How Psychogenic is Dystonia? Views from Past to Present

Alexander G. Munts; Peter J. Koehler

Disclosures

Brain. 2010;133(5):1552-1564. 

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Primary Generalized Dystonia

In 1871, William Hammond (1828–1900) reported on three patients 'in which the most characteristic symptoms are an inability to retain the fingers and toes in any position in which they may be placed, and their continual motion' (1871). He mentioned the acquired disorder 'athetosis' and hypothesized on a striatal lesion. Hammond's patients may not be considered dystonic patients—although today most authors consider athethosis as part of the dystonia spectrum (Morris et al., 2002)—but it is important to mention Hammond's coining of the term athetosis. In 1897, the Spanish physician Lluis Barraquer I Roviralta (1855–1928) reported another patient with athetosis, which later was considered the first description of generalized torsion dystonia (Barraquer, 1897; Barraquer-Bordas and Gimenez-Roldan, 1988). In 1908, the German Marcus Walter Schwalbe (1883–1927) described hysterical symptoms in the siblings Fanny, Heimann and Wulf Levin, suffering from tonic cramps, which is now recognized as early-onset generalized torsion dystonia (Figure 1) (Schwalbe, 1908; Truong and Fahn, 1988). Among the most important hysterical characteristics, there was the presence of pressure points [called 'hysterogenic zones' in Charcot's work (Micale, 1995)], i.e. body areas in which cramps may be provoked by pressure.

Figure 1.

Developments on generalized dystonia in the 20th century. See the text for references.

Familial involvement was another feature. In 1911, Oppenheim launched the term dystonia musculorum deformans for the same disorder (Oppenheim, 1911). He reported on four patients, who were Jewish children. Illustrative is the description of a 14-year-old girl with a 'dromedary gait'; 'indem der sättelformige ausgebuchtete Rücken in eine fast horizontale Lage kommt, und zwar fällt die Rumpfbeugung zusammen mit dem Aufsetzen des linken Beins, während der Rumpf sich hebt beim Schwingen des linken Beins' [because the saddle-shaped back acquires an almost horizontal position, in which the left leg posture phase is accompanied by trunk flexion, and the swing phase by trunk elevation]. He was convinced that it was an organic disease without concomitant hysteria.

Subsequently, dystonia musculorum deformans became a collective term for a variety of neurologic disorders (Zeman, 1976). There was a continuing discussion on the characteristics of the disorder, and a pathological substrate was still unknown. For these reasons, the concept of dystonia as a disease was demolished during the tenth Réunion Neurologique Internationale Annuelle in Paris (1929). Subsequently, the Danish physician Auguste Wimmer (1872–1937) concluded that dystonia was no more than a syndrome (Wimmer, 1929). In the meantime, a psychogenic explanation had emerged for various nervous disorders without anatomic lesions. One of the founders of psychogenesis was Sigmund Freud (1856–1939). From 1888 to 1910, he described several patients who suffered from hysteria and in whom symptoms were related to conflicts and psychological defence (Tomlinson, 2006). The effectiveness of psychological intervention supported this new and revolutionary theory. Exploring and resetting the unconscious mind, by means of 'psychoanalysis', became a successful therapy in many cases of hysteria. Since then, many patients with generalized dystonia underwent this or other forms of psychotherapy.

In 1944, Ernst Herz (1900–1965) published three frequently cited articles on his studies of dystonia cases. He considered dystonia a 'clinical entity' with 'characteristic irregular, involuntary motor phenomena', 'a peculiar distribution of "excess of motion" and "excess of tension" ' and 'without recognizable aetiologic factors at onset' (Herz, 1944a,b,c). In 1959 the hereditary nature of dystonia musculorum deformans was demonstrated (Zeman et al., 1959) and 10 years later a report was published on the limited efficacy of psychotherapy in 44 patients with torsion dystonia (Eldridge et al., 1969). In the same year, Irving Cooper (1922–1985) reported on a 77% success rate after unilateral or bilateral surgery of the thalamus or globus pallidus in 144 patients with dystonia musculorum deformans (Cooper, 1969). In the 1960s, Denny-Brown reported his landmark studies on dystonia. He caused selective lesions in monkey brains that led to uncontrollable abnormal postures and movements resembling dystonia. It was remarkable to observe that damage in different anatomical structures might have the same consequences. Denny-Brown assumed that dystonia resulted from an imbalance of reflex responses in the central nervous system (Denny-Brown, 1965, 1966; Gilman et al., 1999). In 1975, an International Symposium on Dystonia was organized. In the preface of the conference book, Eldridge and Fahn wrote:

In the past, many victims of dystonia and their families have been caused anguish and hardship over and above that caused by the disease itself owing to the frequent misdiagnosis of the symptoms as manifestations of a psychiatric ailment. We hope that the present volume will facilitate accurate diagnosis, assist practicing physicians in treating their dystonic patients, encourage them to report their observations and results, and stimulate clinical and basic research workers in efforts to elucidate the causes and eventual treatment of dystonia and related disorders. (Eldridge and Fahn, 1976).

