Brain (1971) 94, 541-556.
PAINFUL LEGS AND MOVING TOES
BY
J. D. SPILLANE, P. W. NATHAN,1 R. E. KELLY AND C. D. MARSDEN
(From the Cardiff Royal Infirmary and the National Hospital,
Queen Square, London, W.C.I)
CASES
Mr. W. G., aged 48, had been referred to Rookwood Hospital, Cardiff, in 1959 because of
severe aching pain in both groins and of being unable to lift the left lower limb. He reported
that he had had backache for years. The leg pain had started suddenly, it was made worse by
coughing, when it would shoot down his left leg. No abnormal signs were recorded at that time,
apart from the circumference of the left calf being 2-5 cm. less than that of the right. A diagnosis
of sciatica had been made. The patient was treated by bed rest and traction. He recovered satisfactorily, became free from pain, and returned to his job as travelling bank representative.
1
Member of the External Scientific Staff, Medical Research Council.
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THERE is a syndrome of pain in the feet or lower limbs with spontaneous movements of the toes. The pain is a deep aching pulling pain: in most of the patients
there are other pains as well. The movements are always of the toes; they may be
present also in the feet, and exceptionally in more proximal muscles of the limb.
They are spontaneous and purposeless. The patient may be able to stop them when
he tries to but only for a few seconds; they then return despite his efforts. It is
impossible for a healthy person to imitate the movements; nor can a patient who has
them in one limb imitate them with the other. Flexion and extension, adduction and
abduction of the toes combine to cause a continual wriggling and writhing movement.
There is some difference from this basic pattern from patient to patient, though the
movements of any patient resemble those of others of this group rather than those
of any other condition. The pain and movements can be in one or both sides. We
have seen 6 patients with this disorder and from conversations with our neurological
colleagues, we have learned that many of them have seen one or two cases. The
condition is rare; had it been commoner it would have been described before.
A film of the movements of the toes was shown by one of us (J. D. S.) to the
Association of British Neurologists at their spring meeting in 1969. Photographs
of the condition are not presented in this paper as still photographs give no conception of the movement. Cine films of the patients are kept at the Cardiff Royal
Infirmary and at the National Hospital.
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J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
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Three years later, in 1962, he was seen in the Cardiff Royal Infirmary having had pain in his legs
for one year; this was quite unlike the previous pain in the back and lower limbs; it was unaffected
by moving, coughing or straining. On the right, the pain was only slight and came on only
occasionally. On the left the pain was "burning; throbbing, as if the leg were going to burst";
and "crushing." It was throughout the entire left lower limb, with a sensation of tightness in the
thigh. Within a few months of its onset, the pain was preventing sleep. The patient would go out
for a walk at night to try to tire himself enough so that he would fall asleep. At that time no
abnormal signs were found apart from 1 cm. of wasting of the left calf.
As the condition became worse, the patient was referred to one of us (J. D. S.) in 1964. There
were writhing movements of the left fourth and fifth toes. These spread to involve all the toes of
the left foot by 1965. The patient reported that he occasionally had them also in the second and
third toes of the right foot. In addition to the wasting of the left lower limb, vibration sense was
diminished in the leg. There were no other abnormal signs apart from the movements.
During the years 1964 to 1969 he was seen at regular intervals and admitted to hospital on many
occasions. The pains and toe movements persisted relentlessly; but no new signs appeared. The
possibility of a lesion involving the right thalamus and basal ganglia was considered. Lumbar air
encephalography in 1968 showed slight dilatation of the lateral ventricles and widening of cortical
sulci.
By 1969 the patient had lost 30 lb. in weight and insomnia was severe. The pain never ceased.
It was like "being crushed and being on fire." For a few minutes he would get relief by putting
the leg in cold water. It was made worse by walking but it was not relieved by rest. He was obviously
depressed. Drugs (sedatives, tranquillizers and anti-depressants) gave little or no relief.
The movement of the toes continued: purposeless, fanning, clawing and writhing. The patient
was aware of them and felt them against his shoe, but they troubled him little in comparison with
the constant severe pain. In general the patient was tense, depressed, tremulous and at times
tearful. He presented a picture of abject misery. The only other abnormal finding was a blood
pressure of 190/100.
Professor Rawnsley considered that his condition was due to organic disease and was not
psychogenic. This was also the opinion of all neurologists who saw the patient.
To treat the pain, it was decided to give the patient injections of phenol in iophendylate around
the posterior roots. Five injections between the third and fourth lumbar vertebrae were given.
They did stop the pain and movements; but the effect never lasted more than a few hours, the
longest duration being for one night.
The effect of blocking the tibial nerve with 1-5 per cent lignocaine was also tried. It produced
almost complete paralysis of the foot, and abolished the pain and movements. As so commonly
occurs with such injections of local anaesthetics, the effects far outlasted the paralysis and the loss
of sensibility on testing. The pain returned within eighteen hours and the movements in twenty-four
hours.
The patient was seen at the National Hospital in December 1969. He described the pain as like
a red-hot wire being dragged through the foot, like being burned: "but the pain is not in the skin,
it is deep inside." It was worse when he was resting in a chair and "intolerable" at night, preventing
him from sleeping. It was made worse also by walking and by radiant heat. The only thing which
relieved the pain was a hot bath. He told us that the pain had preceded the spontaneous movements
by some years. The movements of the toes continued when he first went to sleep, though they
stopped in deep sleep. There was some wasting of the left lower limb, the circumference of the
left thigh being 1 cm. less than the right, and that of the leg being 2-5 cm. less than the right. The
left ankle-jerk was diminished. Vibration sensibility was much reduced below the knees. Investigations were carried out over the years, many of them more than once, in Cardiff and in London.
