arabinoside therapy of biopsy-proved herpes simplex encephalitis: National Institute of Allergy and Infectious Diseases Collaborative Antiviral Study.’ New England Journal of Medicine, 291, 289-294. - Alford, C. A., Hirsch, M. S., Schooley, R. T.,
tye-movement Tics in Children Sharon Binyon Michael Prendergast
A tic is an abrupt, jerking, repetitive movement involving discrete muscle groups. It mimics a normal co-ordinated movement, varies in intensity and lacks rhythmicity (Lees 1985). Tics can sometimes be suppressed voluntarily for short periods, but this may lead to mounting tension. Ticquers can often imitate their own tics on request. Although it has been thought that tics are a manifestation of underlying psychological conflict, more recent evidence suggests that they may be of organic origin, exacerbated by stress. They persist during sleep; EEG pre-movement potential studies suggest a subcortical origin (Obeso el al. 1981) and an abnormality in dopamine transmission has been postulated (Lees 1985). Tics are common in childhood, with prevalence reported as high as 13 per cent between the ages of six and 12 years (Lapouse and Monk 1964). The majority of these will be transient, and improvement occurs spontaneously with age (Corbett et al. 1969). The most frequent type of tics involve facial muscles, and frequency decreases moving down the body. Gilles de la Tourette syndrome comprises multiple motor tics and one or more vocal tics, not necessarily concurrently, over a period of more than one year, with onset before the age of 21
Luby, J. P., Aoki, F. Y., Hanley, D., Nahmias, A. J., Soong, S.-J., and NlAID Collaborative Antiviral Study Group (1986) ‘Vidarabine versu? acyclovir therapy in herpes simplex encephalitis. New England Journal of Medicine, 314, 144- 149.
(American Psychiatric Association 1987). Family studies point to a genetic basis for Tourette syndrome (Pauls and Leckman 1986). Although blinking and winking tics are well known, eye-movement tics are less familiar and if not recognised as such, may be mistaken for more serious pathology. We describe three children who presented with conjugate eyemovement tics. CASE 1 L.B. presented in 1987 at the age of eight years, having been referred by his family doctor to Birmingham Children’s Hospital. His mother described episodes in which his eyes showed rapid, brief, transient conjugate movements upward and to either left or right. These were preceded by a sensation of pain in the right eye and terminated by one or two eye blinks. These spontaneous eyemovements occurred frequently. He was able to imitate his tics upon request in the clinic. The movements had started at the age of six to seven years and his mother could identify no obvious precipitants. He showed no other tic-like movements and there was no family history. He was delivered normally at term, weighing 39508, and developed normally in all other respects. His height and weight were on the 75th to 90th centiles and other physical examinations, including full neurological examination and ophthalmological testing, were normal. An EEG recorded during these eye movements was normal and there. were no associated changes in cortical activity. The diagnosis was isolated eye-movement tics and his mother was reassured. At follow-up six months later the frequency of the tics had decreased considerably. They later disappeared. CASE 2 M.D. was referred in 1988 to the Convulsion Clinic at Birmingham Children’s Hospital at the age of 12 years. His parents described episodes of brief, transient, conjugate eye-deviations upward and to the left. They were preceded by a sensation in the nasal part of the left cornea and were associated with turning the head in the direction of the eye movements. M.D. also had other tics in the form of frequent blinking and throat-clearing, and he could imitate these in the clinic on request. He was