At this symposium, Marsden emphasized the existence of sporadic torsion dystonia (Marsden et al., 1976). Fahn and Eldridge stated that psychologically based dystonia was a rare or non-existent condition (Fahn and Eldridge, 1976). However, 3 years after the symposium (1978), the 'first case of psychogenic dystonia' was reported (Lesser and Fahn, 1978) and in 1983, at the 35th annual meeting of the American Academy of Neurology, another five followed (Fahn et al., 1983). The first patient was a 15-year-old girl who had simulated her dystonic symptoms and signs. She was admitted after a failed suicide attempt and told that she had faked her symptoms: 'she discarded her leg brace, and the sustained contractions in her leg and arm immediately improved'. The histories of the other five patients were not included in the publication.

In 1984, an ad hoc committee, consisting of members of the Scientific Advisory Board of the Dystonia Medical Research Foundation, re-defined dystonia as 'a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures' (Fahn, 1988). Four years later a classification for psychogenic dystonia followed (Table 1) (Fahn and Williams, 1988). The first locus (9q32–34 region) for idiopathic dystonia (DYT1) was found in 1989 (Ozelius et al., 1989) and 10 years later the same group identified the gene, describing a unique 3 base pair deletion in the coding region, which was responsible for almost all their cases with early-onset, but for only a few with late-onset idiopathic torsion dystonia (Ozelius et al., 1997).

Cervical Dystonia

One of the earliest descriptions of cervical dystonia was given by the Swiss physician Felix Platerus, also known as Plater (1536–1614) (Steyerthal, 1906; Platter, 1963). He described a case of 'spasmi species, in qua caput in sinistrum latus torquebatur' [a kind of spasm in which the head was turned to the left side]. The Dutch Nicolaas Tulp, or Tulpius (1593–1674), well known from the famous Rembrandt painting The Anatomy Lesson of 1632, described dissection of the sternocleidomastoid muscle as a therapy for what he called 'obstipi capitis' [crooked head] in his Observationes medicae (Tulp, 1641; Tulp and von Wolzogen, 1740). However, this patient had had torticollis from childhood and the origin was probably mechanical.

The well-known Scottish surgeon-anatomist Charles Bell (1774–1842) stated that the origin of 'spasmodic contortion of the head and neck' was nerve rather than muscle dysfunction (Bell, 1844). One of his patients, Mary Preston, developed the disease following a hard and protracted labour. In Bell's view, a disorder of the accessory nerve but not other nerves, due to strain, might lead to unbalanced muscle drive and thus to the disease. Interestingly, the same case was also reported elsewhere (Anonymous, 1825), but according to these authors, the disease was not limited to the distribution of the accessory nerve. Their disagreement with Bell was underlined by their commentary 'We have frequently had occasion to notice the very ingenious manner in which Mr Bell perverts facts, in order to meet his own particular views of a case'. The French physician Guillaume-Benjamin Duchenne (1806–1875), who applied electricity for a variety of disorders in Parian hospitals, reported that 'spasme fonctionnel du sterno-mastoïdien' is quite resistant to treatment (Duchenne, 1861). Instead, he advised therapy by applying continuous stretch of the antagonists with the use of an instrument.

During one of his well-known Tuesday Lessons, on 26 June 1888, Charcot presented a 63-year-old man with 'spasme clonique du sterno-mastoïdien et du trapèze' [clonic spasm of the sternocleidomastoid and the trapezius muscles], which had been present for 8 months (Charcot, 1887). The disorder started after the patient, who was a stockbroker, had lost all his money. On 10 July 1888, he was presented again after being successfully treated with electricity. Five years later, one of Charcot's former students, Edouard Brissaud (1852–1909), introduced the term 'torticolis mental' (Brissaud, 1895). In his view, the condition was psychogenic, which was evident from the fact that the patient was able to correct the powerful muscle activity by simply touching the head, later named the 'geste antagoniste efficace' (Meige and Feindel, 1902). Emphasizing the psychogenic nature again, the Hungarian Jenö Kollarits (1870–1940) reported on six 'torticollis hystericus' cases in 1905 (Kollarits, 1905). Therapeutic dissection of the involved muscles or nerves, as performed by the Swiss surgeon Fritz de Quervain (1868–1940) (de Quervain, 1896), was considered malpractice according to Kollarits who, instead, stated that therapy should be based on suggestion.

