History

Fact Explanation
Repetitive Throat clearing and shouting Most commonly seen tics are vocal tic.[1]. Phonic (or vocal) tics can consist of any noise produced by movement of air through the nose, mouth or pharynx. Tongue clicking is, therefore, not classified as a phonic, but a motor tic. The term ‘phonic’ should be preferred over ‘vocal’, since not all sounds (For an example, sniffing) are produced by the vocal cords. Less than 5% of patients with tics have phonic tics alone without associated motor ones, but motor tics without phonic tics are very common.[2].
Premonitory urges Many adult patients (up to 90%), are aware of premonitory sensations preceding the tics, with a mean age of starting to become aware of 10 years. More automatic movements such as eye blinking are less often preceded by sensory urges. These sensations are experienced as unpleasant somatosensory sensations. It can be within the muscles of the upcoming tic or somewhere else in the body or the head (tiredness, itch, pressure, stabbing pain, abdominal discomfort, heat or cold) . Sometimes difficult to articulate.They are often relieved by execution of the tic. Younger children are much less aware of premonitory urges. 37% of children between 8 and 19 years are able to report on premonitory urges.And 64% of these children were able to suppress their tics. However tic awareness does not seem to be a prerequisite for the ability to suppress tics, and awareness seems to increase with age. It is closely associated with cognitive development. Premonitory urges can be bound to small localised areas, with ‘hot spots’ in the shoulder girdle, hands, feet and front of the thighs. They can also be more generalised, and described as a sense of ‘inner tension’ .[2].
Repetitive thoughts A type of cognitive tic. These tics have been described in adolescents and adults with TS (Tourette Syndrome). Cognitive tics are described as repetitive thoughts that are not anxiety-driven. But it occurs as a response to the excessive urge to give in or act upon provocative auditory, visual, tactile or inner stimuli. Although exact frequencies are not known, cognitive tics encompass, echophenomena in thought, mental play, aimless counting and repetitive thoughts with sexual or aggressive content that produce no fear.[2].
Self-injurious behaviour A motor tic. Trying to wound oneself. In general complex motor tics are aimless or in response to an excessive premonitory urge. However, when the tic sequences are complex and elaborate it can be difficult to distinguish them from compulsions as seen in ‘pure’ OCD (obsessive compulsive Disorder). The latter being more cognitively driven, goal-directed and aimed at reduction of anxiety.[2].
Echolalia Repeating others or repeating words.[1],[2]. A complex phonic tic.Complex phonic tics occur when sounds are elaborate or have a semantic content, including for instance words or phrases.[2].
Palilalia Repeating oneself. A complex phonic tic.[2].
Obsessive compulsive (OC) symptoms It is generally agreed that about 50 percent of patients with TS have substantial obsessive-compulsive (OC) symptoms. OC behaviors are also commonplace in children with TS. Typically, these symptoms may include a need for things to look or feel 'just right', as well as preoccupation with symmetry, order, counting, and ritualistic repetition.[3].
Attention deficit hyperactivity disorder (ADHD) ADHD is frequently diagnosed in children with TS. The prevalence as high as 70 percent. This comorbidity is associated with disruptive behaviors, such as aggression, inappropriate expression of anger, low frustration tolerance, and noncompliance. Disruptive behavior in TS patients may occur with a wide frequency range of 26 to 75 percent.They often add considerable burdens to affected patients, including academic problems, peer rejection, and family conflict.[3].