The blood and CSF W.R. were negative; the CSF contained 80 mg. of protein per 100 ml. and the
CSF pressure was normal. Radiograms of the lower limbs and feet showed nothing abnormal,
PAINFUL LEGS AND MOVING TOES
543
Ischxmic Block of Left Lower Limb
An incidental point to be made was that the patient was quite definite in his opinion that the
pain of the blood pressure cuff inflated to 200 mm.Hg around his mid-thigh was far less than the
constant pain which brought him to hospital. The significance of this statement will be clear to
anyone who has had a cuff inflated at this pressure on a thigh; for this is a painful procedure and
requires some determination to tolerate the pressure for periods of over a minute or two. This
statement of the patient fully convinced us of the severity of the pain from which he was suffering.
The pain: his spontaneous pain continued throughout the thirty minutes of ischaemia. At the
end of that time, touches with cotton-wool were not felt below the knee, and sense of position was
absent in joints of the toes, foot and ankle. Pin-prick was not felt normally in the distal half of the
foot; repeated hard pin-prick and strong squeezing of the toes caused the typical burning pain that
occurs after half an hour's ischaemia.
From this test it was concluded that either the pain was not arising in the distal part of the limb
where it was felt, or else it was conducted to the spinal cord via non-myelinated fibres.
The movements: after twenty-ithree minutes of ischaemic block, the movements of the toes had
become less; but there now appeared similar movements of dorsi- and plantar-flexion of the foot
at the ankle-joint. This change in the spontaneous movements occurred at the same time as the
ability to perform voluntary movements went off; he could no longer move his toes but he could
still move his foot. By the twenty-eighth minute all movements, spontaneous and to command,
were absent in the toes and at the ankle-joint. Movements returned twenty-five seconds after
release of the cuff. As would be expected, they returned first in the foot and then in the toes.
This test showed that the movements were occurring in the long extensors and flexors and not
only in the small intrinsic muscles of the foot. It was striking how the activity of the large muscles
increased when that of the small muscles ceased. The spontaneous movements no longer occurred
when the somatic fibres were blocked. The test also showed that the movements were not the cause
of the pain, which persisted after the movements stopped.
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and that of the lumbar spine merely showed small marginal osteophytes at the borders of the
vertebral bodies. Dr. K. Howlett performed a myelogram on January 30, 1970. He reported
"free flow of contrast from the lumbar subarachnoid space to the foramen magnum, and back
again. There is narrowing of the spinal canal at the L4-5 level, with a small disc protrusion."
In June 1968, Dr. J. G. Graham had carried out an electromyographic examination of the left
lower limb. He had concluded that there was no electromyographic abnormality, stating "the
abnormal movements of the toes seem to arise from normal muscular volitional activity." In
December 1969 Dr. T. Heric found that maximum conduction velocity of the motor fibres of the
deep peroneal nerves was within normal limits (41 m/sec. right, 44 m/sec. left); he found a normal
latency for ascending volleys recorded at the head of the fibula on stimulation of the dorsum of the
foot (9-5 msec, on right), but the nerve action potentials were smaller than normal (1 to 2 nv).
On January 28,1970, the patient was re-examined by Wing-Commander Wynn-Parry, who reported
"at rest there are repetitive spontaneous discharges of units grouped in bursts of 8 or 9 at a rate
of 30/sec; this suggests irritation of the nerve." In 1968, a biopsy of the left sural nerve was
performed. Dr. D. G. F. Harriman examined the specimen and reported: "The nerve is for the
most part normal, with no changes in interstitial tissue and no sign of oedema or inflammation.
The myelin-stained sections do, however, reveal a few fibres only (three were noted) showing
Wallerian-type degeneration."
In addition to the syndrome of painful legs and moving toes, we considered that there was
evidence of prolapsed lumbar disc, which occurred first in 1959.
Other investigations were carried out to try and find out what group of fibres the impulses
giving rise to the pain were travelling in, to see if the movements stopped when the motor fibres
to the small muscles of the feet and toes were blocked, and to examine sympathetic nervous function.
544
J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
Drugs Affecting Movements
Diazepam.—Diazepam 10 mg. was given intravenously. It had no effect on the movements
or on the pain.
Investigation of the Sympathetic System
Further investigations were directed at examining the function of the sympathetic nerve supply
of the lower limbs, as we considered that this system could be playing a role in the syndrome.
Sweat test.—A sweat test was performed with the patient lying prone. Sweating was equal
and normal on the two lower limbs.
Drugs acting on the sympathetic system.—The effect of sympathomimetic and anti-adrenergic
drugs was examined. Adrenaline 1 mg. subcutaneously had no effect; adrenaline 0-5 ng. into the
femoral artery had no effect on the pain; it perhaps caused a slight increase in the movements.
Guanethidine 20 mg. intramuscularly and thymoxamine 5 mg. intravenously had no effect.
Block of left sympathetic chain.—The left lumbar sympathetic chain was blocked with 40 ml. of
bupivacaine hydrochloride 0-25 per cent. This, in effect, blocked both chains, for the temperature
of the left toes rose from 24°C. to 33-5°C. and of the right toes from 23°C. to 31-5°C. The block
caused some loss of sensibility in the left second and third lumbar dermatomes; but there was no
loss of sensibility below the knee, and the knee and ankle jerks were unchanged.
This block temporarily removed both the pain and the abnormal movements. At first the pain
went. The burning throbbing pain and the pain described as though the leg were bursting subsided;
a "bearable ache" in the shin remained. Ten minutes after the pain ceased, the movements stopped.
The pain went as full vasodilatation developed; the movements went off later. This was the first
time the movements had ceased for many years. The movements remained absent for about
three and a half hours; the pain remained absent for about four and a half hours. Blood-vessel
tone did not return for twelve hours.
Sympathectomy of Left Lumbar Chain
As we had already seen that two sympathetic blocks removed the pain and movements in another
patient, and had observed the satisfactory temporary result of the block in this patient, Mr. R. D.
