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J Neurol Neurosurg : first published as 10.1136/jnnp.52.Suppl.90 on 1 June 1989. Downloaded from

Journal ofNeurology, Neurosurgery, and Psychiatry. Special Supplement 1989:90-95

Psychopathology and movement disorders: a new perspective on the Gilles de la Tourette

MICHAEL TRIMBLE From the Department ofClinical , Institute ofNeurology, the National Hospitalfor Nervous , London, UK

SUMMARY Attention is drawn to associations between movement disorders and . The historical background of this is briefly reviewed, and then some recent research findings with regards to the Gilles de la Tourette syndrome are presented. In particular, associations between aggressive and obsessive compulsive disorder and Gilles de la Tourette movements are reported. Further there are associations between a family history of and obsessive compulsive disorder in the subsequent generation. It is concluded that the Gilles de la Tourette syndrome should not be viewed as a disorder, but a far more complex condition in which the tics form but one aspect.

The relationship between movement disorders and syndrome which highlights specific aspects of the psychopathology is of growing interest from several psychopathology of the syndrome. This has not onlyProtected by copyright. points of view. First, abnormal movements are noted led to new directions for research, but also challenges in a number of patients following drug for some of the currently held conceptions about the psychotic illnesses. Many distinct have condition. been described, but often their presentations can remain unrecognised, misdiagnosed as , or Gilles de la Tourette syndrome mistaken for worsening of the underlying psychiatric illness. Secondly, there are a number of primary Gilles de la Tourette described the syndrome that movement disorders in which psychopathology occurs bears his name in 1885.4 Following a flurry of case during the course of the illness, or on occasions prior reports which followed shortly thereafter, the condi- to the onset of the abnormal movements. Hunting- tion virtually disappeared from the literature until the ton's would be an example. Finally, recent 1960s when a resurgence ofinterest occurred following developments in and neurochemistry the reported success of in its treatment. have highlighted important relationships between Since then the Gilles de la Tourette syndrome has been neuronal systems which modulate both movement and subject to several reviews5 6 and, far from being the rare emotion, suggesting biological underpinnings for disorder it was once thought, is now being reported in clinical observations which have been made over the increasing numbers, especially the less severe variants. http://jnnp.bmj.com/ years. The most recent diagnostic criteria, as given in DSM There have been several reviews of this topic'2 III R are shown in table 1. It is clear that the essential including a recent assay from Professor Marsden3 feature of the disorder is the tic, and indeed the in which he concluded "movement is one ofthe robust condition is invariably classified as a .7 It is bridges between neurology and psychiatry, built upon here suggested, however, that to refer to the syndrome solid foundations that tantalise those in both dis- of Gilles de la Tourette simply as a tic disorder is to ciplines". ignore many of the richer manifestations of the It is the purpose ofthis paper to review briefly some condition, and to be unfamiliar with its pleomorphic on September 27, 2021 by guest. research which has been carried out at the Institute of clinical presentations. Neurology, Queen Square, on the Gilles de la Tourette There can be no better starting point for discussing the psychopathology of tics than to read Meige and Feindel's Tics and their Treatment published in 1907.8 Address for reprint requests: Dr M Trimble, Institute of Neurology, National Hospital for Nervous Diseases, Queen Square, London The Confessions of a Victim to Tic describes the WCIN 3BG, UK. symptoms of a 54 year old man who would now be diagnosed as having the Gilles de la Tourette syn- Accepted January 1989 drome. He suffered from a wide variety of tics, 90 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.Suppl.90 on 1 June 1989. Downloaded from