Poor attention and impaired school works Children with TS tend to have difficulties with attention, perseverance, and the ability to keep themselves focused and their work organized. Many have poor penmanship.Schoolwork may also be impaired by a variety of compulsions, such as the need to scratch out words or return to the beginning of a sentence. So the child with TS requires a careful assessment of cognitive functioning and school achievement.[3]. Frequent phonic tics can impair fluency of speech and thus conversations. Moreover, children can expend mental energy in the classroom to suppress their tics, thus reducing their attention to schoolwork and interfering with their academic performance.[4].
Sleep disturbances Sleep studies have repeatedly described insomnia and inefficient sleep, parasomnias (sleep walking, sleep terrors), and agitated sleep in TS.Tics may be seen during sleep. Studies on the impact of sleep problems in children are rare. However, a recent study suggest that sleep deprivation can have a profound impact on children’s behaviour and academic achievement. It suggests that improving sleep quality in TS may improve symptom.[1]
Restless legs Restless legs syndrome (RLS) is an urge to move a limb, usually one or both legs, associated with focal dysesthesia, which is increased by rest, reduced by movement, mostly in the evening. There is an increased RLS symptoms, seen in children with TS (10%).There may be parallels between premonitory urges, relieved by tics and the dysesthesia/urge to move relieved by leg movements in RLS.[1].
Pain Pain in TS may arise from the actual performance of frequent or intense tics causing discomfort by sudden or repeated extreme exertion (e.g. with head or neck). This kind of pain is usually musculoskeletal, although rare examples of neuropathic pain may occur.Additionally, some patients obtain relief from tics while experiencing pain, to such an extent that they will deliberately provoke pain to obtain benefit. A smaller number of patients complain of pain associated with the irresistible urge to tic or with aggravating premonitory urges during voluntary efforts to suppress their tics.[4].
Socially Isolation Persistent complex motor tics and loud phonic tics can cause social problems. Tics may cause isolation, bullying, or social stigmatization.loud phonic tics may result in the child being put out of the classroom.In higher school classes, bullying and social stigmatization due to tics becomes more common. After proper psychoeducation, many children and adolescents will accept their tic symptoms and await the natural remission.[4].
Social and emotional problems Tics can cause social and emotional problems for the patient. Sustained social problems, consequent to negative reactions of the social environment, some patients develop depressive and anxious symptom, low self-esteem, and/or social withdrawal. [4].
References
  1. SROUR M, LESPéRANCE P, RICHER F, CHOUINARD S. Psychopharmacology of Tic Disorders J Can Acad Child Adolesc Psychiatry [online] 2008 Aug, 17(3):150-159 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2527768
  2. CATH DC, HEDDERLY T, LUDOLPH AG, STERN JS, MURPHY T, HARTMANN A, CZERNECKI V, ROBERTSON MM, MARTINO D, MUNCHAU A, RIZZO R, THE ESSTS GUIDELINES GROUP. European clinical guidelines for Tourette Syndrome and other tic disorders. Part I: assessment Eur Child Adolesc Psychiatry [online] 2011 Apr, 20(4):155-171 [viewed 08 August 2014] Available from: doi:10.1007/s00787-011-0164-6
  3. SWAIN JE, LECKMAN JF. Tourette Syndrome and Tic Disorders: Overview and Practical Guide to Diagnosis and Treatment Psychiatry (Edgmont) [online] , 2(7):26-36 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3000195
  4. ROESSNER V, PLESSEN KJ, ROTHENBERGER A, LUDOLPH AG, RIZZO R, SKOV L, STRAND G, STERN JS, TERMINE C, HOEKSTRA PJ, THE ESSTS GUIDELINES GROUP. European clinical guidelines for Tourette syndrome and other tic disorders. Part II: pharmacological treatment Eur Child Adolesc Psychiatry [online] 2011 Apr, 20(4):173-196 [viewed 08 August 2014] Available from: doi:10.1007/s00787-011-0163-7

