Gilles De La Tourette's Syndrome- a Case Report

GILLES DE LA TOURETTE’S SYNDROME- A CASE REPORT.

E.O Adayonfo (MBBS, Benin) Departmentof Mental Health, University of Benin Teaching Hospital, Benin City, Nigeria.

Correspondence: Dr Adayonfo EO. Mental Health Department, University of Benin Teaching Hospital, Benin City, Nigeria. E-mail: [email protected]

ABSTRACT aristocratic lady lived as a recluse and This case report is on Gilles de la ticked and blasphemed1. Her Tourette’s syndrome, a disorder of disturbance began when she was movement and sound. seven years old and persisted until her It is a very uncommon diagnosis that death at the age of eighty years. was made on a female 29 years old During the closing years of the 19th Nigerian undergraduate. The diagnosis century to the mid 1900’s the was predicated on clinical features, syndrome was well documented and like virtually all psychiatric diagnoses. extensively reported. It had seemed to Management was behavioural disappear as interest was lost in it. In interventions and low dose haloperidol 1978 Shapiro and his colleagues with resultant complete remission. published a comprehensive This case brings to the fore the need multidisciplinary monograph about the for appropriate referral and high condition and thereafter Gilles de la clinical diagnostic acumen. Tourette’s syndrome was accepted as Key words: Gilles de la Tourette’s a specific entity4. syndrome, uncommon diagnosis, Several historic figures might have clinical features, haloperidol, been affected by this syndrome, behavioural interventions) including Prince Conde, a member of the French royal family, Wolfgang INTRODUCTION Amadeus Mozart and Dr. Samuel Gilles de la Tourette’s syndrome is a Johnson, the British diarist1,5. rare psycho-neurological tic disorder1. A diagnosis of the syndrome is made Tics are defined as rapid and repetitive when there are multiple motor tics and muscle contractions resulting in at least one vocal tic, occurring many movements and or vocalizations that times a day for nearly everyday for are experienced as involuntary2. more than one year6. The syndrome was named by Jean The life time prevalence of the Martin Charcot after the French syndrome is estimated to be 4 to 5 per neuropsychiatrist Georges Albert 10,000; commoner in children and 3 Edouard Brutus Gilles de la Tourette 3. times more in males2,7. The motor tics In 1884 Gilles described nine patients usually precede the vocal tics 2. who were affected with tics Examples of motor tics that occur in characterized by echolalia, pallilalia, this syndrome are eye blinking, facial coprolalia, a want for touch and twitching or grimacing, shoulder stuttering3. One of the nine patients shrugging, neck jerking, grooming was the Marquise de Dampierre. This behaviour, smelling of objects,

