Brief Report

Sign Language Tics in a Prelingually Deaf Man

*Huw R. Morris, MRCP, †Alice J. Thacker, PhD, ‡Peter K. Newman, FRCP, and *Andrew J. Lees, MD

*National Hospital for Neurology and Neurosurgery, Queen Square, London, U.K.; †St. George’s Hospital Medical School, London, U.K.; and ‡Middlesbrough General Hospital, Middlesbrough, Cleveland,U.K.

Summary:

Gilles de la Tourette syndrome is characterized byvocal and motor tics starting in childhood. Vocal tics may beeither noises or words, and the vocal language tics may consistof obscenities (coprolalia) and repetitions of speech that hasbeen heard (echolalia). We describe a prelingually deaf manwho has the full array of tics seen in Gilles de la Tourettesyndrome, but in whom vocal language tics are replaced byequivalent sign language tics. This is, to our knowledge, thefirst report of sign language tics in a person with prelingualdeafness. The implications of this phenomenon for the separa-tion between language and ideas in tics and the equivalence of sign language to spoken language is discussed.

Key Words:

Tic—Coprolalia—Sign language—Tourette’s syndrome.

Tics are motor and vocal actions produced inappropri-ately. They are characteristically suppressible, occur inresponse to an inner premonitory urge, and occur duringsleep. Tics may be simple, consisting of sniffing or throatclearing or other nonverbal sounds, or complex consist-ing of words. Word-based tics may be primitive, emo-tionally charged ejaculations or words, scatologic in con-tent, occurring in a seemingly inappropriate context. Thefunctional anatomy of tics is thought to lie in failure of the gating of cortico-thalamo-striato-cortical circuits.

1,2

The cingulate cortex may be important in the productionof tics and possibly particularly of language tics, becausecingulotomy has been reported to lead to resolution of tics in humans, and stimulation of the cingulate cortex inhumans leads to tic-like obscenities and vocalizations.

The supplementary motor area (SMA) and the mesence-phalic areas are also potentially important in the genera-tion of vocal tics as stimulation of both the SMA inhumans

and mesencephalic grey matter

in non-humanprimates may lead to vocalization. Tics can occasionallyarise in association with a variety of underlying braindisorders such as Sydenham’s chorea, Huntington’s cho-rea, neuro-acanthocytosis, and cerebral tumors, but ticsare most commonly seen in Gilles de la Tourette syn-drome.

Gilles de la Tourette syndrome usually appearsin childhood, overlaps with obsessive-compulsive disor-der, and is often familial.The analysis of vocal language tics is of interest in theassessment of the relationship between environment andtic production. Culturally determined unacceptable ob-scenities (coprolalia) and racial abuse appear to be pref-erentially used in some patients’ tic repertoires.

Cross-cultural analysis of the words used in tics shows thatthere is a bias toward sexual as opposed to religiousobscenities regardless of the patient’s native language.However, it is uncertain whether the use of these wordsis determined by their phonologic content or their se-mantics. While obscenities have culturally determinedmeaning, they are also usually short, explosive wordsconsisting of high-frequency phonemes, and this is truein most languages.

A computer program which ran-domly generates high-frequency phonemes may produceobscenities,

and it has been suggested that the phono-logic features of obscenities determine their appearanceas coprolalia rather than their meaning. Sign language isa nonverbal language which is considered to be a neu-rologically and culturally equivalent language to spokenlanguage, and thus provides the possibility of dissociat-

A videotape accompanies this article.Received September 16, 1999; revision received January 5, 2000.Accepted January 8, 2000.Address correspondence and reprint requests to Andrew J. Lees,MD, National Hospital for Neurology and Neurosurgery, QueenSquare, London WC1N 3BG, U.K.

Movement Disorders

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ing the phonologic and semantic content of tics. We de-scribe a prelingually deaf man with Gilles de la Tourettesyndrome who used British sign language and developedsign language tics.