Illingworth carried out a left lumbar chain sympathectomy. To the great disappointment of all of
us, the operation had no effect on the pain and none on the movements. The sympathectomy was
complete. The limb was obviously hot and skin vessels dilated; a sweat test showed no sweating
on the left.
Comment
The only suggestion we could offer to account for the success of the left sympathetic
chain block with local anaesthetic and the lack of effect of the left surgical sympathectomy, was that the anaesthetic had in fact blocked the sympathetic chain to
both lower limbs. Accordingly, after the operation, we attempted to block the right
sympathetic chain. We failed on two occasions; and so we asked Dr. D. J. Coleman,
our anaesthetist colleague, to try. He also failed. We concluded that the right
sympathetic chain probably lay on the ventral surface of the lumbar bodies; and that
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Cold pressor test and Valsalva manoeuvre.—The cold pressor test raised the blood pressure from
130/85 to 170/100 and also induced slight shivering. Neither it nor the Valsalva manoeuvre had
any effect on the pain or the movements.
PAINFUL LEGS AND MOVING TOES
545
was the reason why the local injection to the left chain had also blocked the right
chain. However, we were not sufficiently sure of this to recommend to the patient
to have another surgical sympathectomy on the right side.
The patient was admitted to the National Hospital in 1968, when the movements were not
restricted to the toes and foot, for contractions were occasionally occurring in the left tensor
fascias latae. The movements of the toes were rapidly repeated flexions and extensions with an added
rotatory element. There was no wasting of any muscle; all deep reflexes were equal and normal.
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Mr. F. T., aged 54, was referred to Dr. J. N. Blau at the National Hospital in 1966; we are
grateful to him for letting us investigate his patient.
The patient gave a history of having had several attacks of lumbago in the past. In some of
these the pain had radiated down the back of the left thigh. These had been acute episodes of pain,
which had come on when he was bending and which prevented him straightening his back.
When the patient came to hospital in 1966, he was complaining of a surging sensation in the left
leg, a discomfort rather than a pain, associated with spontaneous movements of the two middle
toes. He said that the trouble had started in 1964. At that time, he noticed a sensation in both
upper limbs and in the left lower limb. In the upper limbs it was slight feeling of numbness in the
fingers and hands. It gradually went off in the upper limbs, whereas the character of the sensation
in the left lower limb changed. At first it was merely a strange feeling, but then it became painful.
It was a feeling of intense pressure in the dorsum of the foot, a bursting feeling. After a few weeks
it became continual and in addition there was a throbbing, burning pain. Towards the end of 1966
it spread to involve the left calf. He had no symptoms in the right lower limb. These sensations
were quite unlike the pain he had had with lumbago, and they were not influenced by changes in
position of his back or lower limbs.
In 1967 he came up to hospital to report that he had developed spontaneous involuntary up and
down movements of the third and fourth toes of his left foot.
Since 1968 his state has been as follows: He would get up in the morning with the left lower
limb feeling numb or dead from the knee downwards. Within an hour spontaneous movements
would start in the fourth and fifth toes. At the same time a deep pressure pain would be felt in
the lateral part of the dorsum of the foot, including the lateral three toes. There was also an ache,
a sensation of tightness in the calf, a pulling pain along the dorsum of the foot and in the ankle,
and a deep burning and pricking in the foot. When the pain was most severe, the calf would feel
subjectively numb. The pain was unaffected by movements of his back, by straining, crouching,
sneezing, defalcation or micturition. It was made worse by pressure on the posterior part of the
thigh just above the popliteal fossa or by pressure on the foot in walking or standing. This was so
important a factor that he had changed his car to an automatic one so that he did not have to use
his left foot on the pedals. The pain was also worse when the patient was upset. He found that his
pain was less with exercise; keeping still was the worst thing for it. Other relieving factors were
removing his shoe, lying down with the foot raised, putting the foot in very hot or very cold
water, squeezing his foot with his hands, or the following manoeuvre: he would lie flat on the
ground on his abdomen, with the foot in full plantar flexion and try to relax. He told us that the
movements of the toes were correlated with the severity of the pain: when his toes were moving
very fast, he would get more severe pain "because the toes are pulling"; when there was no pain,
there were no movements. When he was asked to choose between the alternatives of the movements
starting the pain or the pain starting the movements, he considered that the movements caused
the pain. However, even when the movements stopped, he still had an unpleasant pulling pain
and a "feeling as if the whole foot is alive." During the previous three to four months, he had
been getting cramps at night. The toes would flex strongly and involuntarily; this would stop the
involuntary movements.
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J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
Block of Left Sympathetic Chain
When the patient was an in-patient in 1968, Dr. Blau asked one of us (P. W. N.), if he had any
ideas for the relief of this pain. As the pain was of causalgic type, it was suggested that the lumbar
sympathetic chain should be blocked with a local anaesthetic. Dr. Atwood Beaver did this; in fact
both lumbar chains were blocked; the temperature of the toes rose from 29-5°C. to 35°C. on the
right and 36°C. on the left. As this was the first of these patients in whom we did a sympathetic
block, we were amazed to find that this block removed not only all the pain but also the abnormal
movements. The removal of both disturbances occurred while the skin temperature of the feet
was rising. These effects lasted for a month although the anaesthetic was a short-acting one (2 per
cent procaine). The pain then returned, building up to its previous intensity by the end of a week.
The movements recurred three weeks later. The sympathetic block with procaine was repeated and
again it abolished the pain and movements, this time for a week. The symptoms returned, but
over the next year the pain gradually decreased. After this, the patient had long periods without
pain, and when the pain was present, it was less severe. During the periods with slight or no pain,
the movements stopped. From the end of 1969 there were no more movements.
In April 1970 the patient was faced with a lot of extra work and also with increased family
responsibilities as his brother-in-law suddenly died of a coronary thrombosis. This situation
precipitated the patient's pain and movements once more; but both cleared up after a few
weeks.