jumping, touching behaviour, These abnormal movements and echopraxia, copropraxia; while the sounds could happen at the same time vocal tics include grunting, sighing, or separately. They have no functional throat clearing, coughing, sniffing, significance and do not follow a snorting, barking, coprolalia, pallilalia particular pattern. They are sudden and echolalia2. and rapid, with each episode lasting The aetiological factors are numerous for about a minute. These symptoms and could be idiopathic. The factors have remained stable. She had no full include hereditary, psychogenic, post- control over them but was sometimes traumatic, post-central nervous system able to suppress them. The abnormal infections, derangement of the movements and sounds occur biogenic amines, drugs like everyday, sometimes several times a methylphenidate, levo dopa, long-term day. She could predict their chlorpromazine therapy etc.8-17. occurrence but unable to tell what There was no local literature found on brings them up. The episodes were Gilles de la Tourette’s syndrome on self-limiting. literature review, possibly because of its rarity. The patient maintained awareness of self, the event and able to recall the CASE REPORT experience; communicates normally in A 29 year old female Nigerian the absence of vocal tics. undergraduate was referred from the It has never happened in her sleep, or neurology team of University of Benin caused her road traffic or domestic Teaching Hospital to the psychiatric accident, but has occurred team of same hospital. occasionally in the class and church. She was referred on account of She refuted hearing strange voices, abnormal movements and sounds of claiming they are her thoughts. She two years duration and a one year thinks of herself negatively because of history of fleeting 2nd person auditory the above symptoms. hallucination. No history of fever, loss of She was apparently well until 2 years consciousness, headache, seizures, prior to presentation when she heat or cold intolerance, sore throat, suddenly began to observe abnormal carbon monoxide poisoning, movements. The eyes could suddenly substance use disorder, tremors, close and she would be unable to dancing movements or regularly open them, her head could turn to one prescribed medication was elicited. side, or she could be grimacing, Also, no psycho-social stressors grinning, smiling or showing her teeth. predated these symptoms and no The neck and the trunk sometimes family history of similar condition or twist. The head or any of the limbs mental illness generally. could shake individually or in In her mental state evaluation there combination. One or both knees could were no thought disorders, compulsive suddenly bend. Some times her mouth acts or cognitive deficits. However becomes as if glued together and she several episodes of abnormal motor would be unable to talk. behaviours and vocalizations which About six months later, she also began consisted of stretching and turning the to make abnormal sounds. She could neck, shrugging the shoulders, facial hiss, grunt, or at times actually spoke grimacing, flexing elbow and words or make sentences that no one shoulders; as well as hisses, grunts or she could understand. and sniffs were extravagantly

manifested during the evaluation. She able to achieve complete remission pointed at the reviewing doctor, made within ten weeks of treatment further incoherent speeches as if she were buttresses this assertion. abusing the doctor. They were sudden, The patient had these symptoms for rapid, non-rhythmic, and stereotypic two years during which she suffered and appear to be involuntary. They psychologically, socially and lasted between 30 and 60 seconds. academically. She was conscious and accessible The imperative of a high diagnostic during each episode. acumen is emphasized. Physical examination including central Human movement involves an nervous system was normal. astonishing complex interaction of the A diagnosis of Gilles de la Tourette’s brain, nervous system and muscles; syndrome was made. even a simple action such as picking She could not afford a cranial up a biro engages several different computed tomographic scan. This parts of the brain 20. The conscious would have ruled out intracranial thought areas of the brain trigger the neoplasm. Serum calcium and motor area to send signals to the phosphorus were normal. This was muscles of the arm, as the movement done because their derangement begins, sensors in the arm are could cause abnormal movement. activated, sending signals back into Urinalysis was also normal. the different areas of the brain that Individual psychotherapy and interpret them and then send further behavioural therapy were commenced. messages to the motor area to fine She was educated on the disorder. tune power, speed, coordination and The prognosis was discussed with her balance 20. With this level of and she was helped to gain insight into complexity, it is not surprising the disorder. movement disorders occur. She was thought self monitoring, and Other conditions in which movement how to control or modify the disorder occur are stereotypic symptoms. She was reassured. movement disorder 21,Parkinson’s She was also put on Tablets disease,parkinsonism,Parkinson-plus Haloperidol 2.5 mg daily and trial of syndromes, Huntington’s disease, anticonvulsants. She did not comply Wilson’s disease,inherited ataxias, with medications because of side essential tremors, restless leg effects. syndrome, dystonia, cerebrovascular Within ten weeks of treatment she had accident, cerebral palsy, complete remission. encephalopathies, intoxication, It is noteworthy that the emphasis of poisoning by carbon monoxide, the treatment was on behavioural cyanide, methanol or manganese 22. modification to which unarguably she The diagnosis of Gilles de la Tourette’s responded. syndrome is a clinical one. Brain imaging techniques and other DISCUSSION investigations are done to rule out Some authorities opine that most other causes of movement and sound cases of tic disorder including Guiles disorder. de la Tourette’s syndrome start as Management of this syndrome habit. This habit progresses to become includes behavioural interventions involuntary and self perpetuating. such as habit reversal techniques, The fact that this patient who was not massed (negative) practice, self compliant with her medication was

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