the causalgia—dystonia syndromedystonia always appeared at the same time or after the causalgia....

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Brain (1993), 116, 843-851 The causalgia—dystonia syndrome K. P. Bhatia, M. H. Bhatt and C. D. Marsden University Department of Clinical Neurology, Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, UK SUMMARY We report 18 patients (16 women and two men) with causalgia and dystonia, triggered by peripheral injuries in 15 cases and occurring spontaneously in three. The injury was often trivial, and did not cause overt peripheral nerve lesions. The mean age at presentation was 28.5 years. None had a family history of dystonia. The leg was affected initially in 12 patients, the arm in the remaining six cases. All had burning pain, allodynia and hyperpathia, along with vasomotor, sudomotor and trophic changes. All developed dystonic muscle spasms in the affected part. Dystonia always appeared at the same time or after the causalgia. The spasms were typically sustained, producing a 'fixed' dystonic posture, in contrast to the mobile spasms characteristic of idiopathic torsion dystonia. There was spread of the causalgia and of the dystonia from its initial site both in the affected limb and to other extremities, the latter in hemiplegic, transverse and triplegic distribution. All investigations were normal. All modes of conventional treatment failed to relieve either the pain or the dystonia, but two patients recovered spontaneously. At present it is impossible to decide whether this distressing syndrome is a true functional disorder of the central nervous system, or is of psychogenic origin. INTRODUCTION Causalgia, that occurs in reflex sympathetic dystrophy (sometimes termed Sudek's atrophy or sympathetic algodystrophy), is a terrible pain which is often untreatable (see Schott, 1986a). Causalgia has proved difficult to define. Literally it means a burning pain, but historically it has been associated with peripheral nerve lesions. Thus, the International Association for the Study of Pain (IASP) has defined causalgia as 'a syndrome of sustained, burning pain after a traumatic nerve injury, combined with vasomotor and sudomotor and later trophic changes' (Merskey, 1986). However, causalgia may occur without overt nerve injury, so we have employed the term in the sense used by Schott (1986a) (see below). The pathophysiology of causalgia is uncertain. Implicit in the term reflex sympathetic dystrophy is the notion that overactivity of the sympathetic nervous system is involved (Bonica, 1979). This is based upon the observation that sympathetic blocks of the affected limb may give temporary relief. But surgical sympathectomy rarely provides a cure (Ochoa, 1991). Indeed, recently it has been suggested that a substantial part of the benefit of sympathetic block, by whatever means, is due to a placebo effect (Verdugo and Ochoa, 1991). No abnormality of sympathetic neuronal discharge has been found in affected limbs on microneuronography (Wallin et al., 1976; Torebjork and Hallin, 1979). Correspondence to: Professor C. D. Marsden, Institute of Neurology, Queen Square, London WC1N 3BG, UK. © Oxford University Press 1993 by guest on November 1, 2012 http://brain.oxfordjournals.org/ Downloaded from

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Page 1: The causalgia—dystonia syndromeDystonia always appeared at the same time or after the causalgia. The spasms were typically sustained, producing a 'fixed' dystonic posture, in contrast

Brain (1993), 116, 843-851

The causalgia—dystonia syndromeK. P. Bhatia, M. H. Bhatt and C. D. Marsden

University Department of Clinical Neurology, Institute of Neurology and National Hospital forNeurology and Neurosurgery, Queen Square, London, UK

SUMMARY

We report 18 patients (16 women and two men) with causalgia and dystonia, triggeredby peripheral injuries in 15 cases and occurring spontaneously in three. The injury wasoften trivial, and did not cause overt peripheral nerve lesions. The mean age at presentationwas 28.5 years. None had a family history of dystonia. The leg was affected initiallyin 12 patients, the arm in the remaining six cases. All had burning pain, allodynia andhyperpathia, along with vasomotor, sudomotor and trophic changes. All developed dystonicmuscle spasms in the affected part. Dystonia always appeared at the same time or afterthe causalgia. The spasms were typically sustained, producing a 'fixed' dystonic posture,in contrast to the mobile spasms characteristic of idiopathic torsion dystonia. There wasspread of the causalgia and of the dystonia from its initial site both in the affected limband to other extremities, the latter in hemiplegic, transverse and triplegic distribution.All investigations were normal. All modes of conventional treatment failed to relieveeither the pain or the dystonia, but two patients recovered spontaneously. At present itis impossible to decide whether this distressing syndrome is a true functional disorderof the central nervous system, or is of psychogenic origin.

