Journal ofNeurology, , and Psychiatry 1990;53:691-694 691

SHORT REPORT J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.53.8.691 on 1 August 1990. Downloaded from

The treatment of Gilles de la Tourette syndrome by limbic leucotomy

M Robertson, M Doran, M Trimble, A J Lees

Abstract Case report A patient with Giles de la Tourette syn- A nineteen year old trainee computer drome and severe self-injurious compul- programmer was referred to the National sions who had failed to respond to drug Hospital for Nervous Diseases with a suspec- treatment and behavioural therapy ob- ted diagnosis of Gilles de la Tourette syn- tained a complete and sustained resolu- drome. He had been born at term by tion of his destructive behaviour and Caesarian section following an ante partum improvement in his tics following haemorrhage due to placenta previa; he was bilateral limbic leucotomy. nursed in an incubator for the first four days of his life. Developmental milestones were normal and he had had no severe febrile illnesses in the first five years of life. At five The Gilles de la Tourette syndrome is charac- and a half years he developed inappropriate terised by the presence of multiple motor and eye-winking and facial grimacing. vocal tics which wax and wane in severity. Over the next ten years he displayed a wide Other commonly associated features include repertoire of motor tics. At fifteen he was echo-phenomena, copro-phenomena, atten- compelled to open and close doors and tion deficits and obsessional-compulsive showed other forms of checking behaviour. behaviour. The disorder is usually lifelong, He had distasteful intrusive thoughts related but the tics may be helped by dopamine to lurid scenes he had seen on television. receptor antagonist drugs and the compul- Inapposite vocalisations began a year later sions by behavioural therapy. Self-injurious with grunts which he attempted to incor- behaviour is also common.' porate covertly into conversation. Coprolalia Considerable clinical overlap exists between and self-injurious behaviour began at the primary obsessional-compulsive disorder and same time at seventeen years with head-bang-

Gilles de la Tourette syndrome and it has ing, lip-biting, burning himself with cigarettes http://jnnp.bmj.com/ even been suggested that they may be dif- and putting his hand onto hot frying pans ferent phenotypic expressions of the same being the most destructive activities. He genetic abnormality.2 Compulsive behaviour became fearfully obsessed that he might jump occurs in both disorders but in Gilles de la off high buildings. Two years later he de- Tourette syndrome there appears to be less veloped an irresistible desire to touch the back voluntary resistance and whereas self- of his eye. On several occasions he succeeded The National Hospital injurious behaviour is common it is extremely in this causing orbital haemorrhages and on October 2, 2021 by guest. Protected copyright. for Nervous Diseases, rare in dis- London primary obsessive-compulsive damage to the inner chamber of his eye, Department of order. ulceration of the cornea and abrasions of his Neurology There have been a few neurosurgical eyelids with chemosis. These serious injuries M Doran attempts to alleviate severe cases of Gilles de resulted in several emergency A J Lees admissions to Department of la Tourette syndrome. Improvement in both ophthalmological departments. Both eyes Psychiatry tics and compulsions has been reported foll- were attacked, but the right was favoured and M Trimble owing stereotactic coagulation of the rostral he was unable to give a rational explanation Academic intralaminar and medial thalamic nuclei,34 for this abnormal behaviour. Department of Psychiatry, The lesions in the cerebellar dentate nuclei5 and On examination he had frequent eye-wink- Middlesex Hospital, following frontal .67 Severe ing tics, facial grimaces, neck twitches and London primary obsessive-compulsive disorder has grunts. Both eyes were severely traumatised M Robertson also benefited following surgical lesions in the with conjunctival haemorrhages. A CAT scan Correspondence to: orbital frontal cortex and anterior . of the brain and an electroencephalogram Dr Lees, Department of Neurology, The National In a review of 374 patients from twelve were normal. A pre-operative MRI scan Hospital for Nervous neurosurgical series, 64% of patients revealed a single high signal focus in the Diseases, Queen Square, London WC1N 3BG, improved considerably and a further 24% region of the right of uncer- United Kingdom. moderately.8 We report the case of a man with tain pathological significance (see figure). Received 17 July 1989 Gilles de la Tourette syndrome and Neuropsychological assessments revealed a and in revised form compul- 13 October 1989. sive self-injurious behaviour who was success- WAIS verbal IQ of 117 and a performance IQ Accepted 23 November 1989 fully treated by limbic leucotomy. of 114. 692 Robertson, Doran, Trimble, Lees

