
control in pianist’s and cellist’s right
woodwind players, 20% played plucked performance of a hand gripping
small and ring fingers.
instruments, 15% were bowed string movement can actively degrade cortical
players, and 11% were brass players representation in the primary sensory
(Jabusch 2006). cortex (Byl et al 1996a and b; Wang et al
There are reports of predisposing 1995). The changes are not only at the
medical conditions, which may include: cortical level, but also involve other
ulnar neuropathy (Charness et al 1996; Ross brain regions, which are also involved
et al 1995), local trauma or peripheral in sensory processing (Lenz and Byl
nerve injury (Brandfonbrener 1995) and 1999; Sanger and Merzenich 2000). Similar
biomechanical limitations (Leijnse 1997a alterations have been found with

The British Journal of Hand Therapy Autumn 2006 Vol 11 No 3 PAGE 73


Focal Hand Dystonia Affecting Musicians. Part I: An Overview Of
Epidemiology, PathoPhysiology And Medical Treatments

increasing severity in healthy musicians cramp, also showed an alteration in in musicians. Early onset dystonia,
and musicians with dystonia. An size and location (Thompson et al 1996), which can manifest itself as focal or
increased cortical representation of the suggesting some reorganisation of generalised dystonia, is commonly
left hand in healthy string players (Elbert cortical excitability in dystonia. Ridding attributed to the gene DYT1 (Bressman
et al 1995), and a blurring/overlapping of et al (1995) found that there was less 1998; Bressman et al 1998). A family history
cortical representation in musicians with intracortical inhibition in patients with of movement disorders or writer’s
focal hand dystonia (Elbert et al 1998), echo focal, task-specific primary dystonia cramp has been described in about 10%
the animal study findings. when tested at rest. They proposed that, of musician patients (Altenmüller 1998;
under normal circumstances, one role Hochberg et al 1990). Brandfonbrener
DISTURBANCE OF SENSORIMOTOR of the inhibition was to ‘focus’ the motor (1995) found no positive family history.
INTEGRATION command within the cortex so that the There is no specific genetical study
correct muscles were activated by the on larger samples of musicians with
Animal studies have shown that cortical right amount in any task. A deficiency focal hand dystonia, but one study does
abnormalities lead to deficient motor in this cortical inhibitory system could analyse ten Ashkenazi Jewish patients
preparation (Feve et al 1994; Van der Kamp therefore contribute to the overflow of with focal hand dystonia (eight with
et al 1995; Yazawa et al 1999). The same activity in dystonia. Indeed, reduced musician’s cramp, two with writer’s
phenomenon was displayed in a study excitability of this inhibitory system could cramp) and no common mutation could
of affected musicians. Due to disturbed also account for the increased cortical be found, thus arguing against a genetic
sensory representations, the motor output again noted from above. role in the etiology of musician’s cramp
cortex receives altered information (Gasser et al 1996).
(Rosenkranz et al 2000), and deficiencies In conclusion, it is not clear if sensory
in sensorimotor integration can lead to changes drive motor alteration, or PSYCHOLOGICAL FACTORS
loss of motor control (Abbruzzese et al whether repetitive movements drive
2001; Siebner et al 1999). Both functional sensory changes that lead to motor Once established, the symptoms of
and structural changes occur in any remapping. Due to the close interlinks musician’s cramp can be aggravated
musician’s brain, as they adapt to within the loop, it is difficult to by psychological stress, such as
the demands of their activity. These differentiate where the alteration has performance situations. However,
plastic changes are necessary in order occurred. Functional disturbances of the psychological factors are not thought
to achieve a high performance level; basal ganglia have been found in primary to significantly contribute to the
however it is argued that they may render dystonia (Ceballos-Baumann and Brooks development of dystonia (Sheehy and
the musician susceptible to development 1997 and 1998; Berg et al 2000; Naumann Marsden 1982). There is evidence that
of dysfunctional motor control et al 1998 and 1996), however, no studies blepharospasm may have an association
syndromes such as focal hand dystonia were found that specifically related to with obsessive-compulsive disorder
(Pascual-Leone 2001). musicians in this regard. Musicians (Bihari et al 1992a and b), and thus
appear to be a special group of people psychological status is included as a
MOTOR DISTURBANCE as the tasks they perform are extremely possible risk factor for development of
specific. Therefore, it is likely that, in focal dystonia. It could be argued that
Studies with transcranial magnetic musicians with focal hand dystonia, to be a musician of outstanding ability
stimulation of the motor cortex have higher order motor areas are involved. requires a certain personality in order to
shown that cortical output again seems For an in-depth presentation of the achieve the standard.
to be higher in patients with dystonia, pathophysiology of focal dystonia, see It is thus understandable that musicians
which may contribute to the excessive Lim et al (2001). with focal dystonia often despair when
motor output that occurs during they cannot control their hands to
voluntary movement (Mavroudakis et GENETICS perform tasks that they have practised
al 1995; Ikoma et al 1996). Mapping of for hours everyday. This can, in turn,
cortical sites, from which specific muscle There appears to be conflicting evidence cause development of secondary
responses can be elicited by brain to support a genetic contribution to psychological difficulties.
stimulation in patients with writer’s development of focal hand dystonia
PAGE 74 The British Journal of Hand Therapy Autumn 2006 Vol 11 No 3
Focal Hand Dystonia Affecting Musicians. Part I: An Overview Of
Epidemiology, PathoPhysiology And Medical Treatments