Examination

Fact Explanation
Repetitive eye blinking It is a common type of motor tic.Tics are repetitive, sudden, rapid, nonrhythmic, stereotyped movements which usually occur in response to a sensation or an urge and often occur in bouts. Motor tics usually manifest first in the head and face and then migrate to more distal regions.[1].Motor tics arise in the voluntary musculature and involve discrete muscles or muscle groups. Tics can be seen as fragments of normal motor movements that appear out of context . The most frequent tic is eye blinking.Examples of Other simple motor tics include,nose twitching, tongue protrusion, head jerks and shoulder shrugs,[2]. Tic disorders are classified as TS [Tourette Syndrome], chronic motor or vocal tic disorder, or transient tic disorder.[1]. In TS, motor tics often begin between the age of 3 and 8, several years before the appearance of vocal tics.[3].
Copropraxia So called repetitive obscene movements. It is a complex motor tic. Complex motor tics often have a repetitive and/or compulsive nature. Other examples include repetitive touching of objects or people, making elaborate sequences of movements.[2].
Echopraxia It Means mimicking others or imitating actions.[1],[2]. It is also a complex motor tic.[2].
Coprolalia It is an involuntary verbalization of obscene or scatological words. Appears only within a minority of subjects and it is often a temporary manifestation.[1]. It is a Complex phonic tic.[2].
References
  1. SROUR M, LESPéRANCE P, RICHER F, CHOUINARD S. Psychopharmacology of Tic Disorders J Can Acad Child Adolesc Psychiatry [online] 2008 Aug, 17(3):150-159 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2527768
  2. CATH DC, HEDDERLY T, LUDOLPH AG, STERN JS, MURPHY T, HARTMANN A, CZERNECKI V, ROBERTSON MM, MARTINO D, MUNCHAU A, RIZZO R, THE ESSTS GUIDELINES GROUP. European clinical guidelines for Tourette Syndrome and other tic disorders. Part I: assessment Eur Child Adolesc Psychiatry [online] 2011 Apr, 20(4):155-171 [viewed 08 August 2014] Available from: doi:10.1007/s00787-011-0164-6
  3. SWAIN JE, LECKMAN JF. Tourette Syndrome and Tic Disorders: Overview and Practical Guide to Diagnosis and Treatment Psychiatry (Edgmont) [online] , 2(7):26-36 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3000195