INTRODUCTION

Causalgia, that occurs in reflex sympathetic dystrophy (sometimes termed Sudek's atrophyor sympathetic algodystrophy), is a terrible pain which is often untreatable (see Schott,1986a). Causalgia has proved difficult to define. Literally it means a burning pain, buthistorically it has been associated with peripheral nerve lesions. Thus, the InternationalAssociation for the Study of Pain (IASP) has defined causalgia as 'a syndrome of sustained,burning pain after a traumatic nerve injury, combined with vasomotor and sudomotorand later trophic changes' (Merskey, 1986). However, causalgia may occur without overtnerve injury, so we have employed the term in the sense used by Schott (1986a) (seebelow). The pathophysiology of causalgia is uncertain. Implicit in the term reflexsympathetic dystrophy is the notion that overactivity of the sympathetic nervous systemis involved (Bonica, 1979). This is based upon the observation that sympathetic blocksof the affected limb may give temporary relief. But surgical sympathectomy rarely providesa cure (Ochoa, 1991). Indeed, recently it has been suggested that a substantial part ofthe benefit of sympathetic block, by whatever means, is due to a placebo effect (Verdugoand Ochoa, 1991). No abnormality of sympathetic neuronal discharge has been found inaffected limbs on microneuronography (Wallin et al., 1976; Torebjork and Hallin, 1979).

Correspondence to: Professor C. D. Marsden, Institute of Neurology, Queen Square, London WC1N 3BG, UK.

© Oxford University Press 1993

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844 K. P. BHATIA AND OTHERS

Levels of noradrenaline and its intracellular metabolite 3,4-dihydroxyphenylethyleneglycol(DHPG) in venous blood from affected limbs are reduced rather than increased (Drum-mond, 1991).

Some patients who develop causalgia also develop muscle spasms causing dystonicdeformities, jerks and tremors (Marsden et al., 1984; Jankovic and Van der Linden, 1988;Koller et al., 1989; Schwartzman and Kerrigan, 1990). How frequently this occurs iscontroversial. Schwartzman and Kerrigan (1990) described 43 patients out of 200 withcausalgia who exhibited movement disorders; all were said to have some degree of dystonia,although this was 'subtle' in 10 cases. We do not use the term dystonia to describe aphysiological posture of immobile disuse which is often adopted by causalgic patients.

There are, however, patients with causalgia who develop severe distorted dystonicpostures of the affected limb(s). These postures are maintained by abnormal musclecontractions (Fig. 1). The origin of this bizarre syndrome is obscure and its treatmentis very difficult.

FIG. 1. Examples of dystonia associated with causalgia. Top left Case 16. Middle left: Case 14. Bottom left: Case 2,note the fingers of the left hand are held out of the palm by splinting. Top right and bottom right. Case 4.

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CAUSALGIA-DYSTONIA SYNDROME 845

We describe 18 patients with causalgia and dystonia, diagnosed according to thedefinitions below.

Causalgia"The term causalgia will be used here for convenience to embrace the entire range ofconditions accompanied by persistent burning pain; the conditions are often, but notnecessarily, accompanied by vasomotor, sudomotor and trophic changes and responseto sympathetic blockade. The pain is often accompanied by hyperpathia (delay, over-reaction and after-sensation to a stimulus, especially a repetitive stimulus) (IASP, 1979)and allodynia (pain due to a non noxious stimulation)' (Schott, 1986a).

DystoniaThe definition used in that proposed by a subcommittee of the Dystonia Medical ResearchFoundation. 'Dystonia is a syndrome dominated by sustained muscle contractions frequentlycausing twisting and repetitive movements or abnormal postures' (Fahn, 1988). We donot refer to the limb held in a physiological manner due to disuse as dystonia.