DYSTONIA DIFFERENTIAL into treatment approaches. Progress writer’s cramp, oromandibular and
DIAGNOSIS in scientifically analysed and reported spasmodic dystonia (Sojer et al 2001).
treatment options for this condition are Intramuscular BTX injections weaken
Dystonia can be distinguished from most necessary, in order to offer affected the muscle by reducing the release of
common painful conditions, in which patients evidence-based options. acetylcholine (a neurotransmitter) at the
muscle pain usually continues after neuromuscular junction (Kedlaya et al
task completion, as pain is usually not 1999; Singer and Weiner 1995; Coffield at al
Current treatments include:
evident with focal dystonia. If a patient 1994). BTX injections must be repeated
• oral medications regularly (Marion 1999), as the effects are
is trying to override contractions by
abnormally positioning joints and soft • Botulinum toxin (BTX) injections reversible and may only last one to three
tissues, pain may result (Kember 1997). • surgery months. It is important to administer
Muscle ache may occur after a prolonged • rehabilitative therapies and enough, but not too much BTX, otherwise
spasm. When making a diagnosis of muscle weakness and impairment can
• supportive approaches.
focal hand dystonia, other pathologies occur (Altenmüller 2001; Cole et al 1991 and
such as ganglions, Dupuytren’s disease, 1995; Ross et al 1997).
trigger finger, meningiomas (Wynn Parry Medical-based treatment options will be Musicians who are affected by focal
1998), compression neuropathies and discussed in this article. Rehabilitative dystonia in a single digit are most
compartment syndrome affecting the therapies and supportive approaches are effectively treated by administration
intrinsic muscles of the hand must be the focus of Part II. of BTX (Altenmüller 1998). However, in
excluded (Amadio and Russotti 1990). most musicians, rather more complex
movement patterns than just single
ORAL MEDICATIONS
fingers are impaired. As BTX injections
are limited due to the associated
Oral medications do not cure focal hand
TREATMENTS weakness of nondystonic muscles (Priori
dystonia, but can be used as palliative
et al 2001), and the as yet unknown long-
treatments. Anticholinergic drugs
Current literature that focuses on term effects of these injections on the
influence neurotransmission in the
medical-based treatments for focal hand sensorimotor system, there is a need to
basal ganglia, and can thus be useful
dystonia will be reviewed. Dystonia is develop new treatments for musicians
for treating patients with focal dystonia
difficult to treat (Fahn et al 1987) and is with focal dystonia. Furthermore,
(Altenmüller 1998). Trihexiphenidyl is,
recalcitrant to intervention (Byl and Topp these injections only blur the obvious
at present, the most effective oral
1998). At present there appears to be no symptoms without tackling the origin
medication; however the side effects,
one cure for dystonia and many of the (Cole et al 1991).
even when using small doses, limit its
treatment modalities have significant
long-term use (Altenmüller 1998). The
limitations. It is therefore important SURGERY
side effects can include a dry mouth,
that patients are educated regarding the
fatigue and slight memory impairment.
limitations of treatment (Lim et al 2001). There is controversy surrounding
Dopaminergic medication appears to be
Many years ago, Gowers (1893) observed the use of surgery as a treatment
less effective in treating focal dystonia
that patients with writer’s cramp could technique for patients with focal
than trihexiphenidyl (Muller et al 1996).
benefit from a freer approach to writing, dystonia. Winspur (1998) believes that
and if possible should be encouraged to any surgery (eg tendon transfers and
BOTULINUM TOXIN (BTX)
learn to use the other hand. However, he nerve transpositions) is contradicted
comments that in about half of the cases when treating such patients as it only
BTX type A is the most widely studied
the other hand can become similarly causes further scrambling of disturbed
and utilised treatment of focal hand
affected. The option of swapping hands motor programmes. He believes
dystonia. BTX injections are seen as
is not as convenient for musicians, that attention to technique, change
the treatment of choice for cervical
as they are often required to use two in instrument, resolving mechanical
dystonia and blepharospasm, and
hands to play their instrument. Many problems, and instrument adjustments
good results have been reported for
articles identify a need for investigation should all be considered before surgery.

