A case of Gilles de la Tourette's syndrome.

Gilles de la Tourette's syndrome is an uncommon illness associated with repetitive un-voluntary abnormal movements and utterance. It is often associated with other psychiatric morbidities. Management requires awareness of this uncommon illness, keen observation, relevant evaluation, and combination of pharmacology and psychotherapy for an optimal outcome. This case is brought out here for florid presentation and nuances of management.

Gilles de la Tourette's syndrome (GTS) is a neuropsychiatric movement disorder which has onset in childhood. It is characterized by multiple motor tics and one or more vocal/phonic tics, lasting more than a year.[1] Tics are rapid, repetitive, irregular, brief uncontrolled movements, or vocalization.[23] Tics can be temporarily suppressed by volition but appears soon with bursts of movements. It can affect various groups of muscle including laryngeal, pharyngeal, or respiratory ones. Tics generally reduce during periods of mental or physical activity but reappear when idle or relaxed. It may occur in sleep also.[4] GTS is complex syndrome which includes a spectrum of associated behavior problems which may be tic-related inappropriate vocalization or socially embarrassing actions.[5] GTS is not that common with prevalence ranging from 0.4% to 3.8%.[6] Diagnosis of GTS requires a comprehensive assessment of symptoms and progression of the disease over time. GTS is often associated with other psychiatric morbidities like obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD) or both. Management of GTS is generally a multidisciplinary approach involving medication and behavioral intervention. Mild cases of GTS might not require any definite treatment. Treatment may additionally require improving the quality of life and working on social distress caused due to the abnormal movements. Antipsychotics, alpha 2 adrenergic agonist, and stimulants, etc., have been found useful. The presence of OCD may complicate the issue further and may require selective serotonin reuptake inhibitor (SSRI). ADHD is generally tackled with the institution of stimulants.[4] Authors here report a case of GTS, who reported with insidious onset gradually progressive development of multiple motor and vocal tic with associated psychiatric, medical, and social complication. The case is discussed here for the florid nature of the presentation and multiple modalities of intervention in not so common a disorder.

CASE REPORT

A 24-year-old male was referred to Psychiatry Department with insidious onset gradually progressive multiple repetitive abnormal movements of body parts for last 14 years and repetitive abnormal production of sounds and utterances for last 5 years. Individual developed repetitive blinking of eyelids at 10 years of age. These movements were spontaneous and not discomforting. It was not in his control but could stop it for a while. Two weeks later, he developed hopping movements while standing or walking which were spontaneous with varying frequency. Any move to suppress these movements led to an unpleasant sensation. There was no alteration of consciousness, preceding aura, preceding thoughts or impulse, accompanied hallucinations or delusions. Individual subsequently developed frequent holding and releasing movements while riding a scooter as pillion in an attempt to hold the driver. These movements were un-voluntary, repetitive, with variable frequency.

Over the years, movements increased further in number and frequency. Now there were multiple abnormal movements at a time involving upper and lower limb, eyes, and head. These movements used to get exacerbated when he was made fun of by people or when he is excited while playing video games, etc., The same used to get relieved when he used to get engrossed in an activity or while at sleep. Within few months, individual was noted to produce repetitive un-voluntary sounds like grunting, throat clearing which were spontaneous with variable frequency. These bouts were initially once or twice; a month later used to come almost every day.

During the same time, individual developed repeated thoughts of hands being dirty. He found these thoughts as his own, considered it as irrational, however, could not resist and was compelled to wash his hands 3–4 times a day. These acts if resisted led to anxiety and when performed relieved it temporarily. He would also get repetitive unreasonable impulses such as switching off computer without shutting it properly, putting his finger in the electric socket, putting a finger in the hinge of the door, or suddenly laughing aloud for no rhyme or reason. He noticed an increase in movements and sound production over months. There was also associated production of obscene words and abuse which were not clearly in his voluntary control. During the same time only he was noticed by people to be repeating their words and imitating movements unnecessarily. Initially, these used to occur once or twice a month later progressed to once or twice a day. While riding a bicycle due to these abnormal movements, he hit another person and injured his knees. During hospitalization for the same, his abnormal movements were noted. Subsequent he was referred for psychiatric management.

There was the past history of repeated joints pains, joint swelling, and fever during childhood. However, he did not require any long-term medications/injectable antibiotics. There was no family history of mental illness. Mother had obsessive compulsive traits.

On examination, general and systemic examinations were within normal limit. Mental status examination revealed multiple abnormal un-voluntary purposeless movements such as eye rolling, clenching of teeth, moving jaw sideways, jaw opening, shoulder shrugging, jerky movement of upper limbs, and jerky movements of the lower limb. Complex motor tics were seen in hand movements as if making 8, movement of the head in + fashion, and circular movements of the foot. Obsessions of dirt and contamination and impulse of putting a finger in an electric socket and the hinge of the door was present. The compulsion of washing hands was present. Sensorium was clear with intact cognition, insight, and judgment. Bio drives were stable. Relevant investigation including liver function, thyroid function, slit lamp examination, antistreptolysin O titer, Video electroencephalography, and magnetic resonance imaging brain was normal.

He was diagnosed by combined vocal and multiple motor tic disorder (Gilles de la Tourette's syndrome) (F95.2) and OCD (F42). He was managed with tablet haloperidol 1.25 mg HS, tablet fluvoxamine 200 mg in divided doses, relaxation therapy, and habit reversal therapy. He developed akathisia following which haloperidol was withdrawn. Tablet clonazepam 0.5 mg HS was added and built up to 1 TDS. His restlessness subsided. His jaw movements, circular movements of lower limbs decreased. He could prioritize his movement. Frequency and intensity were lesser. Obsessive and compulsive symptoms reduced significantly.

DISCUSSION

Individual had GTS with associated OCD. Common theme generally seen associated with tics are those of orderliness, symmetry, counting, repetitive checking, aggression, sexual, religious, etc. Our patient had primarily those of contamination and impulses.[7] Pathophysiology for both tic and obsessive disorder is thought to be in the basal ganglion. Both OCD and chronic tic disorder are thought as alternate phenotypes of putative Tourette's syndrome gene. Males have been found more prone to tics; whereas females have been found more prone to OCD.[8] Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections are poststreptococcal tic disorders. They are generally prepubertal and abrupt in onset and have a temporal association between exacerbations and Group A beta-hemolytic streptococcal infection. In this case, there have been no definitive indication of such infection and the onset of illness was of an insidious nature.[9] There was a family history of an obsessive trait in this case. Tourette's syndrome is known to have an autosomal dominant pattern of inheritance with incomplete penetrance.[10] The aim of management is primarily symptom reduction, management of comorbidities, and management of precipitants. Pharmacological management primarily includes antipsychotics (haloperidol, risperidone, aripiprazole, quetiapine, pimozide, etc.), alpha agonists (clonidine, guanfacin), and benzodiazepine (clonazepam, etc.). SSRIs are used when comorbid OCD is present. Other medications which have been used are oxcarbazepine, atomoxetine, tetrahydro carbinol, and botulinum toxin. Deep brain stimulation has also been tried. Behavioral management primarily includes habit reversal, desensitization techniques, and relaxation exercises.[1112] In this patient also a multimodal approach involving behavioral measure and medication was found more useful. Tourette's disorder has a fluctuating course (waxes and wanes). It may decrease, persist, or increase and old symptoms may be replaced by new ones.[13] Our patient had a significant reduction in tic frequency and severity and that of associated OCD.

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