delivered normally at term, weighing 27808, but had an apnoeic attack two to three hours after birth. He had been seen previously at the Children’s Hospital in 1982, following a single seizure at the age of seven years. At that time he had had an abnormal EEG, with spikes and complexes noted bilaterally, but more frequently on the left, and C T scan showed asymmetrical ventricles, suggestive of minor atrophy on the left. He is colour blind and had nocturnal enuresis until the age of 11 years. There were minor educational difficulties requiring remedial support in normal school from an early age, and his concentration continues to be poor. His weight was on the 97th centile, height on the 90th centile. Physical examination and remaining neurological examination were normal, as was a further EEG. A diagnosis was made of multiple tics, including eyemovement tics. Management was again conservative and his tics have improved considerably during follow-up of six months. CASE 3 J.B. was referred for assessment of pervasive developmental problems to the Department of Child Psychiatry at the Maudsley Hospital at the age of 14% years. When first seen, he was noted to have bilateral conjugate movements of the eyes upward, outward and predominantly to the left, but occasionally to the right. They were associated with blinking. Over the course of the following year he developed many more tics in the head and neck, spreading down to involve the trunk and limbs. There were also complex movements involving these parts of the body. He began jumping up and down on the spot, sometimes to the point of exhaustion, and vocal tics appeared; grunts and barks progressed to frank coprolalia. His tics were worse when he was left alone with his widowed mother and when he was under stress. He described his problem as ‘twitches’, and said that he felt a tension which compelled him to twitch. He believed his twitches to be related to years of contact with a severely disabled boy called Dean, who went to the same school. He talked about Dean being inside him and making him twitch. He sometimes referred to the twitches as an indication that he would end up as severely disabled as Dean. At other times he claimed that by performing the twitches he was getting Dean out of his system. He was the only child of older parents. His mother was 35 when she became pregnant and had been taking anticonvulsants for epilepsy. She had high blood-pressure during the pregnancy and he was born by lower-segment caesarean section for placenta praevia, weighing 38608. The neonatal period was uneventful. Although he said ‘Oh dear’ at the age of nine months, this was not repeated. He did not use words with meaning until 3 % years, or sentences until age six. He used names instead of pronouns until age eight. He did not engage in to and fro conversation, but liked repetitive questions and answers. His play showed stereotyped and ritualistic elements, including lining up and spinning objects. There was insistence on sameness. He gazed normally at his mother and father, but not at other people. He developed strong attachments to a pillow and a toy monkey, but would not notice if his mother left the
room. He shunned the company of other children and was socially odd. There was also a history of feeding faddiness, fear of animals and minor selfdestructive behaviour. On admission his speech was pedantic, with unusual intonation and little reciprocity. Use of gaze was still unusual. He preferred to remain on his own, but also kissed and touched female nurses inappropriately. Physical examination was normal, apart from motor tics, vocal tics, a large, flat, pale abdominal naevus and an undescended right testicle. He had a performance IQ of 58 (Wechsler Intelligence Scale for Children-Revised). EEG recorded during his tics showed no changes in cortical activity associated with the eye movements. CT scan and copper studies were normal and a thick blood film did not show acanthocytes. The final diagnosis was atypical infantile autism, with mild to moderate learning difficulties complicated by Tourette syndrome. He was treated as an inpatient. Pimozide and behavioural methods produced some improvement in the tics.
