distonia focal patologfia

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Brain (1982) 105,461-480
WRITERS' CRAMP—A FOCAL DYSTONIA
by M. P. SHEEHY and c. D. MARSDEN
(From the University Department of Neurology, Institute of Psychiatry and King's College Hospital
Medical School, de Crespigny Park, London SE5 8AF)
SUMMARY
We have examined 29 subjects with writers' cramp (and 4 with typists' and one with pianists' cramp)
and have noted two major groupings, simple and dystonic. We have observed spread from one to the
other. We have seen, repeatedly, in patients with isolated simple writers' cramp certain subtle physical
signs which are found also in other basal ganglia diseases. We have noted also the frequent association
of other features of segmental and generalized dystonia in patients with dystonic writers' cramp. We
have demonstrated that patients with isolated writers' cramp have no higher an incidence of psychiatric
disturbance, as judged by formal Present State Examination, than the normal population. We
conclude that isolated writers' cramp is a physical illness rather than a psychological disturbance, and
that it is a focal dystonia.
INTRODUCTION
The aetiology of writers' cramp, Schreibekrampf, or la crampe des ecrivains, has
been a matter for conjecture. Brain (1933), in his textbook, discussed the entity in a
section entitled 'The Neuroses' and called it a functional nervous disorder that 'is
primarily psychogenic', and 'resembles the disorder of function which occurs in
hysterical paralysis'. Even in the current edition of Brain's Diseases of the Nervous
System (Walton, 1977), this view still holds. The purpose of the present paper is to
put forward the case that writers' cramp and other 'occupational neuroses' with
onset in adult life are isolated manifestations of the syndrome of idiopathic torsion
dystonia; we believe them to be focal dystonias (Marsden, 1976).
We have studied a series of 29 patients with isolated writers' cramp. The clinical
features of simple writers' cramp and dystonic writers' cramp will be discussed,
transitions from one to the other will be described and their association with other
neurological disorders will be highlighted. We have also studied four patients with
simple typists' cramp, one of whom developed other manual dystonia, and one
patient with simple pianists' cramp. A formal psychological assessment of the
mental state of these patients will be presented and compared to that of a control
population.
Before doing so, we will present a brief historical review of the development of
thought on the subject. ^
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462 M. P. SHEEHY AND C. D. MARSDEN
H I S T O R I C A L R E V I E W
According to Gowers (1888), the first description of writers' cramp appeared over
a century and a half ago (Bell, 1830, 1833; Bruck, 1831). Since that time most
authors have found common ground when describing some of the clinical features
of this and other occupational neuroses, but opinions have differed widely as to
whether writers' cramp primarily is a psychiatric or a physical condition.
Initial reports in the European literature, including those of Duchenne (1883)
were descriptive. Duchenne reported cramp not only in writers but in several other
professional groups (pianists, tailors, cobblers, florists, fencers, tinmen and turners),
but beyond proposing that the trouble depended upon some derangement of the
'nerve centres', he did not speculate further. The number of professions to be
associated with craft palsies has been enlarged and, most recently, Hunter (1978)
listed 55 different occupations. Babinski (1921) suggested that writers' cramp was
'un syndrome strie' and noted an association between writers' cramp and spasmodic
torticollis. Barre, first in 1925 and as late as 1952, proposed that writers' cramp was
an organic condition akin to 'les troubles reflexes sympathico-cerebro-spinaux'. He
noted an association with cervical cord disease, with spasmodic torticollis, with
apical pleural afflictions and with other local disease of the neck. He noted also that
writers' cramp could follow encephalitis, and reported an association with 'maladie
des tics'. Charlin (1954) felt there was evidence for central ('un syndrome strie non
parkinsonian'), peripheral (reflex changes, cervical spondylosis) and psychological
factors; but he felt that the presence of psychological factors could not detract, in
any way, from the primary importance of the neurological features in the genesis of
writers' cramp.
In the English literature, Poore (1872, 1878, 1897) described his examinations of
many hundreds of patients with writers' cramp. Indeed, the frequency of the
disorder in that late Victorian era must stand as a tribute to the success of the British
Empire, the enormous office staff required to run it, and the difficulties of
manipulating the quill pen. As Samuel Solly, FRS, Senior Surgeon to St. Thomas'
Hospital, wrote in 1864 in a clinical lecture on 'Scriveners' palsy, or the paralysis of
writers','... the greatest part of the middle classes of London got their bread by the
use of the pen, either as the exponent of their own thoughts or the thoughts of others,
or in recording the sums gained, lost, or spent in this great emporium of commerce—
this vast Babylon'.
When Gowers (1888) adopted the term 'occupation neurosis', he took it from the
German Besshaftigungsneurosen, and used it as a convenient designation for writers'
and related cramp. However, Gowers did not believe that writers' cramp was a
psychological illness—his use of the term 'neurosis' was in keeping with the 19th-
century meaning of the word, a physical disease with no discernible cause, rather
than contemporary understanding of neurotic illness. Indeed, in the same publica-
tion, he expressed the opinion that craft palsies were physical disorders. He
suggested that a 'writing centre' existed, probably in the cerebral cortex, and that its
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WRITERS" CRAMP 463
output was excessive or irregular, giving rise to cramp. He said also that faulty
penmanship was to blame: 'the smaller the muscles employed (to move the pen).. .
the more readily does cramp occur . . . The worst mode of writing is with the little
finger as a fixed point of support (and) the pen is moved up and down by the muscles
of the thumb and the first two fingers, which are constantly contracted almost to
their maximum'. He advocated a freer manner of writing, employing the shoulder
rather than the wrist and fingers to move the pen, as both preventative and curative
therapy.