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Focal Hand Dystonia Affecting Musicians. Part I: An Overview Of
Epidemiology, PathoPhysiology And Medical Treatments

Lozano and Linazasoro (2000) comment symptoms without tackling the origin of in patients with idiopathic spasmodic
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for many patients. Others report no be entered into extremely cautiously by DL (1992b). ‘Blepharospasm and
improvement in focal dystonic symptoms, a senior consultant with experience in obsessive-compulsive disorder’ Journal of
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are released (Charness et al 1996). Thus, rehabilitative approaches are seen
Brandfonbrener AG (1995). ‘Musicians
Marion (1999) states that surgery is as being the treatment options that
with focal dystonia: A report of 58 cases
only indicated in very severe cases should be trialled first with this patient
seen during a ten-year period at a
and requires thorough planning and group, and these will be covered in the
performing arts medicine clinic’ Medical
discussion of indications, while Singer next paper.
Problems of Performing Artists 10(4): 121-127
and Weiner (1995) state that only a
surgeon with extensive training and Bressman SB (1998). ‘Dystonia’ Current
experience in operating on patients with Opinion in Neurology 11: 363-372
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(Dystonia musculorum deformans)’ stimulation of the primary motor relaxation in patients with focal hand
Neurologisches Zentralblatt 30: 1090-1107 cortex in patients with writer’s cramp’ dystonia studied by movement-related
Pascual-Leone A (2001). ‘The brain that Neuroscience Letters 262: 133-136 potentials’ Brain 122: 1357-1366
plays music and is changed by it’ Annals Singer C and Weiner WJ (1995).
of New York Academy of Science 930: 315-29 ‘Primary dystonias current therapeutic Katherine Butler
Priori A, Pesenti A, Cappellari A, recommendations’ CNS Drugs 3(3): 186-193 Clinical Specialist in Hand Therapy
Scarlato G and Barbieri S (2001). ‘Limb Sojer M, Wissel J, Muller J and Poewe 30 Devonshire Street
immobilization for the treatment of focal W (2001). ‘Treatment of focal dystonia in association with The Princess Grace Hospital
occupational dystonia’ Neurology 57(3): with botulinum toxin A’ Wiener Klinische London W1G 6PU
405-409 Wochenschrift Supplement 113(4): 6-10 Tel + 44 20 7908 3660

Ridding MC, Sheean G, Rothwell Thompson ML, Thickbroom GW, Sacco Fax + 44 20 7908 3661

JC, Inzelberg R and Kujirai T (1995). P, Wilson SA, Stell R and Mastaglia FL Email: Katherine.Butler@HCAHealthcare.co.uk

‘Changes in the balance between motor (1996). ‘Changes in the organisation of


cortical excitation and inhibition in the corticomotor projection to the hand © British Association of Hand Therapy Ltd
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59:493-8

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Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

Focal Hand Dystonia Affecting Musicians. Part II:


An Overview Of Current Rehabilitative Treatment
Techniques
Katherine Butler BAp(Sc) Occupational Therapy, AHT (BAHT), A MusA(Flute) The Princess Grace Hospital, London, UK
Dr Karin Rosenkranz MD Sobell Department, Institute of Neurology, Queen’s Square, London, UK