Figure (A) MRI scan J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.53.8.691 on 1 August 1990. Downloaded from showing small high signal focus in the right globus pallidus seen in the patient pre-operatively; (B) MRI scan to show the surgical lesionsfollowing bilateral cingulotomy; (C) post-operative MRI scan six monthsfollowing surgery.

hospital he resumed his eye-poking behaviour and increasing doses of sulpiride to 1200 mg, clomipramine to 200 mg and the addition of L-tryptophan failed to improve him. At this time he also started to mutilate his fingers leading to tendon injuries and severe grazing. After detailed discussion with the patient and his family and consideration of his case by an independent Mental Health Review Tribunal he was referred to the Atkinson Morley's Neurosurgical Unit for bilateral limbic leucotomy where considerable experience has been built up over the last twenty years with this procedure.9 While waiting for the opera- tion to be carried out he began to damage his nose and carry out forced Valsalva man-

oeuvres leading to extensive petechial http://jnnp.bmj.com/ haemorrhages. Stereotactic limbic leucotomy was perfor- med with an electro-coagulative probe. Air encephalography was used to localise the ven- tricles on plain films. Pre-operative cal- culations were then carried out to determine the standard fourteen targets. Stimulation studies with a small bipolar electrode were on October 2, 2021 by guest. Protected copyright. He was treated with the D2 receptor conducted for the lower medial quadrant tar- antagonist drug sulpiride 200 mg twice a day get through burr holes, confirmation of the with almost complete resolution of his tics. correct location being taken when transient No improvement in his self-injurious compul- apnoea occurred. Lesions were made in both sions occurred, however, despite a subsequent lower medial quadrants of the frontal lobes increase in the sulpiride to 1000 mg a day in and separate lesions made in each anterior three divided doses. He was admitted to a cingulum.' 1' psychiatric ward as it was felt that he was in The patient was agitated immediately post- imminent danger of enucleating his right eye. operatively, but then made an uneventful At this time he was agitated and pessimistic post-operative recovery and within two days with suicidal thoughts, disturbed sleep and of surgery his compulsions had markedly les- weight loss. He was treated with chlorpro- sened and within six weeks had disappeared thixene, another dopamine antagonist, in completely; he was then treated with further doses up to 500 mg a day and clomipramine behavioural therapy. A post-operative scan 100 mg daily. Twenty four hour personal showed bilateral, almost symmetrical signals nursing care using response prevention be- in the centrum semiovale in the posterior part havioural techniques led to minor improve- of each . These altered signals ment over the two weeks. extended up to small cortical lesions and down However, shortly after his discharge from to the anterior part of the cella media (figure). The treatment of Gilles de la Tourette syndrome 693

