J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.1.1 on 1 February 1973. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry, 1973, 36, 1-9 Neurological features of Gilles de la Tourette's syndrome RICHARD D. SWEET, GAIL E. SOLOMON, HENRIETTE WAYNE, ELAINE SHAPIRO, AND ARTHUR K. SHAPIRO From the Departments of Neurology and Psychiatry, Cornell University Medical College, New York, New York 10021, U.S.A. SUMMARY Clinical neurological examinations of 22 patients with Gilles de la Tourette's syndrome and written reports of examinations of seven other patients are reported. Half the personally exam- ined patients had minor motor asymmetries in addition to the typical motor and vocal tics found in all the patients. Thirty-six per cent of patients were left handed or ambidextrous. Electroencephalo- grams performed on 17 of the 22 patients showed non-specific abnormalities in 12 of them. These findings suggest that a neurological disorder underlies Tourette's syndrome, but they do not clarify its nature. Protected by copyright. Tics may be defined as brief purposeless move- (Yeh, McNay, and Goldberg, 1969). It has been ments of a body part which occur at random suggested that a dopamine excess may underlie intervals and cannot be suppressed for long Tourette's syndrome (Snyder, Taylor, Coyle, and periods oftime. Wilson (1927) believed they were Meyerhoff, 1970), but the actual anatomical or psychogenic in origin, while others have empha- pathophysiological abnormality which might sized developmental (Balthasar, 1957) or in- cause Tourette's syndrome and be controlled by flammatory disorders (Creak and Guttman, haloperidol is still unknown. 1935) of the basal ganglia as possible causes. Clinical neurological techniques have been Gilles de la Tourette (1885) described nine used by several authors in an effort to demon- patients with persistent multiple tics beginning strate a neurological deficit. Fernando (1967) in childhood Five of these patients also had reviewed the world's literature and noted no coprolalia. Tourette linked tics with other rare specific findings, but found reports of abnormal motor and speech disorders, such as latah, electroencephalograms (EEG) in 25%. Corbin, myriachit, and 'jumping Frenchmen', and Goldstein, and Klass (1966) reviewed seven http://jnnp.bmj.com/ emphasized the chronic stable course such patients at the Mayo Clinic and again found no patients followed in adulthood. pertinent changes on examination but found six Subsequent authors have differentiated 'mala- patients with rolandic sharp waves on EEG, four die des tics' from other involuntary motor dis- while awake and two while asleep. Lucas (1970) orders (Kelman, 1965; Chapel, 1970). Both described the EEGs of 12 patients, of which psychiatric and neurological disorders have seven were abnormal. Five showed diffuse mild been invoked as causes of Tourette's syndrome to moderate one had delta slowing, posterior on October 1, 2021 by guest. without conclusive evidence (Kelman, 1965; activity, and one showed bilateral spike and Challas, Chapel, and Jenkins, 1967; Corbett, wave complexes. Mathews, and Cornell, 1969; Morphew and Since subtle neurological abnormalities and Sim, 1969). clinical correlations may escape notice in a few Tourette's syndrome responds best to treat- patients, or in a group of patients examined by ment with haloperidol (Chapel et al., 1964; various physicians, we thought it worthwhile to Shapiro and Shapiro, 1968), a butyrophenone report the findings in a large series of patients compound which blocks dopamine receptors with Tourette's syndrome. 1 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.1.1 on 1 February 1973. Downloaded from 2 Richard D. Sweet, Gail E. Solomon, Henriette Wayne, Elaine Shapiro, and Arthur K. Shapiro ~< Cu0 0 0uo)Q~ .0 Cu 0 oCa la 2 C q a 0 Cu6 z0 f0t ._.C m m) ,Q Q i? 0-" u 04~~~~~~~~0 O~E IC >,c C,>,, :0 -C u (A 0.3 - ut - m 0 bo 0~~~~ 0 0 0~~~~; W Cu C)Cur'?0 4) Cu Cu o Co '. 0; C) Q .u E I~ ~ ~a~e °a" @ Cu0 z Protected by copyright. x m mC)<C U) ut .C=CE tt Y 8 . z U) Cu <,, _.4 <: m ;< Is 0 Po *0 .3 I 0. 