Discussion These children all showed conjugate eyemovement tics, either in isolation or in combination with other tics. Patient 3 is unusual, but the coincidence of infantile autism and Tourette syndrome has been reported before (see Kano el a!. 1988). Kinnier Wilson’s translation of Meige and Feindel’s monograph Les Tics et Leur Traitement in 1907 describes tics involving the extrinsic muscles of the eye. They wrote: ‘tic of the eyeball is generally associated with other tics, ocular or facial, but it may occur alone and bear a resemblance to nystagmus’. Since then, there have been few descriptions in the literature. In 1981, London and Kirschen reported the case of a boy who presented with a five-day history of brief, conjugate deviation of the eyes to the right and upward at a 45” angle, occurring about four times a minute. Three months later they were replaced by a mouth twitch, and he was symptom-free one year after onset. In 1984, Frankel and Cummings reported 29 patients meeting the diagnostic criteria for Tourette syndrome. Four had eye-rolling tics and others had gaze abnormalities, including staring and forced gaze deviation, similar to that seen in oculogyric crises, but no further clinical details were provided. All these patients had blepharospasm and most were taking neuroleptics or other medication. Neuroleptics have been implicated in a number
of movement disorders, including tics and oculogyric crisis (Jankovic 1987). None of our children was taking neuroleptics at the time of their presentation, nor had they been prescribed in the past. Eye-movement tics may present a diagnostic problem if they occur in isolation or in a child on neuroleptics. Neuro-ophthalmological examination is normal between tics. The movements are conjugate, recurrent and stereotyped: they are distinguishable from nystagmus, ocular flutter and ocular myoclonus by their lack of rhythmicity, and from oculogyric crisis by their brevity. In macro square-wave jerks, which are spontaneous horizontal saccades, the eyes fix momentarily before returning to target (Miller 1985). The wild, chaotic eyemovements of opsoclonus (saccadomania) should not cause confusion. The differential diagnosis of tics includes epilepsy. A child’s ability t o imitate his tics on request is a useful indicant that consciousness is not impaired. As demonstrated by cases 1 and 3, there are no EEG changes in cortical activity during eye-movement tics. In 1980, Joseph Bliss, in his personal description of the sensory experiences of Tourette sydnrome, suggested that tics were actually secondary to a sensation in part of the body which led t o the urge to perform the particular movement. Two of our cases described a corneal sensation preceding their tics. Ophthalmological
inspection for causes of local irritation is prudent if this symptom is of recent onset. Tics in themselves are not normally an indication for further aetiological investigation, the need for which is determined by additional findings in the history or on examination. Treatment of tics consists mainly of explanation and reassurance for patient and parents. The tics are best ignored and parents’ attention refocused onto more positive aspects of their child’s behaviour. Neuroleptics and other medication are reserved for severe and disabling tic disorders. The long-term prognosis of childhood tic disorder is reasonably good, with approximately two-thirds showing spontaneous remission (Corbett et al. 1969). The possibility of progression to a more chronic tic disorder or Tourette syndrome must always be considered, as occurred in case 3, and follow-up of these children is advisable while the tics persist. Accepted for publication 6th November 1990. Acknowledgements The authors are grateful to Professor Michael Rutter for permission to report case 3, who was under his care, and to Claire Brittain for typing the manuscript. Authors’ Appointments Sharon E. Binyon, Senior Registrar in Psychiatry, Walsgrave Hospital, Coventry. *Michael Prendergast, Consultant Child Psychiatrist, The Children’s Hospital, Ladywood Middleway, Birmingham B16 8ET. *Correspondence to second author.
SUMMARY Tics are relatively common in childhood, especially between the ages of six to 12 years. While eyeblinking and eye-winking tics are well recognised, eye-movement tics are not. Three children with conjugate eye-movement tics, occurring alone or in combination with other tics, are described and the differential diagnosis is discussed. Treatment of tics in the first instance comprises reassurance and explanation. Detailed further investigation is not indicated, but follow-up is desirable while tics persist, since some children may go on t o develop Tourette syndrome or chronic tic disorder. RESUME Tics de mouvements oculaires chez I’enfant Les tics sont relativement communs durant I’enfance surtout entre six et 12 ans. Alors que les tics de clignement et de fermeture des yeux sont bien reconnus, les tics de mouvements oculaires ne le sont pas. Trois enfants avec des tics de mouvements oculaires conjuguts survenant seuls ou en combinaison avec d’autres tics sont etudies et le diagnostic differentiel discute. Le traitement de premiere intention des tics consiste a rassurer et a expliquer. Une investigation ulterieure n’est pas indiquee mais le suivi est souhaitable si les tics persistent puisque certains enfants peuvent evoluer vers un syndrome de la Tourette ou une maladie chronique des tics.