Osier (1892) also felt that writers' cramp was due to a physiological disturbance of
a localized area of the brain responsible for the movements involved in writing, and
Jelliffe in 1910 suggested that the disorder might be caused by a 'disordered cortical
control affecting improper writing commands'.
Collier and Adie (1922) went further in the first edition of Price's Textbook of the
Practice of Medicine where they suggested that 'heredity and neuropathic conditions
have no causal connection with the malady, nor have local abnormalities in the
forms of arthritis, neuritis, nor organic nervous disease. The malady is certainly of
central origin, and the combination of pain, spasm and loss of control points to the
region of the basal ganglia as the site of the breakdown in function which produces
the disability'. Subsequent contributors to Price's Textbook (Martin and Elkington,
1946; Williams, 1966) adopted a more neutral stance, stating 'causative factors are
no doubt numerous and often multiple, and both physical and psychological in
nature, but in their summation they result in the breakdown in the smooth execution
of a stereotyped movement, and ultimately lead to the setting up of a faulty habit
closely akin to a stammer or a tic'.
Kinnier Wilson (1940), whilst noting that predisposing factors included a
neuropathic or psychopathic constitution, said that 'without doubt, however, the
most usual agent is long-continued and excessive use of some musculature,leading
to chronic fatigue'. He advocated a free rather than a cramped style of penmanship,
to avoid an endless succession of little movements involving continued contractions
of the small muscles, as this is 'principally to blame in this neurosis production ... ' .
Early attempts at treatment, including tenotomy and immobilization, were
discarded in favour of the 're-education' therapy advocated by Gowers (1888).
Exercises were employed to reduce the palpable hypertonus of forearm muscles and
unusual writing postures adopted to circumvent spasm. Subjects with writers'
cramp were treated by behaviourists who regarded the malady as a bad habit arising
out of a faulty learning experience (Janet, 1925). This treatment, coinciding as it did
with the increasing emergence of psychoanalytical concepts, shifted attention away
from a physical cause for writers' cramp towards a psychological explanation. Those
afflicted were called 'neuropaths' who because of 'an inadequate mode of
adaptation' suffer 'agitation . . .'. Subsequently, it became accepted generally that
occupational cramp was a psychoneurotic disorder, as it was associated frequently
with various emotional disturbances—anxiety, obsessive-compulsive behaviour,
hysteria, stammering, depression and maladjusted personality (Culpin, 1931; Pai,
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464 M. P. SHEEHY AND C. D. MARSDEN
1947; Ferguson, 1971; Sarkari et al., 1976). Pai (1947) ascribed the illness to 'poor
muscular efficiency' as well as 'severe psycho-neurotic symptoms' and concluded
his discussion with these words: 'Every person complaining of cramp for which
there is no organic cause should be considered as a psychiatric patient and treated
accordingly'. This is a view with which we do not agree.
More recently, Crisp and Moldofsky (1965) viewed writers' cramp as a
psychosomatic disorder. They hypothesized that as the upper limbs are associated
developmentally first with 'grasping, clinging, rejecting, incorporating and support-
ing', and also with the expression of emotional states, so the upper limb may become
a 'major organ of the expression of anger at the non-verbal musculo-skeletal level'.
They proposed that motor skills, including handwriting, develop against this
background. Initially, they suggested, a great deal of attention to the posture and to
the learning and execution of the work of writing is required. Eventually this
becomes automatic and, like other automatic skills, is dependent for its adequate
execution on the relaxation of antagonist musculature, so that it is upset by any rise
in the general level of muscle tension in the upper limb. They studied seven subjects
with writers' cramp and 'considered that they were particularly tense, strong,
sensitive, conscientious, precise, emotionally over-controlled people . . . This
personality type has been said to be generally characteristic of patients suffering with
various psychosomatic diseases...'. All their patients were found to have particular
difficulties in expressing their anger in important interpersonal relationships and in
their work. This difficulty in expressing anger, the authors proposed, found its outlet
in writers' cramp. Because of the widespread belief that writers' cramp was a form
of neurotic disorder, various psychological treatments were tried. Crisp and
Moldofsky treated their patients with psychotherapy, relaxation and retraining
techniques, but noted the recurrence of symptoms outside the clinical situation
where any stress might evoke his or her conflict over anger.
Other therapeutic endeavours have been reported, for example, avoidance
conditioning (Sylvester and Liversedge, 1960; Beech, 1960) and biofeedback
(Bindman and Tibbetts, 1977), but without enduring success. Despite the obvious
lack of therapeutic response to such therapy, the view that writers' cramp is a
psychosomatic or psychoneurotic illness has been accepted by many, not only in
psychiatric publications, but in some neurological texts as well. Our purpose is to
marshal the evidence to show that this is not the case.