Focal Hand Dystonia is a complex and difficult condition to treat. Many treatment many interlinking techniques that can
techniques have been trialled with musicians who suffer from this condition. be employed to assist in achieving this
Current treatments include: oral medications, Botulinum toxin (BTX) injections, goal. Sensory re-education is a treatment
surgery and rehabilitative therapies. The medical-based treatments have that focuses on sensory discrimination.
been discussed in a prior paper, and thus rehabilitative therapy treatment The treatments that combine both
options and the related clinical implications are the focus of this paper. These sensory and motor aspects of focal
include: sensory re-education, sensory motor retuning, rest, splintage and limb hand dystonia include: sensory motor
immobilisation. Future research areas relating to focal hand dystonia and the retuning, rest, splintage, occupational
musician are highlighted. therapy and physiotherapy and the use
of a multi-disciplinary approach. Another
treatment option is limb immobilisation,
INTRODUCTION which interrupts motor performance
and decreases sensory afferents from
ocal hand dystonia occurs in REHABILITATIVE THERAPIES the limb. Other treatment approaches
F 2-10% of professional musicians that can complement the ones above
and often has devastating consequences Rehabilitative therapies and supportive include: assistive devices, instrument
for their careers. Treating these patients approaches for the treatment of focal modification, Feldenkreis therapy,
is difficult and requires, in almost hand dystonia include: Alexander technique, biofeedback
every case, an individual approach. The therapy, counselling and psychotherapy.
specific presentation of the dystonic • sensory re-education In order for treatment(s) to have any
symptoms, personal circumstances • sensory motor retuning (constraint- success, high patient compliance and
such as psychological involvement induced movement therapy) motivation is required, and associated
and professional circumstances (time • rest and splintage complications need to be reviewed (ie
pressure on recovery due to concert • limb immobilisation the musical instrument must be well
schedule, risk of losing position/job) all • occupational therapy and physiotherapy maintained, and associated medical
need to be considered and must influence • multi-disciplinary team approach conditions such as nerve compression or
therapeutic decisions. There are other • assistive devices/modifications to trigger finger{s} must be ruled out).
pressures that must also be considered. instrument
Often the patient’s expectations of • Feldenkreis therapy/Alexander SENSORY RE-EDUCATION
rehabilitation are unrealistic, in that they technique
are aiming for full recovery so that their • biofeedback therapy The integral role of the somatosensory
performance level is the same as pre- • counselling and psychotherapy. input is emphasised by the fact that a
focal hand dystonia onset. Full recovery reduction in involuntary movements
can only be achieved in certain cases. In Research on focal hand dystonia has and an increase in control can be
most cases, some restriction of motor highlighted a dysfunction of integration gained by performing sensory tricks, for
abilities will remain. Making the most of sensory information from the example playing the musical instrument
of this situation requires collaboration periphery into adequate motor whilst wearing a latex glove (Berardelli
of several disciplines including the commands in the brain. et al 1998; Hallett 1995; Lederman 1991).
medical profession, music teachers, This seems to be especially the case in Recent studies in primates suggest that
instrument makers, hand therapists and, musician’s hand dystonia (Rosenkranz repetitive motions can induce plasticity
on occasion, psychotherapists. The first et al 2005). The aim, when treating a changes in the sensory cortex, which
article outlined the medical treatments musician affected by focal hand dystonia, may degrade the hand representation
for this condition. This article considers is to re-establish integration of sensory and interfere with motor control (Blake et
the different rehabilitative strategies in proprioceptive afferent and cutaneous al 2002; Byl et al 1997, 1996a and b). Through
detail and the use of a multi-disciplinary information into a controlled, appropriate, this research, the possibility of utilising
team approach. adjacent motor command. There are specific sensory training to re-wind

The British Journal of Hand Therapy Autumn 2006 Vol 11 No 3 PAGE 79


Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

sensory dedifferentiation and therefore


successfully treat patients with focal
dystonia is raised. Sensory discrimination
training is thus emphasised during
treatment sessions, and is an integral
part of the home exercise programme
(Byl and McKenzie 2000; Byl et al 2000; Byl
and Topp 1998). The same amount of
repetition may be needed to restore the
hand representation as that which led to
its degradation, thus patient compliance
is integral to the effectiveness of this Figures 1 a and b: Cellist utilising latex glove as a sensory trick
treatment (Byl et al 1996a, b).

The sensory discrimination activities ie visualising healing, imagining normal hand dystonia who displayed changes
include: identifying various textures and sensory processing, motor control, and in cortical topographical representation
temperatures of sensory stimulation on effective target task execution (Byl et al of the hand, clinical somatosensory
the skin; discriminating and matching 2000; Byl and McKenzie 2000; Byl and Topp discrimination and fine motor control
coins, beads, buttons and small animal 1998). after carrying out a comprehensive
figures; identifying matched pairs of rehabilitation programme that
objects in a game or out of a bag of rice, High patient compliance and emphasised sensory retraining, i.e.
beans or noodles; asking the vision- commitment is required to perform specific, repetitive, goal-directed sensory
occluded patient to locate where they are the number of repetitions of sensory activities (Byl et al 2000). Patients with
being touched (Byl and McKenzie 2000); retraining tasks that are necessary to writer’s cramp have displayed similar
vibration sensitivity; backward masking; restore the somatosensory function, and results where, due to continual training
manipulating embossed letters; work in turn improve motor control and stress- through braille reading, a decrease in
on detecting mismatched letters on a free motor movements. It is expected disability levels and an improvement in
keyboard and palpation of directional that patients complete at least one to two spatial discrimination were noted (Zeuner
lines on a cube (Byl and McKenzie 2000; hours of sensory discrimination activities et al 2002).
Byl and Topp 1998). To facilitate normal at home each day (Byl and McKenzie 2000).
sensation and perception and reinforce
normal hand functioning, patients are This treatment approach is supported
asked to spend time at home meditating, by a case report of a flautist with focal

Figures 2a, b and c: Sensory discrimination activities can include: identifying sensory stimulation, discriminating and matching
common household items (a, b) and manipulating embossed items such as dominoes (c).