Comment
It is clear in this patient that once the syndrome had developed, it was precipitated
by psychological stress and would tend to recover slowly when he could cope with
his life situation satisfactorily.
We are also faced with the question why blocking the sympathetic chain stopped
the movements, and further, why this effect lasted for a week or more, when the block
itself lasted for only two hours.
Mr. E. B., aged 54, was referred to one of us (R. E. K.) in 1963 on account of involuntary
movements of the toes of the right lower limb and cramps. At the age of 20 the patient had fallen
off a horse and injured his back. Ten years later he had had an attack of backache with pain in
the right hip and thigh. One day, two years later, he suddenly found he was unable to rise from
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The blood and CSF W.R. were negative. The CSF contained 1 lymphocyte per c.mm. and
30 mg./100 ml. of protein. The pressure was 170 mm. of water and there was a free rise and fall.
Plasma potassium, sodium and urea were within normal limits. Radiograms of the lumbar spine
were reported by Dr. J. Campbell as showing a reduction of the disc spaces between L4 and L5
and between L5 and SI. Dr. J. P. Patten carried out nerve conduction examinations. He found a
maximum motor conduction velocity in the peroneal nerves of 47 m/sec. on both sides (normal).
Nerve action potentials recorded at the head of the fibula on stimulation of the dorsum of the foot
were normal in size (5 (iv. right and left) and latency (7 msec, right, 7-6 msec. left).
In March 1970 the patient slipped and fell, landing on his neck. Within four days he developed
typical sciatica in the right lower limb. Lasegue's sign was positive and the right ankle-jerk was
absent. Neck movements were limited by pain. Dr. Campbell reported on the radiograms as
follows: "Cervical spine: all disc spaces below C2/3 are slightly narrowed. All vertebral bodies
below C2 show anterior and posterior osteophyte formation. There is encroachment on all intervertebral foramina from C3 inferiorly on both sides. The neural canal is narrowed to a little below
the normal range opposite the C5/6 and C6/7 interspaces. Conclusion: severe cervical spondylosis.
Lumbar spine: the disc spaces at L3/4, L5/S1, and probably L4/5 are narrowed."
PAINFUL LEGS AND MOVING TOES
547
When investigated in 1969, the CSF contained 60 mg. of protein per 100 ml. and 1
cell per c.mm., and the W.R. was negative. A myelogram showed indentation of the column
of opaque dye on the right at the level of the fourth and fifth lumbar disc space, thought to be due
to a disc protrusion. In October 1969 Mr. Lindsay Symon explored this region and found a large
mass of fibrous tissue in the neighbourhood of the fifth lumbar root. On histological examination,
the tissue was found to be degenerate fibrocartilage and pieces of mature bone. There was no
evidence to suggest an arachnoiditis due to the phenol injections.
This operation cured the sciatic pain. The movements, and the pain associated with them,
stopped for many weeks, but eventually recurred. The patient then returned to hospital. In July
1970 Dr. R. Wise injected the right sympathetic chain putting in 10 ml. of 0-5 per cent lignocaine
and 0-25 per cent bupivacaine in the region of the second, third and fourth lumbar ganglia. The
movements stopped for three hours; the pain ceased for three hours and was much less for twentyfour hours. Following this, 3 ml. of 6-7 per cent aqueous phenol solution was injected into each of
the previous sites. The movements were much reduced and the pain was abolished for three weeks;
it then slowly returned.
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sitting; this was due to stiffness rather than pain. In the next few days he developed weakness of
the muscles of the right thigh with pain in the thigh and leg. He then spent nine months in hospital
being treated for backache, pain and weakness. Since that time he had had occasional attacks of
right-sided lumbago and sciatica. At the age of 46 he had a lumbar spinal fusion on account of
these recurrent attacks.
In 1961, at the age of 51, he developed involuntary movements of the right toes sometimes
associated with cramp-like pain. At first they affected any single toes or all the toes together. They
consisted of a continual flexion and extension.
In 1964 he saw a neurosurgeon, who performed neurectomies of the digital nerves. This stopped
the movements for one week; then they recurred as frequently as before. When the patient was
seen in 1965, the movements were still present. By then they had become accompanied by constant
and severe pain; the patient described this as a"tight band-like pain around the legs, a pulling feeling
as though the muscles were being used in a continuous tug of war" and as "burning" and "bursting."
It was always made worse by walking but it was not relieved by rest; he had noticed no other
aggravating or relieving factors. He insisted that this pain was totally different from his previous
sciatic pain. There was slight weakness of dorsiflexion of the right toes but no other abnormal
physical signs.
As it was considered that nothing would be likely to stop the involuntary movements, efforts
were concentrated on trying to stop the pain. On three occasions, injections of procaine around the
fifth lumbar and first sacral roots stopped the pain and the involuntary movements for two to
three hours. It was, therefore, decided to inject 0-3 ml. of a 5 per cent solution of phenol in glycerol
around the roots. This had the same effect as the procaine—complete abolition of the pain and
movements. Between that time and 1969 these injections were repeated at intervals between two
and nine months, depending on the return of the pain and involuntary movements. With these
injections of phenol, the involuntary movements stopped only when the injection caused some
transient weakness of dorsiflexion of the foot and toes. The pain and movements always returned
together, reaching a maximum within twenty-four hours. When the movements returned in July
1969, they had spread so that they involved the ankle as well as the toes. On this occasion the
injection of the phenol solution around the fifth lumbar and first sacral roots had no effect; and
so it was necessary to inject further quantities around the third and fourth lumbar roots. This
caused a flaccid paralysis of the leg and retention of urine for a week. It stopped the pain and
involuntary movements. By this time the patient had had 12 injections of phenol; there was
moderate weakness of dorsiflexion of the toes and foot and slight weakness of plantar flexion,
inversion and eversion of the foot. The right ankle-jerk was absent and there was slight diminution
of sensibility in all lumbar dermatomes.