P A T I E N T S A N D M E T H O D S
A total of 29 patients with isolated writers' cramp, four with typists' cramp and one with pianists'
cramp (19 males and 15 females), but without dystonia elsewhere, were seen personally in the Neurology
Clinic at the Maudsley Hospital whence they were referred by psychiatrists, local general practitioners
and other neurologists. Clinically, they conformed to the picture typical of an occupational palsy. All
developed muscular spasm and apparent inco-ordination when attempting to write, type or play. None
had torticollis, tmncal or leg dystonia, blepharospasm or oromandibular dystonia. None had evidence
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WRITERS' CRAMP 465
of other neurological deficit such as that due to Parkinson's disease, damage to corticospinal pathways,
peripheral sensory and motor nerves, or cerebellum. The occupations of the 34 patients are shown in
Table 1.
All patients were examined specifically for (1) the nature of the precipitant (for example, writing,
typing, playing a musical instrument); (2) whether single or multiple precipitants were present, and if
multiple, whether they appeared simultaneously or evolved over a period of time; (3) the presence of
dystonic postures at rest or on action; (4) the presence of tremor; (5) the presence of increased tone in
the affected limb; (6) the attitude of the arm on walking.
T A B L E 1. O C C U P A T I O N S OF 34 S U B J E C T S W I T H W R I T E R S ' C R A M P AND
OTHER O C C U P A T I O N A L PALSIES
Clerical duties
Accountancy
Student
Typist
Pianist
Waitress
Teacher
12
5
4
4
1
1
1
Industrial scientist 1
Greengrocer 1
Production line worker 1
Research psychologist 1
Hairdresser 1
Barmaid 1
All patients were assessed by a validated interview technique (the Present State Examination, PSE)
for the presence and severity of psychiatric symptoms (Wing el al., 1974). This method was chosen after
advice taken from the MRC Social Psychiatry Research Unit at the Institute of Psychiatry (Dr J. Leff)
as to the best method of standardizing the assessment of a subject's psychiatric and mental state and of
achieving greater comparability between different examiners. The basis of the technique is a glossary of
definitions which clearly sets out the experiences which constitute psychiatric symptoms. The
technique itself is a semistructured interview which allows the symptoms to be elicited and reliably
recorded—it involved putting and rating 54 compulsory questions to the subject, as well as rating the
presence or otherwise of a further 86 parameters, depending on the replies to the compulsory questions.
All interviews were carried out by one of us (M.P.S.), who had been trained in the use of the PSE. Every
item was rated and entered on a coding form, whence they were transferred to punch cards for
computer analysis. Computer analysis of these 140 items provides a PSE score, an Index of Definition
(ID) level and a sorting of these 140 items (mostly symptoms) into 12 major psychiatric groupings (each
called a CATEGO class) with degrees of certainty. Then subjects may be allotted to one of 50
subclasses of this CATEGO classification.
PSE scores refer to number of symptoms rated, and ID levels ranks these scores so that the presence
of a single key symptom scores higher than two or three nonspecific symptoms. ID levels progress from
one (no PSE symptoms), 2 and 3 (PSE scores between one and 4 and between 5 and 9, made up
exclusively of nonspecific neurotic symptoms), 4 (PSE a total score, nonspecific neurotic symptoms
only, greater than 9 or the presence of a single key symptom), 5 (PSE indicates the presence of several
key affective symptoms), to 6, 7 and 8 where there is an increasing degree of certainty that the
symptoms present can be classified into one of the conventional categories of the functional psychoses
and neuroses. Only when level 5 is attained can a tentativepsychiatric diagnosis be offered.
The PSE scores obtained in these 34 patients with writers' and other cramps have been compared
with those obtained in a group of 310 normal subjects interviewed by the Social Psychiatry Unit at the
Institute of Psychiatry.
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466 M. P. SHEEHY AND C. D. MARSDEN
RESULTS
Writers' Cramp
The 29 patients with writers' cramp could be divided into two groups, those with
simple writers' cramp and those with dystonic writers' cramp. Simple writers' cramp
encompassed that group who exhibited difficulty only when writing, other manual
acts being carried out normally. Dystonic writers' cramp referred to the group in
whom muscle spasms affected not only writing, but also other manual tasks such as
the ability to handle a knife and fork, a cup and saucer, a shaving brush or make-up,
or a mechanical implement.
Of the total of 29 patients, 8 presented with dystonic writers' cramp from the
beginning of their illness, and 21 with simple writers' cramp at the onset of their
disability. However, 8 of those initially with simple writers' cramp later developed
features of dystonic writers' cramp, that is, their illness began with writing
difficulties alone, but subsequently problems were encountered with other motor
tasks. This latter group of 8 patients are described as having progressive writers'
cramp (Table 2).