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Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

REST AND SPLINTAGE

Rest has been considered a therapeutic


approach for the treatment of dystonia
for more than 100 years (Gowers 1893;
Poore 1887; Hoppmann 2001). This
treatment approach has limitations
for musicians earning their living from
playing. If a period of rest can be taken
(eg a holiday), it may calm symptoms
down, but in any case after a period of
not playing, musicians must return with Figures 3a and b: Immobilisation splint
slow graded progression in duration,
tempo and complexity of playing, with
psychological support (Tubiana 2000). supervision and involve one or more LIMB IMMOBILISATION
(See Appendix 1 for graded return to of the other digits to exercise up to 2.5
playing programme) (Warrington 2003). hours per day for eight consecutive Prolonged immobilisation of the forearm
days. (See Appendix 2 for exercises and and hand for four to five weeks has
Some benefit from splintage has been exercise schedule) (Candia et al 2002 and been tested as a treatment for patients
displayed in patients with writer’s 2003). Candia et al (2002 and 2003) have with musician’s cramp (Priori et al 2001).
cramp (Tas et al 2001; Singer and Weiner published findings of two studies where Directly after the removal of the splint,
1995). Lederman (1998) reports on four improvements in both objective and patients displayed weakness and
bagpipers with focal dystonia, one of subjective measurements, utilising a clumsiness, but after they regained
whom devised some splints that he found dexterity displacement device and patient voluntary movement improvements
helpful in decreasing the dystonic finger self-rating dystonia evaluation scale, lasted for up to 24 weeks in 50% of
movements. were noted. The first study (Candia et al the patients. The authors claim that
1999) was based on five musicians and immobilisation allows for plastic
SENSORY MOTOR RETUNING the results supported the authors’ beliefs changes to occur at the cortical level
(CONSTRAINT-INDUCED MOVEMENT that SMR produces short-term functional (Pesenti et al 2001), and that prolonged
THERAPY) improvements associated with neuronal immobilisation, used in conjunction with
reorganisation. The second study (Candia post-splintage rehabilitation, may lead
Constraint-induced movement therapy et al 2002) involved 11 professional to better therapeutic results for patients
(CIMT) is a behavioural therapy approach musicians and evaluated the long-term affected by focal hand dystonia (Priori et
that has been trialled and proven to be effects of SMR. The results suggest that al 2001). A larger sample group, studied
effective with stroke patients (Taub et al SMR is a valuable treatment technique under controlled conditions, is needed
1993 and 1999). The nonaffected limb is for pianists and guitarists, as each of in order to validate this treatment for
immobilised in a cast, thus encouraging these subjects displayed improved general use. Information regarding ideal
the affected limb to be used. These spontaneous repertoire performance length of immobilisation, number of
principles have been generalised and without the splint. The wind players joints to splint, rehabilitation regimes
trialled for treatment of patients with did not display any improvement, and post-splintage, and clinical features
focal hand dystonia. They have been the authors offer two explanations for that would indicate patients who would
labelled sensory motor retuning (SMR) this: either finger-mouth co-ordination benefit from the treatment need to be
(Candia et al 1999). The intervention affects brain mechanisms, or SMR is not scientifically stated.
involves fixing the ‘compensating’ finger effective in treating performers with focal
for the dystonic movements in a splint, dystonia who exert a fairly constant and
while the ‘dystonic’ finger carries out firm force whilst playing.
repetitive co-ordination exercises.
Exercises are completed under therapist
The British Journal of Hand Therapy Autumn 2006 Vol 11 No 3 PAGE 81
Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