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J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
The patient returned on October 24,1970, with "insupportable" pain, asking for another chemical
sympathectomy. The right lower limb was still warmer than the left. The injection with aqueous
phenol was repeated as before. There was immediate relief of the pain and movements. The
movements have recurred but they are now only in the toes. There are intermittent periods free
from movements lasting for five minutes. The pain has not yet returned three months after the
phenol block.
Mrs. M. H. H., aged 67, was referred to Dr. J. N. Blau, to whom we are grateful, at the National
Hospital in July 1966.
One day in March 1965 the patient developed constant tingling and pricking in both feet; they
were in the toes and the distal halves of the feet. The sensations were deep inside the foot and toes
and not in the skin. The feeling would come on within five to ten minutes of waking, regardless of
whether she remained in bed or got up and walked about. Later it would often be present when
she woke up. Since its onset, she had never had a day without it.
Six months after the beginning of the paraesthesise, involuntary movements of the toes started.
These became painful. When she was first referred to the hospital, it was on account of the
movements. But as time passed, the movements became unimportant compared with the pain.
The patient had always been healthy, apart from minor complaints.
The patient was admitted to the National Hospital about eighteen months after the onset, in
July 1966. The pins and needles were better when she was walking or standing, though her feet
then became numb. At first she had had a sensation as though her toes were moving although they
were not moving. Six months later she was surprised to see that the toes were actually moving "as
though they were playing a piano on their own." These movements were accompanied by dragging
and pulling sensations in the toes and sharp jabs of pain in the big toe. For a few months before
admission to hospital, the movements diminished, but the parsesthesiffi and pain which at first
accompanied them continued and became worse. The pulling and dragging sensations became
painful. She also described these sensations as a painful throbbing.
Dr. J. Newsom Davis examined her then and described the movements as follows: "The movements are occurring at a rate of 2/sec. and involve the right toes more than the left. On the right,
the movement is mainly offlexionof the middle toes with abduction and adduction of the little toe;
there is slight inversion of the foot. On the left the movement is almost confined to the second toe.
The movements cannot be stopped voluntarily, but they do stop when the limb is used, as in performing the heel-shin test. They also diminish when the arms are used." He found no other abnormal signs in the nervous system. The blood and CSF W.R. were normal. The CSF pressure was
normal; it contained no cells and 45 mg. of protein per 100 ml. An air encephalogram was normal.
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Comment
The intrathecal injections of phenol solutions around the lumbar roots would not
have affected any sympathetic efferent fibres. These solutions affect all nerve fibres,
motor and sensory, with which they make contact for an adequate length of time.
The solutions would have caused degeneration in a large number of delta fibres of
the A group and non-myelinated fibres, because these are the most numerous fibres
of the spectrum of the posterior roots.
Although a surgical sympathectomy failed in the first case (after block with local
anaesthetic had stopped both pain and movements), in this case the repeated blocks
of the sympathetic chain with destructive solutions of phenol stopped the pain and
movements on every occasion.
PAINFUL LEGS AND MOVING TOES
549
Dr. Shah found radiological evidence of degenerative changes in the cervical spine but the lumbar
spine was normal. Electromyographic examination showed normal motor and afferent conduction
velocities in both peroneal nerves.
Drugs Affecting Movements
Diazepam.—Only 12 mg. of diazepam were given intravenously as the patient could not keep
awake when this amount had been injected. The movements stopped, before she fell asleep, at
the stage when she was talking in a slow sleepy way; at this time she could still move her toes to
command. She was left to sleep for twenty minutes and then woken. At this time all tendonjerks were absent. She moved her toes normally to command, and the spontaneous movements
had not returned. Five minutes later she was fully awake and making conversation. The jerks
were present on reinforcement. She felt as if the toes were moving and she had the usual
paraesthesias, but no movements were present.
Ischcemic Block of Right Lower Limb
The patient found the pain of the cuff far more severe than the pain that brought her to hospital.
The pain: her pain continued throughout the thirty minutes of the ischaemia: At the end of
that time, touches with cotton-wool were not felt in the distal half of the foot and sense of position
was absent in the toes. Pin-prick was not felt below the upper third of the leg. Squeezing the foot
gave her no pain.
The movements: by the twenty-seventh minute, the involuntary movements had stopped,
though she could still move the toes to command. This was the same at the thirtieth minute
when the cuff was released. It must be pointed out that the involuntary movements were minimal
on this day. Within twenty seconds of the release of the cuff, the involuntary movements returned.
From that time and for a further fifteen minutes, they were far more than before, of greater
amplitude and involving movements at the metatarsophalangeal joints as well as at the interphalangeal joints. The patient, on experiencing post-ischaemic paraesthesiae, confirmed that the
tingling and pricking were identical with her spontaneous paraesthesiae.
Thus, thefindingswere the same as those of the previous patient: the spontaneous pain continued
although superficial and most deep pain sensibility was absent from the foot; the spontaneous pain
remained after the movements ceased. The movements ceased when the large efferent nerves were
blocked.
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An electromyographic examination was carried out by Dr. R. J. Burns. He found the conduction
velocities of the motor and sensory fibres of the right peroneal nerve were normal and he concluded
that there was no positive evidence of peripheral neuropathy.
She was readmitted in April 1970, so that we could find out the effect of blocking the sympathetic
chains. At this admission, it was found that when her legs were rubbed or stroked, she got a
pricking sensation, as when one rubs the digits during post-ischaemic paraesthesiae; this was confirmed by giving the patient post-ischaemic paraesthesiae and then rubbing the toes. Sense of
position and of movement and two point discrimination were normal. Pricking the feet and toes
made the involuntary movements more vigorous. There were constant movements of the toes of
both lower limbs; they resembled those of Mr. W. G. rather than those of Mr. F. T. They were a
kind of slow quivering movement; they were not quite continual for there was a second or so every
now and then without any movements. They were more up and down than rotatory or side to
side, more marked on the right than the left, and more in the four lateral toes than the big toes.