TABLE 2. PERSONAL AND CLINICAL FEATURES OF 34 SUBJECTS WITH WRITERS',
TYPISTS' AND PIANISTS' CRAMP
Number of subjects (n)
Sex (M : F)
Age at onset (years)
(range)
Duration of disease (years)
(range)
Handedness
Precipitant (see text)
Other neurological signs:
Tremor
Increased limb tone
Decreased arm swing
Dystonic posture
Simple
writers'
cramp
13
7; 6
30
(4-53)
15
(4-36)
12R, 1L
_
6
1
2
—
Progressive
writers'
cramp
8
4; 4
27
(16-43)
20
(4-39)
8R
_
3
1
1
8
Dystonic
writers'
cramp
8
7; 1
31
(6-53)
13
(6-29)
7R, IL
3
5
1
4
8
Simple
typists'
cramp
3
3F
28,30,61*
10, 5, 5*
3Rf
1
—
_
—
—
Progressive
typists'
cramp
1
F
40
12
R
_
—
_
1
1
Pianists
cramp
1
M
20
5
Rt
_
_
_
—
•Individual ages of subjects or duration of disease, f&e text for further discussion concerning onset and handedness.
Symptoms
Only 7 patients described their difficulty as cramp; 7 other patients complained of
aching in the hand on writing. Most initially were unable to describe the problem in
detail. Some noted only a deterioration in neatness or speed of writing, or that it was
'like having two left hands'. Others described writing becoming a struggle, or a
chore, or just clumsy. Several patients said they had to press hard or hold the pen
tightly; exaggeration of finger grip on the pen was one of the more frequent features
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WRITERS" CRAMP 467
of this condition. Paradoxically, one patient noted lifting and hyperextension of
index and middle fingers whenever the pen was held. Several reported that the hand
froze on attempting to write, or that there was difficulty in moving the pen across the
page. Two patients said that they habitually helped the writing hand by pushing it
across the page with the other hand. Others reported that the hand appeared to
adopt a 'mind of its own' and would, for example, ignore ruled lines or involuntarily
jerk through the paper or off the page. A few patients volunteered curling or flexion
spasms of fingers, including those with typists' cramp. Several patients complained
that cramp, when it occurred, was secondary to muscular effort in keeping the pen in
the hand and in a straight line.
Signs
All patients were examined whilst writing (or typing, or playing the piano) and
certain distinctive features were noted. In many there was difficulty in picking up the
pen; it might be gripped in a closed fist, or the hand and fingers would adopt typical
dystonic postures whilst attempting to grasp the instrument. The pen commonly
was held very tightly, with an exaggeration of the normal semiflexed posture of
thumb, index and other fingers, and with hyperextension of the distal inter-
phalangeal joint of the index finger. On attempting to write, all subjects displayed
abnormal postures of the fingers, hands or wrists (fig. 1). In some instances, the hand
would come quickly to a halt, the paper perforated, and the subject exasperated
after barely a word of script. In other cases, the hand might dart across the page with
a sudden jerk, making nonsense of a single word. If the difficulty was with typing,
frequently one would see spontaneous flexion flicks of fingers, tapping unwanted
keys, slowing the speed of typing and jamming the machine. The script produced
was usually abnormal (fig. 2). Some patients could still produce legible words or
sentences, often at the expense of bizarre compensatory postures, for example
holding the pen like a dagger in a closed fist, but most examples of writing were
squashed, tremulous, jerky or completely unsuccessful.
In those with writers' cramp the remainder of the examination often was normal
(9 patients), but some subtle findings were noted (20 patients). Dystonic postures of
the relevant limb were noted in 16 patients either spontaneously as the patient sat or
walked, or induced by posture or rapid movement. There was evidence of loss of arm
swing on the affected side in 7 patients, and there was a minimal unilateral increase
in muscle tone in 3. However, there were no other signs of Parkinson's disease in
these latter two groups of patients. Decreased arm swing and increased limb tone
generally were seen in patients with simple writers' cramp, dystonic writers' cramp
and those with progression from one to the other.
Tremor in Writers' Cramp
As can be seen in Table 2 there was a high incidence of tremor in all types of
writers' cramp (14 of 29, 48 per cent), whether it was simple (6 of 13), progressive
(3 of 8), or dystonic (5 of 8). This feature has been noted previously in other forms of
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M. P. SHEEHY AND C. D. MARSDEN
\
FIG. 1. A sequential series of photographs of a subject with writers' cramp as she attempted to write. Several
features are noteworthy: she holds the pen in an unusual posture and her grip appears clumsy, a progressive flexion
deformity of the fingers and wrist can be seen as writing progresses so that the wrist is eventually lifted clear of the
desk, and the written word is either untidy or illegible.
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WRITERS' CRAMP 469
4~*
FIG. 2. Specimens of handwriting from 3 subjects with writers' cramp as they attempted to write or print 'King's
College Hospital'. The topmost example was from a patient with writers' cramp associated with tremor: the middle
and lower examples from 2 subjects who found it either difficult or impossible to write.
dystonia (Marsden, 1976; Couch, 1976). However, it is worthwhile emphasizing that
in 3 of the patients presenting with simple writers' cramp, the tremor was
particularly marked. All 3 had typical dystonic muscle spasm when they tried to
write, but also developed a marked tremor; such dystonic spasms were not evident
on other manual acts, but the tremor was and sometimes severely so. In the
remaining patients rest tremor was not seen nor was there any evidence of tremor
appearing (solely) during the finger-nose-finger test. Tremor was noted most
frequently with the arms outstretched or with both index fingers held just beneath
the nose, and not quite touching each other. Tremor usually was unilateral, affecting
the arm with writers' cramp, but occasional instances of asymmetrical bilateral
tremor were noted, and one subject exhibited gross bilateral intermittent tremor or
jerking with the arms outstretched.