PATIENTS AND INVESTIGATIONS

All patients were seen at the National Hospital and examined by one of the authors (C.D.M.)- None hada family history of dystonia. There was no significant history of perinatal injury or developmental delay,or any past history of neurological or psychiatric illness. Apart from the signs of causalgia and dystonia,there were no other abnormal findings on neurological and general physical examination. In particular, nonehad evidence of peripheral nerve or spinal nerve root disease. All patients had normal cognitive and highermental functions. Formal psychometry was carried out in nine cases and was normal. None of the patientsadmitted to psychological distress precipitating their illness, or had overt clinical evidence of psychiatricillness; professional psychiatric evaluation revealed no major or minor psychiatric disorder. Clinical detailsof these 18 patients are shown in Tables 1 and 2.

Secondary causes of dystonia were excluded in all patients by the following appropriate tests: (i) bloodtests: routine blood count, smears for acanthocytes, white cell enzymes including hexosaminidase, B-galactosidase and arylsulphatase A, routine biochemical profile, serum ceruloplasmin (together with slit lampexamination of the eyes), serum protein electrophoresis, Venereal Disease Research Laboratory serology,autoanb'bodies, and alpha-fetoprotein; (ii) tests on urine: amino acids, oligosaccharides, mucoporysaccharides,organic acids, and 24-h copper excretion; (iii) tests on CSF: protein, sugar, cell count, Venereal DiseaseResearch Laboratory tests and oligoclonal bands; (iv) tests for nervous activity: electroencephalogram, evokedpotentials, electromyography and nerve conduction studies, and central motor conduction time; (v) scans:CT and/or MRI scan of the brain, MRI of the spinal cord and/or contrast myelography.

RESULTS

There were 16 women and two men in this study. The mean age at presentation withcausalgia was 28.5 years (range 12—56 years). Fifteen patients had an injury whichprecipitated the onset of their causalgia, while three had a spontaneous onset. All initiallydeveloped burning causalgic pain, and exhibited hyperpathia and allodynia in the affectedregion. All developed sudomotor, vasomotor and trophic changes in various combinations.All patients developed painful dystonia following the onset of their causalgia.

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846 K. P. BHATIA AND OTHERS

TABLE 1. CLINICAL DATA ON 18 PATIENTS WITH CAUSALGIA AND DYSTONIA

Case no.

12345

6

78

9101112

1314

15161718

Age of onset(yean)/sex

20/F28/F13/F29/F12/F

47/F

15/F20/M

56/M15/F48/F30/F

16/F37/F

37/F26/F45/F20/F

Follow-up

6 years3 years3 years9 years4 years

4 years

1 year4 years

10 years3 years

14 years1 year

4 years6 years

2 years8 years3 years1 year

History oftrauma

+++++

+

++

++++

++

+SpontaneousSpontaneousSpontaneous

Site of trauma

Left kneeRight palmLow back, right thighRight kneeLeft ankle

Left wrist

Left ankleLeft ankle

Left thighRight kneeLeft handLeft hip

Right kneeRight hand

Left ankle---

Nature of injury

Playing basketballFall from a horseFall during gymnasticsArthroscopySprain after dancing ata discoSprain while lifting apatientHit by a hockey ballSprain during a fell froman escalatorFracture femur in a fallHockey stick injuryFracture due to fallRemoval of trochantericbursaHockey stick injuryFall on the arm andminor elbow injuryMinor injury

-——

TABLE 2. NATURAL HISTORY OF 18 CASES WITH CAUSALGIA AND DYSTONIA

aseTO.