On the right, when the movements were maximal, the big toe moved up and down together with
the other toes; on the left its movements were not synchronized with those of the other toes. When
they were maximal on the right, the whole distal half of the foot moved up and down, this movement occurring at the metatarsotarsal joints.
550
J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
Investigation of the Sympathetic System
Sweat test.—There was slight sweating on the soles of the feet, equal in amount in the two feet;
it was within normal limits.
Coldpressor test and Valsalva manoeuvre.—The cold pressor test raised the blood pressure from
120/75 to 140/90. There was no effect on the pain or movements. The Valsalva manoeuvre had
no effect on the pain or movements.
Failed block of left sympathetic chain.—This procedure was given up as CSF was obtained from
one of the needles, and it was considered dangerous to inject substances in the neighbourhood of a
hole in the dura mater. This block failure is worth recording, however, as the patient did not know
it had failed. If the effects on movements and pain obtained before had been psychogenic, the same
effects would have followed this block, but they did not; there was no change of any sort in movements or pain.
Block of the left sympathetic chain.—One week later, at 4 p.m., the left sympathetic chain was
blocked with lignocaine 0-5 per cent and bupivacaine 0-25 per cent. The temperature of the left
toes rose from 24° C. to 31° C. and that of the right rose from 23-5° C. to 24° C. Again, there was
no immediate effect on the pain and movements. On the next morning, however, there were no
spontaneous movements and the pain was far less than it had been before. This effect lasted
twenty-four hours.
Comment
Had this been the only patient in whom a block of the sympathetic chain appears
to have had some effect, we would have dismissed the findings as irrelevant, but as
the blocking of the lumbar chain in the three previous patients had had marked
effects both on the pain and movements, it seems likely that these transitory effects
coming on during the night after the block were due to the sympathetic block.
Miss H. N., aged 68, was referred to one of us (J. D. S.) in February 1966. She complained of
pain in both feet of two years' duration and of twitching movements of the toes for three years.
As with Mrs. M. H. H. they were not at first present when she woke in the morning, but later
they started as soon as she woke; she felt them going on inside her shoes all day. About a year
after the movements started, she developed a burning pain in both feet and all toes. The pain was
never present on waking; it became progressively worse during the day. When she went to bed
at night, both the movements and the pain went off. The movements were constant during the day
and ceased when she slept. They consisted of a mild extension and abduction of the toes. When
they were slight they were only in the fourth and fifth toes, but when they were maximal, they
involved all the toes, still being most pronounced in the fourth and fifth toes. The pain was a
constant aching in the feet, which came on soon after the toes started to move in the mornings.
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Block of right sympathetic chain.—The right sympathetic chain was blocked with a 0-5 per cent
solution of lignocaine at 4 p.m. The temperature of the right toes rose from 31° C. to 36° C ,
whereas that of left toes rose from 29° C. to 30° C. This block had no immediate effect on the pain
or movements. However, on the following morning she awoke without pain, and the movements
were minimal. Pain came on later during the day. The movements, however, remained either
minimal or absent for seven days. This was quite different from before the block.
One week after the block of the right sympathetic chain, we decided to try the effect of a similar
block to the opposite, left sympathetic chain.
PAINFUL LEGS AND MOVING TOES
551
The degree of pain was directly related to the amount of the toe movements. Apart from the
involuntary movements of the toes, no abnormal physical signs were found in the lower limbs.
The blood pressure was 170/110. It was confirmed that the movements ceased during sleep and
were constant by day during her two weeks' stay in hospital. The electroencephalogram was
normal. Radiograms of skull, spine and limbs were normal. The CSF was not examined. Serum
folate was 2-6 y-g./ml.; serum B12 was 183 (i^g./ml. Many drugs were tried, without benefit,
including benzhexol, sodium amytal, chlorpromazine and intravenous propanolol. Folic acid
5 mg. daily had no effect. Intravenous diazepam—10 mg.—had no effect on pain or movements.
Since 1966 there has been no change, but she is becoming more anxious and
depressed.
The toes of the left foot were slightly clawed compared with those of the right. When first
examined there were spontaneous intermittent clawing movements of the left second and third
digits, but the patient said that the movements often involved the remaining toes and were usually
more obvious.
The patient was admitted to the Cardiff Royal Infirmary. The movements were present whenever
he was examined. It was confirmed that when the movements were maximal, they were in all five
digits. The main movements were flexion and fanning, and appeared somewhat athetoid. They
were not constant; being absent for a few moments. They ceased during sleep. They varied in
amplitude from day to day. When they were most active his pain was worse. The pain varied much
less than the movements.
Apart from the involuntary movements, no other abnormality was found. The patient was
inclined to be depressed. Diazepam 5 mg. t.d.s. abolished the movements within twenty-four hours,
but did not reduce the pain.
A biopsy of the left sural nerve was done in 1968. Dr. D. G. F. Harriman examined the specimen.
He reported that "with single nerve dissection, two fibres out of sixteen showed segmental
demyelination, and two showed short internodal segments."
Between February and July 1968 courses of diazepam and dummy tablets were given to the
patient, the former practically abolishing the toe movements, the latter having no effect. The pains
persisted and anxiety with depression became more apparent. His sleep remained untroubled.
He carried out his work as a Welfare Officer fairly regularly. During a second hospital admission
in July 1968 it was thought that the left ankle-jerk was impaired.
Comment
As in the second case, there was slight evidence of a lesion of the nerves of the
lower limb, but there was no evidence of this in the other cases.