In this period, there was much discussion on torticollis in the scientific community. At the New York Neurological Society (1914), Pierce Clark (1870–1933) presented an adult man with 'mental torticollis' which, he said, was the consequence of pleasurable stroking movements by his mother, before the age of six (Clark, 1914). In reaction, Bernard Sachs (1858–1944) said, 'if this indicated the future trend for our present-day neurology, then the less we hear of it, the better'. The debate went on for several decades. In 1935 Joseph Yaskin (1891–1955) wrote that before surgery, every case of spasmodic torticollis should receive a trial of psychotherapy (Yaskin, 1935). In 1943, Patterson and Little reported on 103 cases with spasmodic torticollis (Patterson and Little, 1943), stating that the aetiology was usually organic and that surgery, intradural rhizotomy in particular, was very satisfactory. However, in 1945 the Scottish physician Paterson presented 21 cases, concluding that psychotherapy was the treatment of choice unless gross signs of neurological disease were present (Paterson, 1945).

At the 1975 International Symposium on Dystonia (vide supra), Marsden presented arguments that spasmodic torticollis, blepharospasm, oromandibular dystonia and dystonic writer's cramp (vide infra) were focal dystonias with an organic aetiology. He summarized the reasons why they had been regarded psychogenic (Table 3) (Marsden, 1976), and subsequently explained his ideas about functional abnormalities in the extrapyramidal motor system (Marsden, 1976). Obvious arguments were their occurrence in early-onset generalized torsion dystonia and the similarities with late-onset generalized torsion dystonia: both focal dystonia and late-onset generalized torsion dystonia had a comparable age of onset and were usually neither progressive nor hereditary. A new name was introduced in the 1980s: 'cervical dystonia'. In 1985 the Canadian Tsui reported for the first time the successful use of botulinum toxin injections in 12 patients (Tsui et al., 1985), which eventually became the standard treatment. During the past few decades hardly any reports on psychogenic cervical dystonia cases have been published.

Writer's Cramp

In 1713 the Italian physician Bernardino Ramazzini (1633–1714) recognized intense fatigue of the hand and arm, which resulted in failure of power, as an occupational disorder in professional writers (Ramazzini, 1964). In 1844 (published posthumously), Bell most probably described writer's cramp when he reported on an ambiguous condition in which writing had become impossible while the arm strength remained normal (Bell, 1844). In 1861 Duchenne reported on 'crampe des écrivains' in which electricity was not a very successful therapy. However, he advised an ingenious prosthesis (Duchenne, 1861). He preferred the names 'spasme fonctionnel' and 'paralysie musculaire fonctionelle' because the disorder was not restricted to cramps and could be provoked not only by writing but also by other manual actions.

En résumé, les faits et les considérations exposés précédemment démontrent, comme je l'ai dit au commencement de cette note, qu'il existe une maladie caractérisée par un spasme douloureux ou indolent (contracture, contractions clonique, tremblements), ou par une paralysie musculaire; que ces troubles se manifestent seulement pendant l'exercice de certains mouvements volontaires ou instinctifs; enfin, qu'ils peuvent siéger dans des régions fort diverses. (Duchenne, 1861)
[In summary, the former findings and considerations show, as I described in the beginning of this report, that there is a disease which is characterised by painful or painless spasms (contracture, jerks, tremor) or paralysis in which the signs only occur during certain (in-)voluntary actions; the involved body parts are diverse.]

Similar to the situation in cervical dystonia, the debate on aetiology started in the early 20th century. In 1914 the German Fritz Mohr (1874–1957) summarized the two conflicting theories in Lewandowsky's Handbuch der Neurologie (Lewandowsky, 1914). Writer's cramp was explained by some authors as a purely organic disorder, e.g. as a reflex cramp through motor nerves that was initiated by painful sensory input. The German physician Moritz Romberg (1795–1873) was mentioned as one of the early advocates [with reference to the 1853 edition of Rombergs Lehrbuch der Nervenkrankheiten des Menschen (Romberg, 1853)]. Others, including Mohr himself, believed that only people with certain personality characteristics were prone to develop the disorder, a psychological factor possibly being involved. From that view, accurate psychoanalysis would be the best therapy for patients with writer's cramp. Kinnier Wilson's (1878–1937) 1940 edition of Neurology described 'writers' cramp' as an occupational neurosis, physiologically akin to hysteria, and assumed a cortical dysfunction (Kinnier Wilson, 1940). Prevention by excluding people prone to develop 'spasms' from certain occupations was considered the best treatment.