Onset
Most patients experienced the onset of symptoms between the ages of 20 and 50
years (fig. 3). The writers' cramp appeared without any obvious precipitant in all but
4 of the 29 cases. One patient reported writing difficulty soon after the death ofa
parent; another whilst still in hospital recovering from injuries received in a road
traffic accident; a third patient described the dramatic onset of a jerking dystonic
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470 M. P. SHEEHY AND C. D. MARSDEN
o
Z
PI n
0-9 10-19 20-29 30-39
Age at onset
40-49 50-59
FIG. 3. Age at the onset (grouped in decades) of writers' (open columns), typists' (+ columns) and pianists'
(x column) cramp in a group of 34 subjects.
writers' cramp when trying to write to members of his family and tell them of his
son's sudden death; a fourth patient volunteered that writing difficulties started after
treatment for a soft tissue thumb injury. All subjects bar two were right-handed. The
two left-handed patients developed writers' cramp on the left. Of the three right-
handed subjects with simple typists' cramp, two developed symptoms in the left
hand; similarly the right-handed pianist noted difficulty with the left hand only. The
single right-handed subject with progressive typists' cramp developed symptoms on
the right.
Course
Eight patients stated that their difficulty was intermittent, at least at the beginning
of the illness. Thus, they could write normally for a few lines or paragraphs, and only
then would they experience difficulty. Often the problem in writing would become
more apparent when they were being observed (as in a school room or when writing
against the clock as in an examination). In 7 of these 8 patients the disturbance
progressed to difficulties on every occasion they attempted to write. The remaining
21 patients experienced symptoms continuously since the onset of the illness and in
this group the difficulty with writing bore no relationship to the duration or 'effort'
of writing.
The average duration of illness is shown in Table 2. Twenty-one subjects began
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WRITERS' CRAMP 471
with simple writers' or typists' cramp and 8 of this group developed progressive
writers' cramp. This spread occurred within six months of the onset in 5 cases, but in
the other 3 it occurred at five, six and thirty years after the onset of the original
writers' cramp. Another patient, not in this subgroup, described the reverse, that is,
her illness began with difficulty in writing and in picking up objects and
manipulating jar lids, but after a few months these symptoms disappeared, leaving
her only with difficulty with writing.
No patient described a prolonged remission of symptoms. One subject regained
normal writing for four weeks some months after the onset, but then her difficulty
returned. Prolonged rest did not lead to any relief of disability.
Fourteen patients changed hands and learnt to write with the unaffected limb. Of
this group 4 developed simple difficulties and one developed dystonic difficulties on
the contralateral side, within periods ranging from a few months to nineteen years.
A further right-handed patient with dystonic writers' cramp became aware, after a
period of twelve months, of tremulous difficulties with his left hand, although he had
never tried to write with his left.
Other Occupational Cramps
Three subjects (all female) were seen with simple typists' cramp. All were right-
handed, and two developed difficulty with some of the fingers of the left hand. One
woman noted that the left ring finger would not do as she wanted and then noted
slowing of the speed of typing. Within two years the difficulty involved the ulnar
three fingers of the left hand. Latterly she complained of an ache as she typed, and
she could no longer use those fingers to type. The second subject noted first a loss of
speed because of some difficulty with the left hand. She then noted her left thumb
involuntarily flexing under the other fingers whilst typing. She began to hold the
thumb out of the way and no longer used it whilst typing. The third subject noted
curling of the ulnar two fingers of her right hand as she typed. She also noted
occasional involuntary elevation of the right elbow as she typed. None of these three
women has had any difficulty with either hand at any other time, and all held a pen to
write normally.
A fourth female subject was seen with progressive typists' cramp. Her difficulty
began with progressive loss of use of her right ring finger when typing, then of the
right little finger, then of the middle, and finally of the index finger, over a period of
two to three months. She noted that the fingers would not hit the proper keys and
complained that they were 'ungainly'; the problem with the right index finger was
that it could press a key down, but then the finger would remain flexed longer than
necessary, slowing the speed and precision of typing. Some months later she noted
difficulty in picking up objects—she said the hand would go stiff at fingers and
wrist. This was seen most clearly when she attempted to pick up a pen as all
fingers became flexed, the palm pale from pressure, and the hand and wrist
adopted a dystonic flexed posture. In addition, one of the fingers of the left hand
became slower and clumsier when typing, so much so that she was forced to type
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472 M. P. SHEEHY AND C. D. MARSDEN
only with the ulnar three fingers of the left hand and jabbing with the right index
finger.
The one male subject with pianists' cramp noted first a heavy awkward feeling in
the fingers of the left hand when playing and, over a period of months, stiffness in all
fingers with the adoption of a claw-like posture. He was right handed and writing
was unaffected.
Because of the similarity between writers' cramp and these other occupational
palsies, we will consider them together when discussing the results of formal
psychiatric assessment, family history, investigations, disability and treatment.