123

4

56789

10111213

1415161718

Site of initialcausalgia and

dystonia

Left kneeRight handRight thigh

Right knee

Left footLeft wristLeft ankleRight anileLeft thighRight kneeLeft handLeft hipRight ankle

Right handLeft anticLeft armRight hipLeft wrist

Interval betweentrauma andcausalgia

ImmediateImmediateImmediate

Immediate

1 monthImmediateImmediateMonths1 month4 monthsImmediate2 yearsImmediate

ImmediateImmediate

---

Spread ofcausalgia

Left leg and armLeft armRight and left legs

Right leg

NilNilLeft legNilLeft legRight legNilLeft legRight and left legs

Right armLeft legLeft legRight leg and armLeft leg

Interval betweencausalgia and

onset of dystoniaat initial site

Immediate6 monthsTmnvHintf

Immediate

4 months4 months8 monthsImmediate11 months1 month1.5 yearsImmediateImmediate

ImmediateImmediate1 monthImmediateImm^iqtf.

Spread ofdystonia

Left armLeft armRight leg, left foot,pelvisRight and left legs,left arm, retrocollisNilNilNilNflNilNilNflLeft legRight and left legs,left armRight legNilLeft legRight armLeft leg

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CAUSALGIA-DYSTONIA SYNDROME 847

CausalgiaCausalgia began in the lower limb in 12 of the patients and in the upper limb in the othersix. The time interval from the precipitating traumatic event to the onset of the causalgicsyndrome in 15 patients ranged from immediate (within days in nine patients) to as longas 2 years. Causalgia subsequently spread from its original site to other parts of the affectedlimb or elsewhere in 14 of the 18 cases: in the affected limb only in seven cases (sixleg, one arm), to the ipsilateral unaffected limb in hemiplegic distribution in four cases(from arm to leg in two, and from leg to arm in two) and to the opposite limb transverselyin three cases (both arms in one, both legs in two). Spread to previously unaffected limbsoccurred in four of 15 traumatic cases, and in all three spontaneous cases. Two patientshad a further precipitating injury in the subsequently affected limb. In 10 patients, spreadof causalgia was contiguous to an adjacent body part, but did not follow nerve, root ordermatomal distribution. In the remaining four patients the causalgia jumped from onepart of the body to another. Spread of causalgia took place over a few months to as longas 3 years.

DystoniaAll 18 patients developed painful 'fixed' focal dystonia at the original site of causalgia.Characteristically, the patient had great difficulty moving the affected part voluntarily.Of the 15 patients with an injury, the interval between receiving the injury and theappearance of the dystonia was within days in six cases, within 12 months in seven cases,and within 1—2 years in two cases. Dystonia appeared within days after the causalgiain 10 cases, but appeared later, by 1 month up to 18 months after the causalgia in eightcases. In the latter patients, the dystonia often appeared as the severity of the symptomsof causalgia had begun to diminish. The burning pain of the causalgia was often replacedby a different deeper pain associated with the emerging dystonia. However, hyperpathiaand allodynia persisted. Touching the affected limb continued to provoke unpleasantsensations, but the sensitive region often tended to diminish with the passage of time.In eight patients dystonia remained focal only at the initial site of injury, but in 10 patientsthere was subsequent spread of the dystonia. Dystonia spread in a hemiplegic distributionin five cases (arm to leg in three, leg to arm in two), in a transverse distribution in twocases (both arms in one, both legs in one) and in a triplegic distribution in two cases(both legs and an arm). Spread of dystonia within a limb was unusual, occurring in onlyone case; in the rest the dystonia remained confined to its original site, but jumped toother limbs. Out of the 10 cases in whom there was spread of dystonia, this mimickedthe spread of causalgia in seven, while in three cases the dystonia was more widespreadthan the causalgia. In only two cases was the new dystonia precipitated by a new injury.Spread of dystonia took place during several months up to 3 years. In all patients dystoniawas 'fixed' and painful, with minimal mobility at the affected joints.