Differential Diagnoses

Fact Explanation
Stereotypies Motor stereotypies are likely to begin in the early stages of life. A movement becomes a sterotypy when, it is a repetitive, non functional motor disorder which interferes with normal activities or results in injury.In clinical practice the definition is broader as usually children report enjoyment or are unaware of their actions.Childhood Motor Stereotypies often consist of hand flapping or twisting, body rocking, head banging, face or mouth stretching sometimes appearing as a marked grimace. It is imperative to establish the presence of any co-existing developmental disorder Stereotypies can present in those with normal development and without neurological disorder. Motor stereotypies are commonly seen in children with autism spectrum disorder but can also be seen in those with sensory impairment, social isolation and or learning disability.[1],[2].
Compulsive behaviors Compulsions are movements or ritualistic behaviours used to reduce stress. Examples include hand washing and fear of contamination, counting behaviours possibly associated with switches and arranging objects in a specific, perhaps symmetrical fashion. The movements are not stereotyped and are purposeful.The actions are voluntary but there is a need to perform them, patients describe a fear of impending doom if they are not carried out.Tic and stereotypies may also be present due to the overlapping nature of these conditions.[1].[2].
Paroxysmal dyskinisias The paroxysmal dyskinesias are part of the group termed ‘hyperkinetic movement disorders’. A term which refers to abnormal, repetitive involuntary movements. Between ‘attacks’ most people are well. Bouts of abnormal movements are not usually accompanied by a loss of consciousness.Paroxysmal kinesiogenic dyskinesia (PKD) movements can occur up to a hundred times per day. There is often a preceding sensation in the affected limb and resulting movements are short, seconds to minutes in duration. Usually a particular side of the body or single limb will be affected and movements can be dystonic.In Paroxysmal Non Kinesiogenic Dyskinesia, movements can occur at any time between early childhood and early adulthood. Attacks of movement disorder occur less frequently than in PKD. Often occurring on two or three occasions per year. Certain triggers may be identifiable such as caffeine, tiredness, alcohol or stress. Attacks last from a few seconds to a few hours and often begin in one limb them spread throughout the body to include the face. The affected individual may not be able to communicate during the attack but remains conscious and breathing rate is normal.The pathophysiology of these paroxysmal dyskinesias is attributed to basal ganglia dysfunction[3],[2].
Akathisia Akathisia makes the child feel as if they need to walk or move. There is a feeling of discomfort and movement eases this discomfort.Therefore the movement associated with Akathisia is voluntary and includes pacing up and down, rubbing the legs, face or scalp with the hands.Akathisia can occur in children as a result of Iron deficiency, thyroid disorders and as a side effect of drugs for an example neuroleptic medications such as Haloperidol or Pimozide.[3],[2].
Myoclonus Myoclonus is a movement disorder, which presents itself with sudden, brief, shock-like jerks. Most myoclonic jerks are due to a brief burst of muscular activity, resulting in positive myoclonus. When jerks result from brief cessation of ongoing muscular activity, they are called negative myoclonus (NM). Positive myoclonus is generally more common, while NM frequently occurs in hospital settings, as a result of toxic–metabolic causes. A combination of both forms may be present in the same disease, as in posthypoxic myoclonus or progressive myoclonic epilepsies (PMEs).[3],[4].
Sydenham’s chorea (SC) The clinical features of SC include both neurological abnormalities and psychiatric disorders. The former comprise involuntary choreatic movements, voluntary movement incoordination, muscular weakness and hypotonia. Psychiatric disorders include emotional lability, hyperactivity distractibility, obsessions and compulsion. Choreatic movements are involuntary, irregular, purposeless, non-rhythmic, abrupt, rapid and unsustained. Movements disappear with sleep and rest. Voluntary movements make the chorea worse and are themselves incoordinated making activities such as writing, dressing and eating difficult. The hypotonia and weakness have a range of severity from mild to severe.[3],[5].
References
  1. LUDOLPH AG, ROESSNER V, MüNCHAU A, MüLLER-VAHL K. Tourette Syndrome and Other Tic Disorders in Childhood, Adolescence and Adulthood Dtsch Arztebl Int [online] 2012 Nov, 109(48):821-288 [viewed 08 August 2014] Available from: doi:10.3238/arztebl.2012.0821
  2. MILLS S, HEDDERLY T. A Guide to Childhood Motor Stereotypies, Tic Disorders and the Tourette Spectrum for the Primary Care Practitioner Ulster Med J [online] 2014 Jan, 83(1):22-30 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3992090
  3. SWAIN JE, LECKMAN JF. Tourette Syndrome and Tic Disorders: Overview and Practical Guide to Diagnosis and Treatment Psychiatry (Edgmont) [online] , 2(7):26-36 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3000195
  4. KOJOVIC M, CORDIVARI C, BHATIA K. Myoclonic disorders: a practical approach for diagnosis and treatment Ther Adv Neurol Disord [online] 2011 Jan, 4(1):47-62 [viewed 08 August 2014] Available from: doi:10.1177/1756285610395653
  5. WALKER KG, WILMSHURST JM. An update on the treatment of Sydenham's chorea: the evidence for established and evolving interventions Ther Adv Neurol Disord [online] 2010 Sep, 3(5):301-309 [viewed 08 August 2014] Available from: doi:10.1177/1756285610382063