Psychiatric Examination
Table 3 documents the results of the formal PSE examination in this group of 34
subjects with writers' and other cramp. Results are expressed as a percentage of the
group in each Index of Definition (ID) level. Results from the control population,
examined by the Social Psychiatry Unit at the Institute of Psychiatry also are shown.
TABLE 3. RESULTS OF PSE SURVEY SHOWING NO. (%) OF PATIENTS WITH
WRITERS' OR OTHER CRAMP, OR OF A NORMAL POPULATION, IN EACH INDEX OF
DEFINITION (ID) LEVEL
ID level
1
2
3
4
5
6
7,8
Writers' cramp subjects
n = 34
5
17
5
4
2
1
0
(%)
14.7
50.0
14.7
11.8
5.9
2.9
Control population
n = 310
129
85
40
22
23
1
0
(%)
41.8
27.3
13.0
7.1
7.3
3.6
Amongst the 34 patients with writers' or other cramp, 3 (9 per cent) scored at ID
levels suggesting psychiatric illness, 2 at level 5 (borderline) and one at level 6. The
CATEGO diagnostic subclass for these 3 patients was retarded depression (one
case) and simple depression (2 cases). In the control population, 11 per cent scored at
ID levels of 5 or more.
More patients with writers' or other cramp (50 per cent) scored at ID level 2 than
in the control population (27 per cent). However, for a subject to rate at this low
level, all that was required was one nonspecific symptom such as a degree of self-
consciousness in public.
Careful enquiry of patients and relatives into the psychiatric state of these patients
at the onset of their writers' or other occupational cramp revealed a remarkably low
incidence of psychiatric disability and certainly no higher than that indicated by the
PSE. All but one of the 34 patients considered themselves to be in normal physical,
mental and emotional health at that time.
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WRITERS' CRAMP 473
Family History
There was one instance of a positive family history; a 50-year-old female subject
with simple writers' cramp described her father as an appalling writer; he had always
found it better to either type or print as she remembered clearly that whenever he
attempted to write, the hand would jerk uncontrollably. Neither father nor
daughter had siblings and further historyof the father's family was unavailable.
Investigations
A small number of patients were admitted to hospital for further investigations.
Most, however, were seen as outpatients and routine biochemical and haema-
tological studies (electrolytes, urea, creatine, liver function tests, calcium, phosphate,
uric acid, haemoglobin, white cell count and differential, ESR, serology, radio-
graphs of chest, skull, cervical spine and hands) and copper studies (in 16 subjects)
were normal. CT scans were undertaken in 5 subjects—they were normal in 4, but a
little bilateral posterior caudate calcification was seen in the fifth (a woman with
progressive unilateral writers' cramp).
Disability
Many of the subjects with writers' cramp could still write with the affected hand,
indeed, only 5 of the 29 had completely stopped writing, preferring to employ aids
such as dictating machines and typewriters. Of those continuing to write, 19 still
employed the first affected limb, while 5 had switched to the opposite hand. Typists
preferred to carry on, not employing the offending fingers. Many sufferers had
developed tricks such as holding the pen like a dagger or using the other hand to
steady or move a trembling or dystonic limb. Ball point pens and similar fine pointed
instruments were most difficult to use, whilst wide felt tip pens were preferred by
many.
An attempt was made to assess the impact of the difficulty of writing on the
patient's life style. Only one subject, a typist, felt obliged to change her profession;
she became an occupational therapist. The remainder were able to continue in their
job, in 7 cases for a lifetime of clerical or administrative work. Two students became
clerks, despite the fact that their writing difficulties began in their student days.
All subjects were asked to estimate how much the cramp interfered with writing in
particular, and life style in general. After a variable period of evolution (Table 4)
subjects with simple writers' cramp felt less incapacitated, in both respects, than did
those with progressive or dystonic writers' cramp.
Those who developed dystonic writers' or typists' cramp had, by definition, other
manual difficulties. Frequently these caused problems with fine tasks such as
knitting, sewing, throwing darts, or building models. Carrying a cup or wielding a
knife or fork often were impaired, but never sufficiently to prevent drinking or
eating. Likewise, although handling buttons, zips or laces might be difficult, all
subjects could dress themselves and attend to toilet requirements without the need
for help.
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474 M. P. SHEEHY AND C. D. MARSDEN
TABLE 4. PATIENTS' ASSESSMENT* OF INTERFERENCE WITH WRITING AND WITH
LIFE STYLE, IN THE THREE MAIN GROUPS OF WRITERS' CRAMP
Interference with writing
Interference with life style
Duration of cramp
Simple writers'
cramp
n = 13
42%
23%
15y
Progressive
writers'
cramp
n = 8
55%
39%
20 y
Dystonic
writers'
cramp
n = 8
65%
44%
13y
*Patients' estimate of percentage of normal function; normal = 100 per cent.
Treatment
The drugs (and their maximum dosages) and other treatments employed in this
group of patients are shown in Table 5.