TreatmentMany different types of treatment were tried in these cases. It should be emphasized thatwe saw these patients after the acute phases of causalgia had occurred. Eleven patientshad splints or plaster casts applied to the affected limb. However, in all cases the dystoniareturned when the cast or splint was removed, and in some patients the spasms wereso strong that they fractured the plaster cast! Two patients had arthrodesis of leg joints,

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848 K. P. BHATIA AND OTHERS

but never walked. Likewise tendon transfer in two cases were of no help. To begin with,we tried sympathetic blocks, sympathectomies and guanethidine infusions in eight patients.Sometimes there was temporary benefit, but no long-term relief of pain or dystonia wasachieved. Lisewise, epidural anaesthesia in three cases was of no long-term benefit. Manydrugs were given, including benzhexol (trihexyphenidyl), baclofen, levodopa, carba-mazepine, diazepam, clonazepam and amitryptiline but without sustained useful benefit.Transcutaneous electrical nerve stimulation also was used, but with limited success. In short,none of those treatments gave relief. However, two patients (Cases 3 and 4) recovered,apparently spontaneously, after 4 and 9 years of illness, respectively.

DISCUSSION

There is accumulating evidence to implicate the role of peripheral trauma in triggeringidiopathic torsion dystonia (Sheehy and Marsden, 1980; Schott, 1985, 1986ft; Koller et al.,1989). In a recent study, Fletcher et al. (1991) suggested that trauma may precipitateidiopathic torsion dystonia in some patients who carry the abnormal dystonia gene.Causalgia and dystonia precipitated by peripheral trauma is an established clinical syndrome(Marsden et al., 1984; Jankovic and Van der Linden, 1988; Schwartzman and Kerrigan,1990), but the causalgia-dystonia syndrome does not appear to be inherited. None ofour patients gave a family history of dystonia, nor did those described by Schwartzmanand Kerrigan (1990) and by Jankovic and Van der Linden (1988). Despite both beingtriggered by trauma, the two dystonic syndromes appear to be different (Table 3),

TABLE 3. DIFFERENCE BETWEEN THE SYNDROMES OF CAUSALGIAAND DYSTONIA AND PRIMARY TORSION DYSTONIA

Causalgia and dystonia Inherited dystonia

Clear preponderance of women No preponderance of women

No family history Positive family history

Painful (causalgic syndrome) Usually painless

Vasomotor, sudomotor and trophic changes Such changes not seen

Fixed spasm Mobile spasms

Contractures common and early Contractures uncommon

suggesting a different pathogenesis. Although trauma often triggers the causalgia—dystoniasyndrome, it is not essential. Causalgia and dystonia appeared spontaneously in threepatients in our study. Causalgia alone may have a spontaneous onset (Schott, 1986a).The trauma precipitating causalgia and dystonia was often relatively trivial, and did notcause obvious peripheral nerve damage.

Because pain and dystonia coexisted in our patients, and often had a similar distribution,they may have had a common origin. There are many causes of causalgia, and a numberof mechanisms may be involved in its genesis. However, there is evidence in some cases

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CAUSALGIA-DYSTONIA SYNDROME 849

that it is due to changes in the central nervous system, even if the provoking trigger wasa peripheral injury. The allodynia and hyperpathia of causalgia cannot be explained solelyon the basis of ephaptic or ectopic transmission at the site of nerve injury (Nystrom andHagbarth, 1981). Also the pain of causalgia may not be abolished by deafferentation andmay persist even in an anaesthetized limb (Appenzeller and Bicknell, 1969; Sunderland,1978; Noordenbos and Wall, 1981). This implies that an intact peripheral sensory nervesupply is not essential for some types of persistent causalgic pain. Successful nervetransplant in a damaged nerve (Noordenbos and Wall, 1981) also may not improve causalgicpain. Persistent pain of similar character in a phantom limb is another reason againsta peripheral mechanism in some cases of causalgia (Melzack, 1974; Melzack andLoeser, 1978).

The causalgia and dystonia in our patients often spread beyond their original site;hemiplegic, transverse and crossed spread was common (see also Schwartzman andKerrigan, 1990). Such spread cannot be explained on the basis of a peripheral origin,and is difficult to explain by invoking changes in the spinal cord. Hemiplegic distributioncould be explained only by involvement of propriospinal pathways, but these control theaxial muscles, which are rarely involved in the causalgia—dystonia syndrome. The footand hand are preferentially affected in the causalgia-dystonia syndrome, suggesting thatthe origin must be more central. It would also be difficult to presume the origin of causalgiaand dystonia to be in the brainstem, as brainstem motor pathways also control the axialmuscles (Lawrence and Kuypers, 1968). Therefore the site of origin of the causalgiaand dystonia seems more likely to reside higher in the brain.