Psychopathology and movement disorders: a new perspective on the Gilles de la Tourette syndrome 91 Table 1 The diagnostic criteriafor Gilles de la Tourette series of follow ups which have been carried out in syndrome (DSM III R)' recent years support any obvious association, and the development of a psychotic state in the condition is A) Both multiple motor and one or more vocal tics have been rare. present at some time during the illness, although not necessarily concurrently. The theme ofaggression was ofparticular interest to B) The tics occur many times a day (usually in bouts), nearly writers of psychoanalytic persuasion suggesting that every day or intermittently throughout a period of more the tic represented some form of internalised, unex- than one year. C) The anatomical location, number, frequency and severity of pressed aggression, or was seen as a defence against the tics change over time. aggressive impulses or as an outlet for unmanageable D) Onset before the age of 21 years. E) Occurrence not exclusively during psychoactive substance aggressive feelings. This theme, and the relationship to intoxication or known central , such obsessive compulsive disorder, have been the subject as Huntington's chorea and post viral encephalitis. of several studies carried out at the Institute of Neurology, the results of which will be briefly sum- marised. and , complicated movements, and "a tic of phonation". The authors drew attention The psychopathology ofGilles de la Tourette to the "fundamental importance of the psychical syndrome: a phenomenological analysis element that precedes the motor reaction", and There has been a referral clinic at the National documented the patient's impulsivity, suicidal tenden- Hospital, Queen Square, for patients with the Gilles de cies and obsessive compulsive behaviour. They noted la Tourette syndrome for a number of years, and his obsessional fears and suggested intimate analogies systematic detailed information has been kept on all between tics and obsessions. They concluded "it has patients attending. The neurological findings from the been possible to determine the origin ofthe tics and to first 53 patients have been fully described'" in which we confirm the association with them of a peculiar mental highlighted the similarity ofour population with other Protected by copyright. state". Meige and Feindel noted that Trousseau, in reported series. We noted the relative low frequency of 1873, had drawn attention to the fact that the mental abnormal neurological signs, only 13% showing an state of patients with tics was abnormal, but gave abnormal electro-encephalogram, and nearly all credit to Charcot for demonstrating the significance of having a normal CT scan. We have further highlighted psychical factors. the interesting finding" that 12 5% of patients show Interestingly, in Gilles de la Tourette's original low levels, which in some cases is related to description, little mention was made of associated abnormalities of copper handing, although these psychopathology with the exception of excessive fear. patients are not identified by any particular In his 1899 paper9 he developed the theme further. neurological features. Acknowledging the hereditary factor, he commented The psychopathology of the first 90 patients who on the anxieties and of his patients. Further, fulfilled the DSM III criteria for Gilles de la Tourette he acknowledged the contributions of Guinon who syndrome was assessed using clinical evaluation had reported that "tiqueurs" nearly always had procedures, and the following rating scales were associated psychiatric problems characterised by mul- completed: the Beck inventory; the Spiel- tiple phobias, arithmomania and . Gilles berger trait anxiety inventory; the borderline syn- de la Tourette made the point that the mental stigmata drome index; the mood adjective check list; the http://jnnp.bmj.com/ appeared late in the natural history of the condition, Middlesex Hospital questionnaire; the Eysenck per- but that it was rare for an adult case to be completely sonality inventory; the hostility and direction of free from them. Further they were more pronounced hostility questionnaire; and the questionnaire of the during the period of exacerbation of the tics. Leyton obsessional inventory. Subsequently, writers have developed three com- In this investigation, no patients were found who mon themes on the relationship between Gilles de la were psychotic, although three exhibited ideas of Tourette syndrome and psychopathology. The first reference. Fifteen were judged clinically to have relates to the development of a paranoid state or depressive symptoms severe enough to constitute a on September 27, 2021 by guest. , the second to aggression, and the third to major affective disorder, and 25 had severe anxiety. obsessive compulsive features. Seventy six patients had disorders of sleep including The potential for patients with the syndrome to night terrors, insomnia, sleep talking, sleep walking, develop psychosis was inherent in the 19th century nightmares and nocturnal . These data with view of degeneration. Gilles de la Tourette concurred regard to general psychopathology, are not dissimilar with this theory and there have been anecdotal reports to those of other series, for example that of Shapiro et of patients who appear to develop psychosis in the al.5 The latter concluded "most Tourette patients had course of their illness. However, none of the extensive some degree of maladjustment. Their problems were J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.Suppl.90 on 1 June 1989. Downloaded from

92 Trimble Table 2 Relationship between certain symptoms of the Gilles de la Tourette syndrome and aggression Hostility and direction ofhostility questionnaire Symptoms or demographic details Aggressive behaviour Criticism ofothers Paranoid hostility Selfcriticism Sum ofhostility Forced to touch * Coprophenomena t() t() *(Coprolalia) *(Coprolalia) Echophenomena t t *< 0.05; t< 0-01.