OCCUPATIONAL THERAPY AND where the patient can perform the given reported no improvement. For a detailed
PHYSIOTHERAPY task normally, referring the patient to a review of this treatment approach, refer
teacher who is trained in working with to Chamagne (2000).
Physiotherapy and occupational therapy injured musicians, and evaluating and
play an important role in performing arts making necessary recommendations/ These findings indicate that a multi-
medicine (Hoppmann 2001). Splinting, alterations to the workplace and disciplinary approach to the treatment
adaptive devices, heat, ice, exercise, instrument may also be helpful (Byl 2000). of patients with focal hand dystonia is
electrical modalities, rehabilitation and Further recommendations may relate probably the most effective. However, the
preventative measures can be useful in to the musician’s general approach treatments are slow and time-consuming
treating the injured instrumentalist. Chen to life, for example: instruction in and success does rely on the patient
and Hallett (1998) state that they use diaphragmatic breathing, ensuring the being motivated and performing the
occupational therapy as one treatment patient is well hydrated and has a healthy exercises regularly and carefully. Muscle
option for patients with writer’s cramp, diet and encouraging involvement strengthening, appropriate modification
whilst Berg and Naumann (1998) in a cardiovascular conditioning to instruments, medications and
comment that they prefer treating this programme (Byl 2000). A comprehensive botulinum toxin injections are utilised
patient group with occupational therapy physiotherapy programme that includes by Hochberg et al (1990) to treat patients
in conjunction with botulinum toxin. an aggressive sensory re-education with focal hand dystonia.
Hochberg et al (1990) prefer to see if element, accompanied by exercises that
conservative hand therapy treatment, facilitate fitness and musculoskeletal
such as strengthening exercises health, can improve sensory processing SUPPORTIVE APPROACHES
for weak hand muscles (interossei, and motor control of the hand (Byl and
lumbricals, abductor pollicus longus McKenzie 2000). ASSISTIVE DEVICES/ MODIFICATIONS TO
and brevis) is effective before they utilise INSTRUMENT
pharmacological intervention. Tubiana and colleagues propose a
four-stage treatment programme Singer and Weiner (1995) comment,
MULTI-DISCIPLINARY APPROACH for patients with focal dystonia. It is but do not provide a scientific base,
based on relaxation, deprogramming that a writing aid may benefit patients
No single treatment modality seems acquired bad habits and a complete with writer’s cramp. Koller and Vetere-
to be effective for the treatment of rehabilitation of the neuromuscular Overfield (1989) present a patient who
focal dystonia. In the multi-disciplinary system (Chamagne 1983 and 1996; Tubiana had tried various medications over
approach, many treatments can be and Chamagne 1983; Tubiana 1998). The a five to six-year history of writer’s
integrated, and occupational therapy and stages are: reconstructing the patient’s cramp, who found benefit from a small
physiotherapy play an important role. body image, relaxation training and writing block that was advertised as
The choice of treatments depends on muscle differentiation, individual muscle an aide for people with arthritis. The
the patient’s symptoms. Each treatment retraining, and technical retraining on authors say that a writing device can be
programme is individual and changes the instrument. Tubiana and colleagues viewed as a ‘sensory trick’ and should
according to the short- and long- believe that re-education involves the be trialled before pharmacological or
term goals of the affected patient and whole body and the mind, not just the botulinum toxin intervention, as it is
those treating them. When assessing upper limb. Tubiana and Chamagne non-invasive and has no adverse effects.
and treating a patient with focal hand report that trust and co-operation in A similar approach should be tested
dystonia, a whole body approach must the therapeutic relationship is essential with musicians, where a change in
be emphasised (Kember 1997; Byl 2000). for treatment success. Patients must instrument may be able to decrease or
Treatments such as soft tissue massage, be dedicated to participating in the improve symptoms. Hochberg et al (1990)
neural mobilisation, splintage, intrinsic retraining programme. Brockman et al comment that modifications to musical
muscle strengthening and sensory (1993) present the results of 483 patients instruments can eliminate postural
discrimination exercises may be who utilised this treatment programme: triggers, decreasing focal dystonic
useful (Byl 2000). Other evaluations and 95 returned to concert performance, symptoms and have profound benefits
treatments, such as finding positions 286 had partial improvement, and 57 for the patient. Possible modifications

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Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

include: changing to a smaller


instrument, using a neck support,
altering location of thumb supports,
altering bridge and string height and
extending or altering finger supports.