The next question that must be asked is whether the causalgia—dystonia syndrome isa true functional neurological disease (with no known visible pathology) or the consequencesof a psychological disorder, as suggested by Lang and Fahn (1990) and Ecker (1990).At first sight it is difficult to conceive of such a distressing and disabling condition beingan hysterical conversion reaction. Indeed, none of our patients exhibited prior conversionsymptoms or overt psychological precipitants (see also Schwartzman, 1990). However,one cannot ignore the overwhelming preponderance of young women in this series, whichalso applies to causalgia itself (Schwartzman and Kerrigan, 1990). Sixteen of our 18 patientswith the causalgia—dystonia syndrome were female; likewise 36 of the 43 cases describedby Schwartzman and Kerrigan (1990) were female, as were five of the nine cases describedby Jankovic and Van der Linden (1988). The absence of overt psychopathology is<x>mmonin isolated conversion reactions. Proof of a psychogenic origin for this syndrome requirescure or resolution of symptoms and signs with appropriate therapy. Unfortunately, thecondition remitted in only two of our 18 patients; the remainder continued with symptomsand signs unchanged or worsening over periods of 1 — 14 years. Most of the patientsdescribed by Schwartzman and Kerrigan (1990) had had their condition for many years.Others, however, have recorded recovery from the causalgia—dystonia syndrome afterintensive psychological and physical therapy (S. Fahn and J. L. Ochoa, personal com-munications). Lang and Fahn (1990) also discuss the possibility that the vasomotor,sudomotor and trophic changes characteristic of the causalgic syndrome may be secondaryto psychogenic dystonia (for a review of psychogenic dystonia, see Fahn and Williams,1988). However, none of the 21 cases described therein had causalgia. In our series ofpatients the symptoms of causalgia coincided with or preceded the dystonia, so was unlikelyto be secondary to the muscle spasms. Nevertheless, the view that at least some of these

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850 K. P. BHATIA AND OTHERS

cases are psychogenic in origin cannot be discounted; that others have a functionalneurological disorder remains a possibility.

What advice can be given on management? Most of our patients had been subjectedto a variety of procedures (sympathetic blocks, surgical sympathectomy, peripheral somaticnerve blocks, epidural anaesthesia, etc.) without sustained relief. We believe that potentiallydangerous or destructive treatments should be avoided. Because of the suspicion of apsychogenic origin, at least in some cases, we believe therapy should be directed towardsgradual psychological exploration, without confrontation, emphasizing the influence ofstress as a cause of physical disability. In parallel, graded physical rehabilitation shouldbe encouraged, using simple pain relief measures, which might include sympathetic blocks(with a placebo trial) and transcutaneous electrical nerve stimulation. Drug therapy rarelyhelps, but carbamazepine and amitriptyline are worth a trial for pain relief, and benzo-diazepines, anticholinergics and baclofen might help the muscle spasms.

In conclusion, the origins of this distressing and disabling clinical syndrome remainmysterious. However, a few facts can be stated with some confidence. The causalgia—dystonia syndrome is relatively uncommon, and in relation to the sort of injuries thatprecipitate the condition, it is very rare. It causes prolonged pain and handicap, and onceestablished is very resistant to treatment. Women seem more prone to the condition thanmen. Spread of both causalgia and dystonia is common. Further investigation of suchpatients is required to decide the relative causative roles of a psychogenic origin or afunctional neurological illness. Approaches employing techniques such as positron emissiontomography may be revealing, but it is going to be difficult to decide whether abnormalitiesare primary or secondary.

ACKNOWLEDGEMENT

We wish to thank our colleagues for kindly referring some of the patients in this study.

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Received August 18, 1992. Revised January 11, 1993. Accepted February 16, 1993

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