Table 3 Examples ofself-injurious behaviour in patients syndrome and self damage to the eyes.'2 Thus self with Gilles de la Tourette syndrome injurious behaviour is reported in several populations ofpatients including the mentally retarded, those with Behaviour No ofcases and those with psychosis. Interes- Head banging 14 tingly, the pattern of damage differs between these Body punching 10 groups, wrist cutting, for example, being common in Head or face punching 9 Body to hard object banging 8 personality disorders, while ocular damage occurs Poking sharp objects into body 2 more frequently in psychotic patients. As we have Scratching parts of body 2 Putting hands through window 2 noted, our patients do not present with psychotic Other single types of self-injurious behaviour 12 features but we have seen several patients who have severely damaged their eyes. We have recently report- ed a case whose eyes were so frequently traumatised no more severe, was an outcome. however, than those of a psychiatric that blindness inevitable Neuro- Protected by copyright. out patient group". surgical intervention with an anterior cingulectomy Aggressive and self destructive behaviour The for this behaviour was considered necessary and association between aggressive behaviours and some dramatically improved his behaviour.'3 of the symptoms ofGilles de la Tourette syndrome are We have also carried out a comparative study of shown in table 2. aggressive behaviour in patients with spasmodic tor- Twenty eight patients had been physically aggres- ticollis, writer's cramp and the Gilles de la Tourette sive to other people, animals or property. The aggres- syndrome.'4 Sixteen patients with spasmodic tor- sive behaviour was significantly associated with the ticollis, eight with writer's cramp, and 20 with the symptoms of being forced to touch and copropraxia. Gilles de la Tourette syndrome were compared with 25 Patients scored highly on the hostility questionnaire, normal controls, each clinical group having its own and extra-punitive items, such as acting out hostility age and sex match controls. They were administered and criticism of others, were unrelated to depression the hostility and direction ofhostility questionnaire of scores. The kinds ofselfinjurious behaviours recorded Foulds. The results from this study are shown in are shown in table 3. Thirteen out of fourteen head table 4. bangers had CT scans carried out and two had cavum The extrapunitive scores of acting out hostility and septum pellucida. criticism of others and the sum of hostility scores http://jnnp.bmj.com/ Self injurious behaviour was related to the showed significant differences between the groups, cumulative sum of motor tics ever and tics of the legs, patients with writer's cramp scoring significantly while subjects with head banging reported a greater higher than controls. On criticism of others they were number of cumulative motor tics than those without. significantly higher than patients with spasmodic We have noted a particular association between the torticollis. The latter patients showed no significant

Table 4 Scores on a hostility rating scale in three abnormal movement disorders and controls on September 27, 2021 by guest.

Acting out hostility Criticism ofothers Sum ofhostility Group Median Range Median Range Median Range Spasmodic torticollis 4-0 1-10 3.0* 0- 8 150 4-32 Writer's cramp 50* 2- 6 70 3- 7 20-0* 8-25 Gilles de la Tourette 5-5* 2-10 5-0 0-11 22-5* 533 Controls 301 2- 8 40 0- 8 13-0 5-27$ * < 0-05; $ < 0-01 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.Suppl.90 on 1 June 1989. Downloaded from