As in other focal dystonias, Marsden advocated the organic nature of writer's cramp (vide supra) (Marsden, 1976), which he and Sheehy further demonstrated in a report on 29 patients (Sheehy and Marsden, 1982). However, in 1983, Cottraux et al. reported on the success of behavioural therapy and biofeedback in 9 of 15 patients with writer's cramp (Cottraux et al., 1983), and the 1985 edition of John Walton's Brain's Diseases of the Nervous System stated:

I find the conclusions of Sheehy and Marsden inherently implausible and unacceptable. In my experience even subtle physical signs are absent in the many simple cases that I have seen and neither focal dystonia nor any other organic disorder could in my view impair movements only when they take part in one co-ordinated act while leaving totally unaffected all other precise and complex voluntary actions involving the affected member. (Walton, 1985).

The 1993 edition stated that writer's cramp had 'in the past been attributed to psychological factors, but there is now good evidence that this is not so'. Interestingly, the author referred to the same single publication of Sheehy and Marsden (Sheehy and Marsden, 1982; Harding, 1993). In 1991 Rivest et al., reported for the first time on the use of botulinum toxin for writer's cramp (Rivest et al., 1991), which is currently considered the most effective treatment.

Fixed Dystonia Related to Complex Regional Pain Syndrome

In 1864, Silas Weir Mitchell (1829–1914) described a series of American Civil War (1861–1865) victims with gunshot wounds who developed burning pain and a shiny red skin after nerve injury (Mitchel et al., 1864; Koehler and Lanska, 2004). He suspected that traumatic nerve irritation was the cause and named the condition 'causalgia'. He recognized that patients might come into an unendurably painful hyperaesthetic state. In 1892, Charcot demonstrated another entity in two patients: 'oedème bleu des hystériques', a painful condition with oedema and blue discoloration of the skin, which may occur in combination with an hysterical limb contracture or paralysis (Charcot, 1892).

L'historique de cette affection n'est pas bien long. Je l'ai pour la première fois mentionnée et distinguée à propos d'un malade de cet hospice [with reference to the Leçons du Mardi from 1889], que je suis d'ailleurs à même de vous présenter de nouveau. Puis, à plusieurs reprises je l'ai observée chez des personnes de la ville, combinée tantôt avec des altérations de la sensibilité (anesthésie ou hyperesthésie), tantôt avec des troubles du mouvement (paralysies et contractures). Il s'agissait presque toujours de sujets marqués, par la présence des stigmates, au sceau de l'hystérie la mieux caractérisée. (Charcot, 1892) [This disorder has a short history. For the first time [in 1889], I reported on a patient from this hospital [Hospice de la Salpêtrière]. From then, I recognised more cases. In a number of them, I observed sensory abnormalities (anaesthesia or hyperesthesia) or movement disturbances (paralysis and contractures). Mostly, patients were extraordinary persons having characteristics which may be considered hysterical.]

In 1946, Evans renamed the latter disorder 'reflex sympathetic dystrophy' because he suspected involvement of spinal reflexes as well as sympathetic efferent fibres (Evans, 1946). It was different from causalgia in that it occurred in the absence of major nerve trauma. However, in 1994, the International Association for the Study of Pain introduced the name complex regional pain syndrome (CRPS) for both conditions: type 1 (reflex sympathetic dystrophy) and type 2 (causalgia) (Merskey and Bogduk, 1994). The diagnostic criteria for CRPS type 1 were: (i) the presence of an initiating noxious event, or a cause of immobilization (not obligatory item); (ii) continuing pain, allodynia or hyperalgesia with which the pain is disproportionate to any inciting event; (iii) evidence at some time of oedema, changes in skin blood flow or abnormal sudomotor activity in the region of the pain; and (iv) no other condition that would account for the degree of pain and dysfunction. CRPS type 2 has the same characteristics, but is accompanied by nerve injury (Merskey and Bogduk, 1994).

In 1984, Marsden et al. reported on four 'reflex sympathetic dystrophy' patients who had dystonia, characterized by fixed, predominantly flexion, postures (Marsden et al., 1984). They believed it to be 'a distinct clinical syndrome'. Six years later Schwartzman et al., reported on motor disturbances in 43 patients with 'reflex sympathetic dystrophy', in whom the most dramatic characteristic was a dystonic posture in all patients (Schwartzman and Kerrigan, 1990). The authors hypothesized on a spinal cause. In 1993, a series of 18 patients with similar characteristics was reported (Bhatia et al., 1993). However, it was remarkable that many patients met the criteria for psychogenic dystonia from 1988 (vide supra) (Table 1). They concluded that the aetiology of this disorder, psychogenic or organic, was unknown (Bhatia et al., 1993). In 2004 it was reported that many patients with features of CRPS and dystonia also had features of psychogenic dystonia (Schrag et al., 2004). In the same year it was stated that a very large proportion had a primary psychogenic disorder (Sa et al., 2004). In a more recent paper on 110 CRPS type 1 patients with dystonia predominantly characterized by tonic flexion postures, the authors hypothesized that maladaptive plasticity with disinhibition of spinal mechanisms might be the cause (van Rijn et al., 2007).

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