CASE REPORT

A 29-year-old deaf man was admitted for evaluationof his movement disorder. He was born at full term fol-lowing a normal delivery and pregnancy but was foundto be profoundly deaf at the age of 10 months. He pro-duced “mmm” sounds to indicate contentment and plain-tive screeches to indicate unhappiness. At around the ageof 5 he was first noted by his parents to have repetitiveabnormal movements such as pausing when he walked tokick one calf with the other foot, bending over to look back through his legs, holding his thumb and forefingerover his thyroid cartilage, making screeching noises, andrepetitively making “ppp” or “prrrr” sounds. He also de-veloped a number of facial twitches and eye blinks thatwere thought to indicate nervousness. A number of otheractions were interpreted as indicative of behavioral dis-turbance such as tipping cigarette ends into cups of cof-fee, throwing possessions onto open fires, and in lateryears fire-raising. His communication up until this timewas limited to primitive gestures and signs. At 7 years of age he was taught sign language and finger spelling andmany of his behavioral problems improved. Throughouthis adolescence his tics continued, and he developed newtics including licking and smacking of the lips, clickingnoises, and rolling of the eyes. When he mixed withchildren with normal hearing, he learned the obscenepalm-back V sign and this was incorporated into hisrepertoire of tics, together with grabbing of his genitals.Psychometric testing during this period revealed that hewas of high intelligence with an IQ of 120 and also thathe was a gifted calculator, in particular being able topredict birth week days from birth dates and years. Ob-sessional traits emerged which included an obsessive de-sire for neatness and tidiness in his possessions and per-sonal life which extended to the arrangement of the foodon his plate and a refusal to handle copper-colored coins.He developed an interest in jigsaw puzzles which wereusually completed in approximately 1 hour and whichwere carefully filed in a color-coded collection of manyhundreds of puzzles. He was thought to have autistictraits, with an apparent difficulty in interacting with otherchildren and adolescents. Language assessment revealedthat he had significant pragmatic deficits, including fail-ure to signal when he did not understand questions, leav-ing his interlocutor to attempt to interpret his often irrel-evant, bizarre-appearing responses. He did not check thathis interlocutor was able to follow his signing.At the age of 20 his tic behavior markedly worsenedand his parents felt that he had developed Gilles de laTourette syndrome (see accompanying video). He madefrequent loud screeching noises (up to 90 dB) and com-plex motor movements such as lying down to touch orkiss the floor, bending sideways, striking aggressive box-ers’ poses, and hand slapping. It became clear at this timethat sign language was an integral part of the expressionof his range of tics. He would randomly intersperse ob-scene signs and finger-spelled obscene words in normalconversation. Signed tic production seemed to be con-textually sensitive. He repeatedly produced sexual signswhen conversing with women and he repeatedly inter-spersed the sign for vomit during a cooking class. It wasnoticed that his tics were suppressed during tasks involv-ing concentration. When directly asked about the tics hewould deny that they had occurred, and no history of asuppressible urge to tic was obtained. He appeared, how-ever, to be aware of and to “convert” nonlinguistic ges-tures, for example, converting a stabbing movement to-ward the interlocutor’s face into a caress.He was admitted for investigation and was found tohave a pigmentary retinopathy with optic atrophy. Mag-netic resonance imaging of the brain was essentially nor-mal. Several peripheral blood films showed no acantho-cytes. Mitochondrial DNA analysis was negative for the3243 and 8993 mutations. A detailed neuropsychologicalassessment showed that he had normal nonverbal intel-ligence but that he had a particular deficit in memory forfaces.

A neuro-otology examination showed total hear-ing loss for all frequencies in both ears. His hearing losswas thought to be the result of either a congenital infec-tion or Usher syndrome. Treatment with haloperidol pro-duced a partial but unsustained improvement in the ticfrequency.

DISCUSSION

This is the first reported case of sign language tics ina prelingually deaf individual. Sign language tics werereported by Lang in 1993 in a woman with preexistingGilles de la Tourette syndrome who developed sign lan-guage tics following the learning of sign language as atherapeutic exercise in adulthood.

The current reportdemonstrates that sign language tics and Gilles de laTourette syndrome can also occur in the prelinguallydeaf in whom all language acquisition and comprehen-sion has been visual.There are several possible explanations for the selec-tive production of obscenities as opposed to other so-cially acceptable utterances in Gilles de la Tourette syn-drome. First, obscenities may be produced by hearing