24
BRAIN—VOL. xcrv
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Mr. A. P., aged 53, was referred to one of us (J. D. S.) in 1967, on account of pain and involuntary
movements of the left foot for one year. They started as "an itching inside the foot." Over a period
of months, there became a constant discomfort and then turned into "a torment." They varied
from day to day in severity, never troubling him at night. During the daytime "it's affecting my
concentration, it's upsetting me, it's ruling my life; in the evenings I may have to stay at home—
it's depressing; and it's affecting my work." He got temporary relief by bathing the foot, holding
the foot in his hands, pressing on the sole, wearing arch supports, and putting the foot on a cold
floor. Seeing that a cold floor helped, the patient even tried putting his foot in a refrigerator. There
was also a sharp pain in the sole of the foot which came on when the movements were marked,
and brought on by walking.
552
J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
These 2 patients were seen by one of us (J. D. S.) before the other authors had
seen their cases. At that time we had not yet proposed a hypothesis of there being an
association of this syndrome and the sympathetic nerves or nerves running with the
sympathetic chain, and no investigations of this system were therefore carried out.
FEATURES OF THE SYNDROME
TABLE
Patient
Sex
Age at onset
W. G.
Male
52
F. T.
Male
52
E. B.
Male
51
M. H.
Female
66
H. N.
Female
68
A. P.
Male
53
Duration of syndrome
Pain
Movement
8 years
6 years
6 years
3 years
9 years
9 years
5 years
4i years
6 years
7 years
4 years
4 years
Lower limbs affected
Left
Left
Right
Both
Both
Left
Description of pain
Burning
throbbing
bursting
crushing
Pressure
throbbing
burning
bursting
Cramp
pulling
burning
bursting
Pulling
dragging
throbbing
Burning
aching
Torment
sharp
Factors aggravating pain
Walking.
Pressure over
Sitting.
sciatic nerve
In bed at night or on feet
Walking
Nil
Activity and
walking
Activity and
walking
Factors relieving pain
Hot bath.
Cold water
Nil
Nil
Nil
Cold
Pressure
Hot or cold
water.
Activity.
Raising foot
History of lumbago and/or
sciatica
Yes
Yes
Yes
No
No
No
Associated depression
Yes
Yes
No
No
Yes
Yes
Description of movements
Ext. and abdn.
Writhing of toes Rapid rotatory Flex, and ext. Slow
of toes. Tensor toes and ankle writhing of toes oftoes
fasc. lat.
Fanning and
flex, of toes
Radiological
Small disc
prolapse at
L4/5
CSF
INVESTIGATIONS:
Narrow disc
spaces L4/5,
L5/S1
Disc prolapse
at L4/5
Normal
lumbar spine
Normal spine
Normal spine
Protein
Normal
80mg./100ml.
Normal
Normal
Not done
Not done
EMG
Size of
Normal
peroneal
afferent
potential dim.
Normal
Normal
Not done
Not done
Sympathetic block and local
anesthetic
Abolished pain Abolished pain Abolished pain Abolished pain Not done
and
and
and
and
movements
movements
movements
movements
Not done
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The features of the syndrome are summarized in the table. All 6 patients gradually
developed pain and involuntary movements in the lower limbs, mainly in the toes,
which have persisted for periods between three and nine years. Both lower limbs
were involved in 2 patients, one limb in the other 4. The syndrome was not associated
with any consistent abnormalities found on neurological examination or on
investigation. Neither the pain nor the movements were typical of any known
disorder.
PAINFUL LEGS AND MOVING TOES
553
The Movements
The movements occurred in the distal parts of the limbs, the toes being affected
in all patients. When the movements were more marked, they involved more
proximal joints; in one patient they were seen in the thigh muscles. They consisted
of flexion and extension, adduction and abduction of the phalanges. In general
terms, one can describe them as sinuous clawing and re-straightening, fanning and
circular movements of the toes. When they were minimal, they were a slow quivering.
In most cases the movements were continuous. When intermittent, the movements
lasted for seconds and the gaps of quiet between the movements lasted for minutes.
The total pattern of the movements was a waxing and waning. The muscles contracting
do not vary from moment to moment and so the movements cannot be classified as
choreiform. The patient cannot imitate the movements of one limb with the other
limb, nor can he, during periods of quiescence, imitate the movements with the toes
and foot of the affected limb. Some of the patients can stop the movements by an
effort of will; but they start again when attention is distracted. Others had more
intense movements of larger amplitude when they paid attention to them. As with
most involuntary movements, they were more apparent when the patient experienced
stress or pain but they disappeared during deep sleep.
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The Pain
It is clear that all the patients described the pain in the same way and were
describing the same kind of pain. It varied in intensity from a discomfort to a pain
of great severity, "a torment." It had the character of a constant deep pain. It was
described as an ache, an intense pressure, a tightness, a feeling that the toes were
pulling or being pulled, a throbbing, bursting, crushing; it could also be accompanied
by a deep burning "as if it is on fire." The pain was neither sharp nor shooting,
and in no way resembled a nerve root pain; nor was it confined to dermatomes or
myotomes. It was felt deeply in the limb although it was not felt especially
in the flexor or extensor groups of muscles. The 3 patients who had previously
had sciatica said that the pain associated with the moving toes was quite unlike
the sciatic pain. It did not occur in acute attacks nor was it affected by sneezing,
coughing, defalcation or bending. It could be severe, at times as severe, it seems,
as the pain of post-herpetic neuralgia or migrainous neuralgia. The only
factors found to relieve it were immersion of the limb in hot or cold water and
local pressure. On the whole, the pain was in proportion to the extent of the
movements: and the patients had the impression that the movements caused the
pain, yet our investigations showed that abolition of the movements did not
abolish the pain.
The severity of the pain, the lack of effective relief and of understanding of the
condition all contribute to making a number of the patients depressed. As with
other painful states, it is a help to the sufferers to realize that their physicians know
the condition and that the pain they suffer is both genuine and severe.