FELDENKREIS THERAPY AND


ALEXANDER TECHNIQUE

Nelson (1989) and Hoppmann (2001) state


respectively, that Feldenkreis therapy
and Alexander technique can be utilised Figures 4a and b: Instrument modifications can include wrapping the end of a bow in
as treatments for patients with focal Coban and adding a Blu Tack bow ‘build-up’.
hand dystonia. They believe patients gain
an awareness of control, with simple
movements being practised initially, the effectiveness of biofeedback as a can examine the patient’s goals and
and then more complex patterns being treatment for focal hand dystonia. unconscious problems. If social problems
introduced once control of muscle activity are evident due to the dystonia, then
and relaxation techniques have been COUNSELLING AND PSYCHOTHERAPY support therapy may be useful. Short
learnt. No scientific studies were found duration behavioural therapy could be
to support or refute the use of these Lim et al (2001) comment that dystonic appropriate if patients are experiencing
techniques for musicians with focal hand movements occur predominately difficulty in adhering to therapy regimes
dystonia. while performing perceptual-motor and in overcoming fears and returning
tasks involving emotion. It is noted to performance. Relaxation therapy may
BIOFEEDBACK THERAPY that there is difficulty in changing assist patients with difficulties relating to
emotional and motor traces that have perception of their surroundings and self.
It is felt that biofeedback, when used in become associated, and this may lead
conjunction with occupational therapy to preservation of dystonic symptoms.
(Hochberg et al 1990) or physiotherapy Psychosocial and somatic aspects are PREVENTION OF FOCAL HAND
(Singer and Weiner 1995), may be useful interacting in patients with focal dystonia, DYSTONIA
to re-educate muscles that have been and thus psychotherapeutic support in
affected by focal dystonia. Biofeedback addition to botulinum toxin is indicated The key to treatment is prevention
can be used to help patients eliminate for some patients (Erbguth 1997). Tubiana (Sataloff et al 1991; Slade et al 1999),
muscle co-contraction, and patients are (2000) comments that treating therapists therefore to decrease chances of a
asked to hold and palpate the target often have a psychological action that is musician developing an injury they
instrument but are not permitted to inseparable from the rehabilitation, due should try to avoid: irregular practice
play it until involuntary movements to developing a therapeutic rapport with with highly intense periods of practice
are controlled (Byl and McKenzie 2000). the patient after spending long amounts and performance, unnecessary changes
However, in a controlled study on of time with them throughout their in instrument or technique, learning a
spasmodic torticollis patients, subjective treatment regime. Emotional support lot of new repertoire at the same time,
improvements in symptoms were noted can be imperative when a musician is unrelated hand-over activity, trauma
in both control and treatment groups affected by such a disabling condition as to the hands and emotional stress.
(Byl 2000). These subjective results were focal dystonia. Referral to professional Musicians should instigate sensible
not associated with significant changes help for clinical depression may be practice techniques with regular breaks
in objective measures, thus the authors necessary (Hoppmann 2001). Kolle (2000) and reasonable total playing/practice
conclude that the sole use of EMG states that a psychological approach to time; utilise strong but flexible bodies
biofeedback for treating torticollis is not treatment of patients with focal dystonia that are well conditioned; perform
supported. More research is needed into can play a role in rehabilitation, as it warm-up and cool-down exercises

The British Journal of Hand Therapy Autumn 2006 Vol 11 No 3 PAGE 83


Focal Hand Dystonia Affecting Musicians. Part I: An Overview Of
Epidemiology, PathoPhysiology And Medical Treatments

and gradually increase the intensity cortex. However, when the speed and With regard to sensory re-education,
and duration of their playing. A holistic force of the repetitive motor tasks are future developments may lean
approach must be adapted, where varied and interspersed with other towards the use of computerised
locomotor problems are corrected and regular activities, the degradation of sensory stimulators to increase
playing technique, lifestyle, psychosocial hand cortical representation and loss of sensory retraining efficiency. Byl
and emotional factors are carefully motor control can be minimised (Byl et al and McKenzie (2000) suggest there
assessed and re-instructed and/or 1997). Thus, it is important to maintain is a need for computer technology
modified as necessary (Wynn Parry 1998). instruments in top playing condition, with ‘…to provide intense, goal-directed,
Stress and anxiety before a performance, the hope of decreasing excessive energy suitable, motivating, repetitive, and
temporal-spatial constraints of the outlay for desired level of performance discriminatory sensory stimulation to
instrument and playing, overuse and (Hoppmann 2001). Musicians need to patients.’ The challenge is to negotiate
hours practised should all be controlled, intersperse practice and playing with with compensatory bodies and maintain
thus hopefully minimising the possible other activities in order to decrease the motivation levels with patients, so that
risk of developing focal hand dystonia chances of developing focal dystonia and sensory discriminative task performance
(Lim et al 2001). other conditions. continues until enough cortical sensory
remapping has occurred to allow
Tubiana and Chamagne (2000) and normal motor control (Byl and Topp 1998).
Altenmüller (1998) believe that freedom FUTURE RESEARCH Availability of computerized equipment,
of interpretation in musicians who that increases the intensity of repetition
improvise, and freedom from external Controlled studies are needed to and the gradation of the sensory
pressures in amateur musicians, may be assess whether therapeutic benefit is decisions, may shorten retraining times
preventative factors in the development accompanied by functional changes in and make tasks more interesting (Byl and
of focal dystonia. Newmark and the brain. Objective parameters need to Topp 1998).
Lederman (1987) carried out research be established, in order to measure the
on musicians at a conference. Most of effectiveness of treatments. Finger force
the players (73%, 79/109 with only two and velocity pre- and post-therapeutic CONCLUSION
being professional musicians) did not intervention may be useful objective
usually perform routine practice. had measurements. The longevity and degree An increased focus on health issues
a rapid increase in playing time and of treatment effectiveness needs to be relating to the performing artist has
were predisposed to over-use injuries. assessed. Assessment of the musician’s occurred in recent years, highlighting
81% (48/79) of those with a significant repertoire and performance levels before the frequency and debilitating effects of
practice increase developed new playing onset of focal dystonia, and following focal hand dystonia affecting musicians.
related complaints, whilst 63% (27/79) treatment, need evaluation. Circulation of Use of splints, biofeedback, mechanical
experienced problems even without a standardised research-based treatment aids, technical retraining, botulinum
significant increase in playing time. The regimes to musicians, music teachers toxin injections and oral medications
authors comment that musicians should and the treating multi-disciplinary team may provide some relief of symptoms.
view themselves as athletes, be more should ensure a validated knowledge However, there is a need to identify
attentive to their physical limitations and base with working guidelines. the mechanisms accurately by which
condition their bodies accordingly, in the focal dystonia develops in musicians.
hope of preventing over-use injuries. There is limited evidence surrounding More research into the pathophysiology
They hope that teachers, performers and the effectiveness of treatments needs to be undertaken to support the
physicians learn from the experiences such as hydrotherapy, acupuncture, development of strategies for prevention
of their respondents and implement a chiropractics, dietary changes, exercise and treatment of this condition. As a first
carefully planned increase to playing time. and magnetic devices for the treatment step towards this goal, an impairment
of focal hand dystonia. It is necessary of sensory-motor control has been
Animal studies show that highly for scientific research projects into the identified in musician’s dystonia and
repetitive motor movement contributes effectiveness and long-term benefits of treatment strategies that aim at re-
to degradation in the somatosensory these modalities to be completed. establishing physiological sensory-motor