Psychopathology and movement disorders: a new perspective on the Gilles de la Tourette syndrome 93 differences from controls while patients with the Gilles toms of the Gilles de la Tourette syndrome are shown de la Tourette syndrome scored higher on acting out in table 5. hostility than controls but were no different from Obsessive compulsive behaviour was significantly patients with the other two movement disorders on associated with the symptom offeeling forced to touch their scores of acting out hostility or criticism of and shoulder shrugging. The clinical diagnosis of others. When the sum of hostility scores were obsessive compulsive behaviour was significantly analysed, Gilles de la Tourette patients scored sig- associated with high scoring on both the Middlesex nificantly higher than the other three groups. Hospital questionnaire obsessional scale and the These data suggest that aggressive behaviour and Leyton obsessional inventory. Specifically, coprolalia self mutilation are not uncommonly reported in was significantly related to the Leyton obsessional patients with the Gilles de la Tourette syndrome, and inventory symptom and total scores, while echo the types of behaviours noted may have forensic phenomena were significantly related to high relevance. and further be amenable to and need Middlesex Hospital obsessional scores and the Leyton specific treatment. The comparison between the obsessional index trait scores. movement disorders suggests that differences with Echo phenomena were further associated in this regard to this aspect of behaviour may exist, patients study with the severity of the Gilles de la Tourette with Gilles de la Tourette syndrome being significantly syndrome as measured by the total number and different from those with writer's cramp and spas- cumulative number of motor tics ever. It should be modic torticollis. We have further identified differen- noted that our clinical diagnosis of obsessive compul- ces with regard to hostility between writer's cramp and sive disorder was based on clinical features and spasmodic torticollis. This is particularly interesting specifically did not include urges to tic, vocalise or to with regard to writer's cramp, as others have noted touch. Typical examples of obsessive compulsive difficulty in expressing anger and hostility in this behaviour were checking rituals, smelling of objects, condition'4 and significantly higher forearm tension avoiding certain eating utensils, repetitive rituals, Protected by copyright. has been noted during electromyographic investiga- folding rituals, walking on squares or lines on the tions of subjects with writer's cramp when discussing pavement, walking in the middle of a busy road to topics concerning anger and hostility.'5 The fact that prevent anything bad or untoward happening, exces- we find significant associations between scaled assess- sive hand washing, arithmomania and obsessions of ments of aggressive behaviour and some of the key doing harm to close relatives. motor symptoms ofthe Gilles de la Tourette syndrome In view of the high frequency of obsessive compul- suggest that this link may reflect underlying biological sive behaviour reported in the study, a cross cultural associations between the two phenomena. comparison has been carried out in conjunction with a Obsessive compulsive disorder In the National group from Los Angeles.'6 A questionnaire was con- Hospital study, 33 patients admitted to obsessive structed specifically to elicit obsessive compulsive compulsive behaviour and/or rituals. This was behaviour which was given to five groups of patients. associated with significantly higher scores on the These were 31 Gilles de la Tourette patients from the obsessional rating scales, but no relationship was United States, and 33 from Great Britain; twelve found between obsessive compulsive disorder scores patients met DSM III criteria for obsessive compulsive and depression scores. The associations between disorder and had no tics, and two control groups were obsessive compulsive behaviour and the motor symp- used, one consisting of patients with differing neuro- http://jnnp.bmj.com/

Table 5 Relationship between certain symptoms ofthe Gilles de la Tourette syndrome and obsessional behaviour Obsessive compulsive phenomena Obsessive Leyton Obsessional Inventory MHQ Symptoms or compulsive Obsessional demographic details behaviour Symptom Trait Total Scale on September 27, 2021 by guest. Forced to touch Coprophenomena *(Coprolalia) *(Coprolalia) Echophenomena * § Family history *Family history of *With family history *With family history TS or tics of psychiatric of TS or tics illness Tic tShoulder shrug lWith :With tWith butyrophenones butyrophenones butyrophenones t< 0-05;t<0-01;t< 0005;§< 0001. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.Suppl.90 on 1 June 1989. Downloaded from