ZUSAMMENFASSUNG A ugenbewegungsticks bei Kindern Ticks treten im Kindesalter relativ haufig auf, besonders zwischen sechs und 12 Jahren. Wahrend Blinzel-und Zwinkerticks gut bekannt sind, sind Augenbewegungsticks eher ungewohnlich. Drei Kinder mit beidseitigen Augenbewegungsticks-allein oder in Verbindgung mit anderen Ticks-
werden beschrieben, und die Differentialdiagnose wird diskutiert. Die Tickbehandlung sollte primar beruhigend und erklarend sein. Weitere detailierte Untersuchungen sind nicht erforderlich, wenn die Ticks jedoch fortbestehen, sollten Kontrollen durchgefuhrt werden, d a manche Kinder im weiteren Verlauf ein Tourette Syndrom oder ein chronisches Tickverhalten entwickeln. RESUMEN Tics oculares en niiios Los tics son relativamente corrientes en la infancia, especialmente entre 10s seis y 10s 12 ailos. Mientras que el cerrar 10s ojos y el guiilar se reconocen con facilidad, no ocurre asi con 10s movimientos oculares. Se describen tres niilos con tics de movimientos conjugados d e 10s ojos solos o en combinacion con otros tics. Se discute el diagnostic0 diferencial. El tratamiento d e 10s tics en primera instancia comprende la reafirmacion y la explicacion. No esta indicada u n a mayor investigacion per0 es conveniente un seguimiento mientras persistan 10s tics, ya que algunos niilos pueden desarrollar un sindrome de Tourette o una alteracion ticosa cronica. References American Psychiatric Association (1987) Diagnostic and Statistical Manual Of Mental Disorders, Third Edn., Revised fDSM III-R). Washington, DC: American Psychiatric Association. Bliss, J . (1980) ‘Sensory experiences of Gilles de la Tourette syndrome.’ Archives of General Psychiatry, 31, 1343-1347. Corbett, J. A , , Mathews, A. M., Connell, P. H., Shapiro, D. A. (1969) ‘Tics and Gilles de la Tourette syndrome: a follow up study and critical review.’ British Journal of Psychiatry, 115, 1229- 1241. Frankel, M., Cummings, J . L. (1984) ‘Neuroophthalmic abnormalities in Tourette’s syndrome: functional and anatomic implications.’ Neurology, 34, 359-361. Jankovic, J. (1987) ‘The neurology of tics.’ In Marsden, C . D., Fahn, S. (Eds.) Movement Disorders-2. London: Butterworth. Kano, Y., Ohta, M., Nagai, Y., Yohota, K., Shimizu, Y. (1988) ‘Tourette’s syndrome coupled with fnfantile autism: a prospective study of 2 boys. Japanese Journal of Psychiatry and Neurology, 42, 49-57. Lapouse, R . , Monk, M. A. (1964) ‘Behaviour deviations in a representative sample of children:
variation by sex, age, race, social class and family size.’ American Journal of Orthopsychiatry, 34, 436-446. Lees, A. J . (1985) Tics and Related Disorders. Edinburgh: Churchill Livingstone. London, R., Kirschen, D. (1981) ‘Psychogenic etiology of conjugate gaze disorder.’ Journal of the American Optometric Association, 52, 355-356. Meige, H., Feindel, E. (1907) Tics and their Treatment. (Translated by: Wilson, S. A . K.) London: Appleton. Miller, N. R. (Ed.) (1985) Walsh andHoyt’s Clinical Neurophthalmology, Vol. 2, 4th Edn. Baltimore: Williams & Wilkins. Obeso, J. A., Rothwell, J . C., Marsden, C. D. (1981) ‘Simple tics in Gilles de la Tourette’s syndrome are not prefaced by a normal premovement EEG potential.’ Journal of Neurology, Neurosurgery and Psychiatry, 44, 135-738. Pauls, D. L., Leckman, J . F. (1986) ‘Theinheritance of Gilles de la Tourette syndrome and associated behaviours: evidence for autosomal dominant transmission.’ New England Journal of Medicine, 315, 993-997.