0 I~~~~~. el 0 -;; IU, Cu r- d .@ I,, 0 -'S D o °oa C ° CI http://jnnp.bmj.com/ ..I| 0 0 I I l D~~U- . 0, G) C.CC- .2t0 I C EECA4) Co~ 5Q on October 1, 2021 by guest. *u .<ce Cd ~~~~~-Jd C 0% L) ,It I- 0 en e 3R Q 00 0 - t It t 1- *a - NC I m £ 00 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.1.1 on 1 February 1973. Downloaded from Neurologicalfeatures of Gilles de la Tourette's syndrome 3 E0)' 00 2> 0 O> >C 0~~ CZC0C's 0~~0 ~ Cl 4F+ +4 ++ ++ ++ ++ ++ ++ ++ CU bO co 0~~~~~0C 0d CZ Z 0 u o - 0, CUo u- rCd- vU D 0 0 .-~~~~~~C4 CU~ 000 o°° cO 0zEn U0OU :I; 5 t~C- -0' ~~0CZ .-0 .0 0~ CC 0 U)C. 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Twenty-two patients from a series of 34 patients with Four older patients reported delayed sitting, walking, Gilles de la Tourette's syndrome treated at the or talking; the reliability of their retrospective his- Payne Whitney Clinic of the New York Hospital- tories is questionable. Another three patients among Cornell Medical Center were examined neuro- the series of 34 had delayed milestones. Six patients logically. In addition, written reports of neurological among the 34 were late in toilet training and two of examinations of seven other patients in the series the boys who were examined were said to have been were reviewed. At the time ofexamination, 10 patients 'clumsy' in early childhood. were taking haloperidol, 2-8 mg/day and two were The patients were examined at an average age of taking benztropine. One other patient had taken 23 years (range 8-62). Ten of the 22 examined haloperidol and benztropine for three years, but was patients and six of the seven reviewed cases were examined after three weeks without medication. A aged 15 years or less. Seventeen of the 22 examined complete medical history was taken with emphasis and six of the seven reviewed patients were males, a on early life injury or illness, developmental ab- predominance also found in other series (Fernando, normalities, intercurrent illnesses, and medication. 1967). This information was obtained from patients and Symptoms began at an average age of 7 years in their relatives and supplemented by previous both examined and reviewed cases. Initial symptoms medical records. A complete neurological examina- in examined patients consisted of frequent inappro- tion with special emphasis on motor phenomena priate blinking or squinting in 10 patients, facial tics was performed, along with a general medical in four, arm and shoulder movements in four, head examination. Electroencephalograms were performed bobbing in three, and barking in one. Progression of on 17 of the 22 patients using unipolar and bipolar tics from face to proximal limb muscles and theProtected by copyright. montages with 22 scalp leads. All patients were appearance of vocal exclamations within a few years hyperventilated during the recording. Five had both were typical. awake and sleep EEGs and four had photic stimula- Concomitant illnesses noted at the time of tion during EEG recording. Electromyographic examination were cervical spondylosis, hiatal hernia, tracings from surface electrodes over muscles in- and transexualism. A woman had undergone bi- volved in tics were obtained concurrently with the lateral cryothalamotomies with partial relief of EEG in one patient. Paper speed was increased from Tourette's symptoms (Cooper, 1963), and a boy had 3 to 6 cm/sec during EEG-EMG recording to received insulin shock treatment and prochlor- facilitate detection of the start of activity. phenazine for tics during his teens. One boy had infectious mononucleosis three months before the HISTORY Data for all examined patients are sum- onset of his tics and a man gave an anecdotal history marized in Table 1. of 'sleeping sickness' at 11 months of age. Gestation and birth were normal in all but three Handedness was inquired about routinely in the of the personally examined patients. One man had interview. Five of the 22 personally examined required oxygen therapy and blood transfusion patients were strongly left-handed and three others because of neonatal blood considered themselves ambidextrous.