94 Trimble psychiatric disorders and the other of 41 normals. Conclusions Obsessive compulsive disorder patients had the high- est mean score on this scale. Using the same criteria, With a little reflection, it seems obvious that 58% and 45% ofthe USA and British patients with the movements and emotions are interconnected. Just Gilles de la Tourette syndrome respectively scored in consider the word emotion itself, composed entirely of the same range as those with obsessive compulsive the word motion with the prefix "e". Although such disorders in comparison with 12% and 18% in the two associations were discussed by the neuropsychiatrists control groups. This study therefore confirmed the of the late 19th century, until recently they have been frequency of obsessive compulsive disorder in the relatively neglected with the exception of a few condition, and its presence in different cultural talented writers over the years, Smith Ely Jelliffe, and settings. Richard Hunter being outstanding examples.20 The Thus obsessive compulsive disorder is a feature of Gilles de la Tourette syndrome is widely regatded as a the Gilles de la Tourette syndrome, and we have tic disorder, but as the data in this paper emphasise, highlighted intimate links between motor symptoms, the tics are but one reflection of the pleomorphic severity ofthe disorder and these obsessive compulsive phenomenology that these patients display. Even phenomena. This has implications for treatment, since those who have expressed only an interest in the in our experience the obsessive compulsive pheno- movement aspects ofthis condition must surely reflect mena can be considerably more disabling to patients on the wide panoply of movements other than tics than their tics. Psychopharmacological treatments are displayed by these patients. Not only would this available including drugs with selective 5HT uptake include a number of the compulsive rituals described, inhibitor properties, but alternative treatments but brief discussion with many patients with the include for the majority and, in condition will reveal their diverse complicated severe cases, psychosurgery. movements which form part oftheir overall abnormal Confirmation of the close association movement spectrum. The between gait disorders, genuflexions, Protected by copyright. obsessive compulsive symptoms and motor symptoms stereotopes, habits and mannerisms, must all be has led to a conceptual shift with regard to an considered along with the symptoms described here as understanding of the syndrome. Data from recent part of the complex behavioural repertoire of patients family studies'7 suggest that the Gilles de la Tourette with the syndrome. Bliss20 a life long sufferer from the syndrome and obsessive compulsive phenomena are Gilles de la Tourette syndrome, described his self aetiologically related with a common genetic basis. observation ofhis symptoms and comments thus "the Some authors believe it to be inherited as an TS movement is not the whole message. The TS autosomal dominant trait, with reduced movement is not rhythmic, spasmodic or involuntary. and variable expressivity'8 such that when family 14 The movement only seems involuntary because of the members with the Gilles de la Tourette syndrome, or instant capitulation to the unrecognised sensory chronic motor tics or obsessive compulsive behaviours stimulus. It can be detected and interrupted when in are included, the penetrance may approach 100% in progress and at any stage". Bliss rails over clinical males and 70% in females. Obsessive compulsive evaluations which concentrate only on tics, noting that behaviour is thus seen as a major phenotypic form of "the movement, even ifgrotesque and miserable, is not expression ofthe Gilles de la Tourette syndrome . the most important part of TS activity". Interestingly, in the National Hospitals study, further confirmation is found. Thus, obsessive compulsive http://jnnp.bmj.com/ behaviour, and scoring on the Leyton obsessional References inventory by Probands, were associated with a family history of Gilles de la Tourette syndrome or tics. I Trimble MR. . Chichester: J Wiley and Sons, 1981. The other side of this relationship is now being 2 Lishman A. Organic Psychiatry 2nd ed. Oxford: Blackwells, 1988. explored. For example, Pitman et al'9 compared 16 3 Marsden CD. Movement Disorders in Neuropsychiatry. In: patients with the Gilles de la Tourette syndrome with Reynolds EH, Trimble MR, eds. The Bridge between Neurology 16 patients who had obsessive compulsive disorder and Psychiatry. Edinburgh: Churchill Livingstone, 1989. 4 Gilles de la Tourette G. Etude sur une affection nerveuse on September 27, 2021 by guest. and 16 normal controls. In addition to the high caracteris&e par de l'incoordination motrice accompagnee incidence of obsessive compulsive behaviour in Gilles d'echolalie et de coprolalie. Archives de Neurologie 1885;9: de la Tourette patients, there was a significantly 19-42, 158-200. greater incidence of tics in patients with obsessive 5 Shapiro AK, Shapiro ES, Bruun RD, Sweet RD. Gilles de la Tourette Syndrome. New York: Raven Press, 1978. compulsive disorder and their relatives. The tics 6 Cohen D, Bruun R. Gilles de la Tourette Syndrome and Tic generally preceded the development of the obsessive Disorders. New York: J Wiley and Sons, 1988. compulsive behaviour. 7 American Psychiatric Association. Diagnostic and Statistical J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.52.Suppl.90 on 1 June 1989. Downloaded from

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