A further MRI scan six months later showed a It would seem reasonable to look on self- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.53.8.691 on 1 August 1990. Downloaded from reduction in the size of the altered signal injurious behaviour in Gilles de la Tourette (figure); both the post-operative scans failed patients as a particular form of compulsive to demonstrate the area of altered signal in the behaviour but one which is rarely seen in globus pallidus seen pre-operatively. Post- primary obsessive-compulsive disorders. Self- operatively he had mild apathy, a mild degree inflicted eye injuries are more commonly seen of general intellectual impairment and some in patients with schizophrenia or borderline minor organisational problems and difficulties personality disorders. We have, however, in concentration. His Leyton Obsessional reported a further patient with Gilles de la Score fell from 17 pre-operatively to 8 post- Tourette syndrome and this particular form of operatively and the Maudsley Compulsions mutilatory behaviour.' Check List to 1 post-operatively. He had a Further support for the possibility of low Beck Depression Inventory Score of 4 anterior involvement in the both pre- and post-operatively. He was dis- pathogenesis ofGilles de la Tourette syndrome charged after two months post-operative comes from a positron emission tomographic inpatient care to continue behavioural therapy study of cerebral glucose utilisation in 12 as an outpatient. Over the next two months he untreated patients in which a 15% reduction became more self-motivated and was able to was noted in frontal cingulate and insular start a training programme as a decorator and cortices."6 In their paper on the use of anterior then subsequently to obtain a job. cingulectomies in the treatment of psychiatric On review after two years he was socially illness Whitty et all'7 reported that the independent, free from destructive behaviour appearance of purposeless movements such as and there had been a 75% reduction in the patting the bedclothes or rubbing the face severity of his tics, which were now restricted occurred post-operatively in several patients to a single stereotyped shoulder tic. According and in one case a tic-like sniff developed. to his mother he was now the same as before They also report operating on a twenty five the operation with bouts of verbal aggression, year old schizophrenic who had bizarre and periods of self-doubt and anxiety. He grimaces, mannerisms and echolalia as well as continued on 1600 mg/day of sulpiride. compulsive destructive outbursts who im- proved considerably following surgery. In 1988 at the American Academy of Discussion Neurology, Kurlan et al described two patients The cingulate cortex may be important in the with Gilles de la Tourette syndrome and mediation of emotionally charged language. severely disabling obsessive-compulsive dis- Stimulation of the anterior cingulate cortex in order who were treated with stereotactic radio humans has led to a number of clumsy yet frequency anterior cingulotomy. A 22 year old coordinated movements including: licking and man with rituals which made it impossible for rubbing the fingers and thumb together, glanc- him to dress, converse, eat or maintain urinary ing eye movements, oscillatory hand move- continence had a cingulotomy performed at the ments and touching the lips. These bear some age of seventeen with transient improvement; a similarity to the stereotyped movements often repeat cingulotomy four months later resulted seen in Gilles de la Tourette syndrome.'2 in modest, persistent improvement. A second Stimulation of this area also causes vocalisa- patient aged twenty eight, also with Gilles de la http://jnnp.bmj.com/ tions in squirrel monkeys and there are a few Tourette syndrome, had marked ritualistic reports ofakinetic mutism in humans following slowness and was improved by surgery. bilateral damage. The anterior cingulate cortex Neither of these patients had self-injurious has a rich dopamine innervation from the compulsions.'8 ventrotegmental area ofthe midbrain and there We recommend that limbic leucotomy are extensive connections between this area of should be considered in patients with severe

the and the corpus striatum. self-injurious behaviour related to Gilles de la on October 2, 2021 by guest. Protected copyright. Self-injurious behaviour is frequent in Tourette syndrome. It is unclear at this stage patients with Gilles de la Tourette syndrome whether disabling obsessions or tics and vocal- who are referred to hospital. The dopaminergic isations can be lastingly improved by this system and neuropeptides have been impli- procedure. cated and it is of interest that a recent necropsy examination of a patient with Gilles de la Tourette syndrome showed total absence of dynorphin-like positive woolly fibres in the 1 Robertson MM, Trimble MR, Lees AJ. Self-injurious behaviour and the Gilles de la Tourette syndrome. A dorsal part of the external segment of the clinical study and review of the literature. Psychol Med globus pallidus with similar, but milder, abnor- 1989;19:61 1-26. 2 Pauls DL, Leckman JF. The inheritance of Gilles de la malities in the ventral pallidum.'" However, Tourette syndrome and associated behaviours. New Eng J attempts to modify tics and self-injurious Med 1986;315:993-7. 3 Hassler R, Dieckmann G. Traitement stereotaxique des tics behaviour with the opiate antagonist naloxone et cris inarticules ou copralalique consideres comme have been inconclusive.'4 15 Self-injurious phenomene d'obsession motrice au cours de la maladies de Gilles de la Tourette. Rev Neurol 1970;123:89-106. behaviour seems to be associated in patients 4 De Devitiis E, Errico A, Cerillo A. in with Gilles de la Tourette syndrome with high Gilles de la Tourette syndrome. Acta Neurochir (Wien) (suppl 24) 1977:73. scores on obsessional and hostility measure- 5 Nadvornick P, Sramka M, Lisy L, Suicka I. Experiences ments and there is also a correlation between with dentatomy. Confin Neurol 1972;34:320-4. 6 Baker EFW. Gilles de la Tourette syndrome treated by the severity of tic disorder and the presence of bimedial frontal leucotomy. Canad Med Assoc J self-injurious behaviour.' 1962;86:746-7. 694 Robertson, Doran, Trimble, Lees

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