There was no instance of apparent cure with any drug. There were 6 patients in
whom benzhexol (6 to 24 mg/day) produced some benefit; in one instance there was
almost complete control of typists' cramp. One subject reported benefit with
diazepam or alcohol. Most patients, however, experienced unwanted side effects,
without relief of their occupational palsy, so preferred to take no drugs. None of the
physical or behavioural therapies produced any lasting benefit.
TABLE 5. DRUG TREATMENT (WITH MAXIMUM DAILY DOSES) AND OTHER
THERAPY EMPLOYED IN THE 34 PATIENTS WITH WRITERS' AND OTHER
OCCUPATIONAL CRAMPS
Drugs
Alcohol
Benzhexol
Orphenadrine
Diazepam
Clonazepam
Amitriptyline
Clomipramine
Sodium valproate
Carbamazepine
Diphenylhydantoin
Primidone
Pimozide
24 mg
150 mg
30 mg
1.5 mg
150mg
30 mg
1200mg
1200mg
300 mg
lOOOmg
12 mg
Halopendol
Thiopropazate
Thioridazine
Perphenazine
Promazine
Tetrabenazine
Sinemet
Amantadine
Baclofen
Tryptophan
Propanolol
15mg
30 mg
75 mg
24 mg
75 mg
200 mg
1650mg
300 mg
20 mg
2000 mg
240 mg
Physical or behaviour therapy
Acupuncture Osteopathy
Behaviour therapy Psychotherapy
Biofeedback Wax baths
Hypnosis
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WRITERS' CRAMP 475
DISCUSSION
Writers' cramp has been the subject of debate in the medical literature for the last
century or more. Most authors find common ground when describing the
appearance of the hand, but it was Gowers (1888) who noted additional features
when he differentiated between simple and dystonic writers' cramp. He described the
beginnings of writers' cramp as a spasm brought on by writing—what we would call
simple writers' cramp. He continued '. . . although the disturbance is in the first
instance confined to the special action, it usually extends, after a time, to other
actions . . . The extension may ultimately be so wide that spasm, and still more often
pain, occur on any movement. I have even met with cases in which a spasm, at first
exerted only by a special act, ultimately extended not only to all acts, but occurred
spontaneously'. Here he described clearly dystonic writers' cramp and the
subsequent development of focal dystonia. He noted also 'The sufferer who finds
himself unable to write with one hand often learns to write with the other. After he
has acquired the needful facility, and has written with the left hand for a time,
similar symptoms may develop in this hand, and they then usually progress more
quickly than in the arm first affected.' Other authors have noted differing patterns of
evolution; for example, Babinski (1921), Barre (1952) and Meares (1971) all have
noted an association between writers' cramp and spasmodic torticollis. Our own
observations support the view that writers' cramp indeed can spread; what appears
to be simple writers' cramp can evolve into dystonic writers' cramp in the same hand
or spread to the opposite hand. What has evolved into dystonic writers' cramp may
proceed then to focal dystonia of the whole limb and even to spasmodic torticollis.
Indeed, in a representative sample of 49 subjects with segmental dystonia (dystonia
affecting adjacent parts of the body, for example, arm and neck, arm and trunk) seen
by one of us (C.D.M.), no less than 21 noted that their illness began with writers'
cramp and one patient noted preceding typists' cramp. Similarly, in a group of 60
patients with generalized dystonia, writers' cramp was the initial feature in 13 and
pianists' cramp in one. We feel that these results indicate that simple or dystonic
writers' cramp is a focal or fragmentary dystonia, and at times, does develop into a
more florid movement disorder (fig. 4).
This study supports Gowers (1888) and Poore (1897) in other findings. Writers'
cramp is a disease of the young and middle aged and is seen in those who earn their
living by writing (in all but five of our subjects). Its true incidence must be a matter
for some conjecture. In Victorian times it was far more common in males, but all
clerks and similar scribblers were men. Poore (1897) reported that he saw over 500
such cases in his lifetime. In this century, Ferguson (1971) reported a 14 per cent
incidence of telegraphists' cramp in a group of 263 operators (confirming a union
claim). He noted also bilaterality of symptoms in 18 per cent of subjects and writing
to be affected in 56 per cent of sufferers. Sarkari et al. (1976) surveyed a group of 3325
office workers and noted an incidence of 5.4 per 1,000. The reason for this variation
in rate from 0.5 per cent for writers' cramp to 14 per cent for telegraphists' cramp is
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476 M. P. SHEEHY AND C. D. MARSDEN
SIMPLE
WRITERS'
CRAMP
OTHER
OCCUPATIONAL
PALSIES
Other actions involved
FOCAL DYSTONIC
WRITERS' CRAMP
1
FOCAL ARM
DYSTONIA
SEGMENTAL
DYSTONIA
I
GENERALIZED
DYSTONIA
Spontaneous dystonia appears
Neck, trunk, face, other
arm involved
Spread to all limbs and
axial structures
FIG. 4. Diagrammatic representation of the directions in which writers' cramp can evolve.
not clear. Ferguson related the higher incidence to the physical load in telegraphy—
'a morse operator in one minute made 515 different muscular contractions, more
than twice as many movements per minute as a typist'. Gowers also emphasized this
aspect of workload (and holding the pen incorrectly) as contributing to the genesis
of writers' cramp. It is clear that in some of the subjects presented here, particularly
in the early stages of the illness, writers' cramp can be proportional to the 'effort' of
writing or appear initially only when writing faster than normal and, like Gowers'
patients, may disappear during and even after a period of abstention from writing.