554
J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
/Etiology
We have found no single cause of the syndrome. Attention can be drawn to the
fact that 3 of the 6 patients gave a history of lumbago and sciatica and had evidence
of damage to the lower lumbar intervertebral discs. In 2 of the patients in whom a
biopsy sample of a peripheral nerve was examined, there were some degenerated
nerve fibres.
DISCUSSION
The most obvious evidences of this syndrome are the movements. Yet, as there
are also painful sensations in the lower limbs, physicians seeing these patients consider that they are examples of Ekbom's restless legs syndrome. In that condition
there are sometimes involuntary movements, but they are not of the toes; they
consist of myoclonic jerks, occurring only when the patient is in bed and they may
interfere with sleep. In Ekbom's syndrome, the patient feels constrained to move
his limbs in an attempt to get relief from the sensations in his feet and legs; for
moving the limbs affords the patient some relief. In the present syndrome, exactly
the opposite occurs. The movements are involuntary; the patient longs to be
able to keep his toes still. Far from the movements seeming to the patient to relieve
the sensations, these patients notice a proportional relation between the degree of
the movements and the amount of the pain. Patients with Ekbom's syndrome,
when asked to say whether their sensations should be considered as a discomfort
or a pain mostly state that they are a discomfort, although a few of these patients
do say that they have a deep aching pain, and very few say that they also have
a burning sensation in the soles. It may well transpire that when further knowledge
of the pathology of the two syndromes is obtained, they are found to be
related.
In the present syndrome, no disorder of the sympathetic nerve supply to the part
was found; and yet it appears that there is some relationship between the pain and
movements and the sympathetic system. In all 4 patients in whom the lumbar
chain was blocked with a local anaesthetic or with phenol solution, there was great
diminution or abolition of the movements and pain. These two symptoms were not
blocked for the same duration, an indication that their genesis is different and that
the movements can occur without the pain. In view of the tests showing normal
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In addition to the 6 patients reported here, we know of 3 other patients with the
same syndrome. One of us has seen one patient in whom similar movements were
present in one upper limb, but there was no pain. The movements in this case were
restricted to the fingers of the right hand. They were not continuous but were brought
on by attempts to maintain a posture or to carry out purposeful movements. The
movements were flexion, extension, adduction and abduction of the fingers. We
are, however, disinclined to regard this single case as being an example of the
syndrome under discussion.
PAINFUL LEGS AND MOVING TOES
555
sympathetic function, it must be concluded that the blocks of the sympathetic chains
work by blocking afferent nerve fibres running with the chain.
It is more difficult to see how the sympathetic nerves could be related to the involuntary movements. There is, as far as is yet known, no condition in which muscles
are induced to contract by activation of sympathetic nerve fibres to the muscles,
their spindles or their blood vessels. It seems more plausible to suggest that the
movements are due to the input responsible for the pain. Any evidence that there
is some relation between the syndrome and afferent nerves running with the
sympathetic chains is at present inadequate; it is a hint for the further investigation
of these patients.
SUMMARY
A new syndrome is described consisting of pain in the lower limb associated with
spontaneous movements of the toes. The pain varies from a constant discomfort
to an intractable torment. The movements affect the phalanges more than the foot.
They consist of a clawing and straightening, fanning and circular movements of the
toes. The patient may be able to stop the movements when he concentrates on doing
so; they start again when his attention is distracted from them.
In 4 patients in whom the lumbar sympathetic chain was temporarily blocked by
local anaesthetic, the pain and movements were stopped or reduced for some
hours.
ADDENDUM
After this paper had been accepted for publication, our colleague Dr. M. R. Dimitrijevic of
Ljubljana sent us the notes of a further example of this syndrome that he had previously discussed
with us.
This patient, who previously had had three operations for herniation of a lumbar intervertebral
disc, developed the pain and movements in one leg. Injections of bupivacaine into the sympathetic
chain removed both the movements and the pain; the effect lasted twelve hours. In view of
this temporary therapeutic success and of our experience with blocking the sympathetic chain, a
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Although the sympathetic system is defined as an efferent system, non-sympathetic
small afferent nerve fibres from the viscera run in the sympathetic chains on their
way to the spinal cord. Any blocking or removal of the sympathetic chains removes
this input from the viscera to the spinal cord. It has recently been shown in physiological investigations in the cat by Pomeranz, Wall and Weber (1968), Selzer and
Spencer (1969a and b) and Fields, Meyer and Partridge (1970) that the afferent
fibres from the viscera end on the same neurons as do the delta fibres from the skin,
and many of these neurons respond to both somatic and visceral input. These
visceral afferents may not be directly sensory but may be, as Fields et al. state,
"purely propriospinal." There is, therefore, good reason for stating that blocking
the sympathetic chain removes a part of the total input to the caudal part of the
spinal cord.
556
J. D. SPILLANE, P. W. NATHAN, R. E. KELLY AND C. D. MARSDEN
sympathectomy was performed, the second, third and fourth lumbar ganglia with the chain
between them being removed. This operation removed the pain and the movements for only
three days; both then recurred as before.
The feature of particular interest in this patient is that, as in our first case, the chemical block of
the sympathetic chain stopped the pain and movements for several hours. But surgical removal of
the chain and ganglia had no lasting effect.
REFERENCES
FIELDS, H. L., MEYER, G. A., and PARTRIDGE, L. D., Jr. (1970) Convergence of visceral and somatic input
onto spinal neurons. Expl Neurol., 26, 36-52.
POMERANZ, B., WALL, P. D., and WEBER, W. F. (1968) Cord cells responding to fine myelinated afferents
from viscera, muscle and skin. / . Physiol., Lond., 199, 511-532.
,
(19696) Interactions between visceral and cutaneous afferents in the spinal cord: reciprocal
primary afferent fiber depolarization. Brain Res., 14, 349-366.
{Received 6 January 1971)
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SELZER, M., and SPENCER, W. A. (1969a) Convergence of visceral and cutaneous afferent pathways in the
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