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Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

integration in the brain are shown to be Appendix 1


effective in reducing dystonic symptoms Graded return to play programme
(Candia et al 2003).
PRACTICE SESSIONS PER DAY MINUTES OF PLAYING
A comprehensive hand therapy Two sessions shadow playing 3-5 minutes
programme with an aggressive sensory
Two sessions on instrument 3-5 minutes
re-education element, accompanied
by exercises that facilitate fitness and Two sessions 5-10 minutes
musculoskeletal health, can improve Two sessions 15 minutes
sensory processing and motor control Two sessions 20 minutes
of the hand. Dialogue between health
Three sessions 15 minutes
care professionals, teachers and
performers needs to be free, so that Three sessions 20 minutes
effective treatment and preventative Four sessions 20 minutes
measures can be facilitated and Four sessions 30 minutes
implemented. Prevention is the primary
Three sessions 45 minutes
aim of performing arts medicine. Mutual
Three sessions 60 minutes
education is imperative for informing
and overcoming barriers amongst all Two sessions 90 minutes
people that come into contact with, and Two sessions 120 minutes
are affected by, focal hand dystonia.
Encouragingly, recent investigation
Start with Simple, Slow and Soft music
into focal dystonia is more frequently
Double minutes of playing every few days
of a scientific nature, rather than
Drop back a level if pain is elicited
the descriptive methods of the past.
Gradually progress repertoire difficulty
Collaboration and a multi-disciplinary
A five-minute break is encouraged every 20 minutes at the higher levels of playing
team approach to prevention, treatment
and research are imperative and will be
of benefit to all.

Appendix 2 - Exercises and exercise schedule

The ‘focal dystonic digit’ and the digit(s) movements of two or three fingers, including Subjects then have a rest of about 40
that perform compensatory movements for the focal dystonic finger, are completed. Five minutes. The patient is then encouraged
the dystonic one are identified. A splint that blocks of exercises are performed in an hour. to play their instrument without the splint.
immobilises the main compensatory finger They are invited to play a piece of music
and in turn permits independent movement Initially, the exercise task is paced by a of their choice for 15-30 seconds. If they
of the dystonic finger is then fabricated. metronome and begins at a medium tempo cannot do this, they are encouraged to
(60bpm). The tempo is then increased and try a second time. After two successful
Sequential exercises are then performed, gradually decreased, as some musicians with repetitions, they are asked to play a new,
in which the subject makes movements of dystonia find slow, controlled movements longer segment of the piece, until they
two or three digits in extension, including more difficult than fast ones. have played for 15 minutes (excluding rest
the focal dystonic digit. These exercises breaks). After a five-minute break, if the
are performed for a 10-minute period, in After completing the first five blocks of patient is not too fatigued, the splint is
a continuous ascending and descending exercises, the splint is removed and patients can reapplied and a second series of alternating
order (e.g. D2, D3, D4, D3, D2 etc. with D4 rest for 10 minutes. Following this, four more digital manoeuvres, each of five-minute
being the focal dystonic finger and D5 the 10-minute blocks of exercise with two-minute duration, is performed. This regimen
immobilised main compensatory finger). rest breaks between the blocks are completed. continues for eight consecutive days.
The patient then rests for two minutes. A variety of possible finger movements are
Following the rest, a different sequence of performed in the different exercise blocks.

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Focal Hand Dystonia Affecting Musicians. Part II:
An Overview Of Current Rehabilitative Treatment Techniques

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