Investigations - for Diagnosis

Fact Explanation
EEG Usually Tic disorders are clinically diagnosed on the basis of a detailed history and a neurological and psychiatric examination. Further diagnostic evaluation is only rarely needed.[1]. However in case of atypical features such as apparent adult onset or severe deterioration or progression in symptoms should always lead to detailed consideration and investigation to include EEG and neuroimaging.Sometimes EEG in a typical tic disorder is mistaken for epilepsy or myoclonus. So it is worthwhile seeking expert opinion if doubt exists about the extent of investigation to pursue.[2].
MRI scanning Additional investigation with the aid of MRI scanning is rarely indicated except in those cases where the presentation is not typical in terms of either the semiology of the movement disorder or the presence of features suggestive of differential diagnoses.MRI studies with different techniques and electrophysiological investigations on neuronal inhibition have identified alterations in brain areas of the cortico-striato-thalamo-cortical (CSTC) circuits.[2].
Videotape recording A considerable difficulty in assessing and quantifying tics is caused by the spontaneous variations of tics in an individual over time, large variability in impact of a given level of physical tic severity on an individual or their family and , the tendency of patients to suppress their tics, especially when in the office with the clinician.Therefore, it is advisable when assessing tics, to use multi informant data. One of the way is collecting video data. Videotape tic monitoring might enhance capturing the whole tic repertoire of the patient.[2].
PET studies PET raclopride studies using amphetamine challenge to study D2 receptor availability in striatal circuits in TS patients have revealed increased phasic dopamine release in ventral striatal areas in TS patients after amphetamine challenge.[2]
References
  1. LUDOLPH AG, ROESSNER V, MüNCHAU A, MüLLER-VAHL K. Tourette Syndrome and Other Tic Disorders in Childhood, Adolescence and Adulthood Dtsch Arztebl Int [online] 2012 Nov, 109(48):821-288 [viewed 08 August 2014] Available from: doi:10.3238/arztebl.2012.0821
  2. CATH DC, HEDDERLY T, LUDOLPH AG, STERN JS, MURPHY T, HARTMANN A, CZERNECKI V, ROBERTSON MM, MARTINO D, MUNCHAU A, RIZZO R, THE ESSTS GUIDELINES GROUP. European clinical guidelines for Tourette Syndrome and other tic disorders. Part I: assessment Eur Child Adolesc Psychiatry [online] 2011 Apr, 20(4):155-171 [viewed 08 August 2014] Available from: doi:10.1007/s00787-011-0164-6

Investigations - Screening/Staging

Fact Explanation
Genetic testing The physical examination includes careful examination for dysmorphic features to identify any indication of genetic syndromes. Unusual features may prompt specific genetic testing by consulting a clinical geneticist. Further, in the presence of additional learning difficulties or autism spectrum diagnosis, it might be advisable to consult a clinical geneticist as well.In some cases this high resolution array might reveal a rare genetic aetiology of these heterogeneous disorders.[1].
References
  1. CATH DC, HEDDERLY T, LUDOLPH AG, STERN JS, MURPHY T, HARTMANN A, CZERNECKI V, ROBERTSON MM, MARTINO D, MUNCHAU A, RIZZO R, THE ESSTS GUIDELINES GROUP. European clinical guidelines for Tourette Syndrome and other tic disorders. Part I: assessment Eur Child Adolesc Psychiatry [online] 2011 Apr, 20(4):155-171 [viewed 08 August 2014] Available from: doi:10.1007/s00787-011-0164-6

