Journal ofNeurology, Neurosurgery, 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 lobotomies.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 cingulum. 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 globus pallidus 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 skull 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 frontal lobe. 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 cingulate cortex involvement in the both pre- and post-operatively. He was dis- pathogenesis ofGilles de la Tourette syndrome charged after two months post-operative comes