However, in the majority of our patients this was not the case; indeed, in some the
illness was to start quite suddenly one day, as soon as the pen was lifted.
Gowers also noted other possible contributing factors: a family history in four
subjects, apparent precipitate onset in a patient afflicted suddenly by great stress (for
example, death of a parent), exacerbation of writers' cramp when emotionally upset
or anxious and lessening of discomfort and disability when emotional factors have
disappeared. One subject in this survey gave a positive family history; 2 of our
patients reported the onset of writing difficulties following the death of a parent or
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WRITERS' CRAMP 477
child, and in 2 other subjects writing difficulties began whilst still recovering from
injuries to face or hand. However, we do not feel that an association with emotional
stress implies a psychological aetiology. Many physical illnesses, and not just other
basal ganglia diseases, can be exacerbated or improved by the presence or resolution
of emotional conflict. We have noted elsewhere (Sheehy and Marsden, 1980) the
apparent precipitant effect that physical injury may exert on another focal dystonia,
namely, spasmodic torticollis.
Tremor is another feature that has been noted for over a century. Gowers (1888)
noted that 'spasm is often accompanied by some tremor' and ' . . . occasionally there
is tremor without other spasm'. Poore (1897) noted it frequently in his large group of
subjects. Duchenne (1883) spoke of'spasmodic' and 'paralytic' types of writers'
cramp. Jelliffe (1910) mentioned 'tremulous' types. Pai (1947) spoke of three
subgroups: tremulous, spastic and ataxic. Gowers (1888) mentioned frank dystonic
postures of the arm at rest. We have also noted the presence of decreased arm swing
whilst walking and a minimal unilateral increase in muscle tone (without any other
evidence of Parkinson's disease) in some patients with both simple and dystonic
writers' cramp. We believe that the frequent occurrence of tremor, and the
occasional finding of increased tone, add support to our view that writers' cramp is a
physical illness, that it is a disorder of motor control, and that it is a focal dystonia.
Treatment of writers' cramp is unsatisfactory. A small number of our subjects
reported some benefit with chronic oral anticholinergic medication, despite side-
effects. A recent double blind study (Lang et ai, 1982), however, failed to show any
benefit in writers' cramp from intravenous administration of benztropine, atropine
or chlorpheniramine. Two patients noted that propranolol improved tremor. Most
subjects, however, preferred to take no medication, as writing was still possible or
because they had developed adequate alternative methods of communication, for
instance, typing, tape-recording. It may be of interest to note Gowers' findings in
1888 ' . . . small doses of strychnia... may be given as having an influence in the right
direction. Sedatives internally, as a rule, effect little in cases of pure cramp, although
hypodermic injections of atropine . . . have been strongly recommended by Vance.
Indian hemp by the mouth, morphia beneath the skin, or inunctions of extract of
belladonna and glycerine, or aconite ointment, are the most useful. Probably cocain
would also be useful in some cases'. He discounted the use of electricity and reported
the failure of tenotomy and immobilization by a plaster of Paris. One surgical
success has been recorded (Siegfried et at., 1969) when thalamotomy abolished what
the authors described as tremulous writers' cramp.
Finally, we turn to the psychiatric overtones attached to this condition. Perhaps
initially by accident, if not by misunderstanding when Gowers spoke of occupa-
tional neurosis and talked of re-education, behaviourists and their successors laid
claim to writers' cramp as either a psychoneurotic or psychosomatic condition. We
believe we have marshalled sufficient evidence to show that writers' cramp is a
dystonic illness and, hence, that psychiatric factors are either accidental or
secondary. The results of the PSE examination confirmed the absence of any excess
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478 M. P. SHEEHY AND C. D. MARSDEN
of psychiatric illness in this group of patients with writers' cramp compared to a
normal population, although they displayed just as many, if not more, of the minor
idiosyncrasies that differentiate us, one from another. Certainly, we have been
impressed by the dogged persistence that many of our patients have shown in their
search (over many years) for an adequate explanation of their symptoms. Although
we can give no cure for their disorder, at least we can agree that it is not caused by a
psychiatric illness.
As to the pathophysiology of the illness, this too is uncertain. There must exist
within the brain some mechanism for storage, retrieval and execution of the motor
programme responsible for an individual's characteristic script. The form in which
we sign our name or write is instantly recognizable, but is independent of the
muscles involved. Our signature is the same whether we execute it in the usual
fashion with hand and forearm muscles, or by writing with chalk or a felt tip pen on
a blackboard with proximal shoulder muscles; the same signature appears whether
we write normally or upside down, or even in the absence of gravity. All this must
indicate that the motor programme responsible for our script can operate whatever
the circumstances, by using which ever muscles are required. We sign our name with
the eyes shut and even with an anaesthetic hand (the fingers being bandaged to the
pen). The brain must contain a mechanism capable of generating the engram of our
script independent of which muscles are required, and of sensory feedback, so we
would look to some breakdown of this mechanism to explain writers' cramp.
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