Management - General Measures

Fact Explanation
Reduction of stress and lifestyle modification Acute and chronic stress can exacerbate tics. So an attempt to reduce the stress of patients is reasonable. Psychotherapy sessions may be useful to improve self-esteem, social coping, family strain, and school adjustment. But it is unclear if they directly affect tic severity. Regular appointments with the same clinical team, who can help the patient deal with the changing manifestations of the disorder through the years, are highly recommended. Regular contact via telephone or e mail may also be helpful. Participation in regular school and extracurricular activities is encouraged.The impact of physical exercise on tic symptoms has not been systematically studied. But though a regular program of exercise can be beneficial by reducing stress, increasing the child's sense of mastery, and contributing to overall well being.[1].
Reduce caffeine Caffeine should be minimized as it may exacerbate tics in some children.[1].
Thorough patient education Psychoeducation should always be the first step of treatment.Many patients and their families, the diagnosis itself brings considerable relief. If the patient is a child, it is important that his or her teachers and other significant adults should also understand the nature of the condition. Thorough patient education should include not only information about the cause and future course of the disease, but also counseling on social issues such as the various types of compensatory aid that are available, how to request certification as a disabled or severely disabled person, the issue of driving, and the choice of an occupation. It is not at all rare for patients to feel stress as a result of their tic disorder for no other reason than the way others respond to it.[2].
Behavioral therapy Habit reversal training (HRT) was recently introduced as an alternative to drug therapy for tics. In HRT, the patient prevents the occurrence of a tic by performing a previously learned alternative behavior instead.This method lessens the frequency of tics by about 30% .Comparable results can be obtained with exposure and response prevention (ERP), a strategy for interrupting the automatism described by many patients in which a premonitory urge is necessarily followed by a tic. A European expert commission recently recommended that behavior therapy (if available) should always be tried before drug treatment. Behavior therapy often fails because of inadequate motivation, particularly in children.[2].
Dopamine Modulating Agents Traditionally dopamine-blockers have been the first line treatment for tics and have the most compelling evidence for effectiveness in double-blind controlled studies.The three most studied agents are haloperidol, pimozide and risperidone. Starting doses of haloperidol and pimozide are 0.25–0.5 mg/day and 0.5–1 mg/day respectively, with usual maintenance doses ranging between 1–4 mg/day and 2–8 mg/day. Dopamine modulators have important but variable side effects such as weight gain, sedation, anxiety, electrographic changes (tachycardia and QTc prolongation) and extrapyramidal symptoms.Because of their presumed lower long-term side effects profile, atypical neuroleptics such as risperidone (0.5–4 mg) or olanzapine (2.5–10 mg) are preferred.Tetrabenazine (given as 12.5–25 mg TID) is a monoamine depletion which operates mainly by inhibiting dopamine liberation. This drug may be effective for the treatment of tics and unlike neuroleptics, does not pose any major risk of tardive dyskinesia at lower doses.[1].
Alpha-2-Adrenergic Agonists Because of contradictory results, the role of alpha-2-adrenergic agents (clonidine and guanfacine) in the treatment of tics is debatable. However, in practice, because of a better side effect profile and no long term potential risk, they are often a first line treatment option especially in patients with comorbid symptoms of ADHD.[1].Clonidine is used preferentially in English-speaking countries, even though its tic-suppressing effect is rather weak in comparison to most of the neuroleptics.[2].
Botulinum toxin Botulinum toxin may be a good treatment for highly-localized motor tics. But some patients treated with botulinum toxin for a single focal tic notice spread of the tic to an adjacent non-injected muscle.[1].
References
  1. SROUR M, LESPéRANCE P, RICHER F, CHOUINARD S. Psychopharmacology of Tic Disorders J Can Acad Child Adolesc Psychiatry [online] 2008 Aug, 17(3):150-159 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2527768
  2. LUDOLPH AG, ROESSNER V, MüNCHAU A, MüLLER-VAHL K. Tourette Syndrome and Other Tic Disorders in Childhood, Adolescence and Adulthood Dtsch Arztebl Int [online] 2012 Nov, 109(48):821-288 [viewed 08 August 2014] Available from: doi:10.3238/arztebl.2012.0821

Management - Specific Treatments

Fact Explanation
Neurosurgical Treatment Multiple neurosurgical procedures including frontal lobe bimedial frontal leucotomy and pre-frontal lobotomy, limbic system anterior cingulotomy and limbic leucotomy, have been tried in patients with severe tics with variable results. None of these procedures have been studied in a large control case studies. More recently, because of a lower side effect profile and potential access to deeper regions, deep brain stimulation has been advocated as an alternative for cases with severe uncontrolled tics.[1]. Very severely affected adult patients with medically intractable Tourette syndrome may benefit from deep brain stimulation.[2].
References
  1. SROUR M, LESPéRANCE P, RICHER F, CHOUINARD S. Psychopharmacology of Tic Disorders J Can Acad Child Adolesc Psychiatry [online] 2008 Aug, 17(3):150-159 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2527768
  2. LUDOLPH AG, ROESSNER V, MüNCHAU A, MüLLER-VAHL K. Tourette Syndrome and Other Tic Disorders in Childhood, Adolescence and Adulthood Dtsch Arztebl Int [online] 2012 Nov, 109(48):821-288 [viewed 08 August 2014] Available from: doi:10.3238/arztebl.2012.0821