What is Tourette Syndrome?

The pathogenesis is unknown, and genetic factors may be the cause, which is believed to be related to the following factors:
tourette Tourette Syndrome

Sun Ying	(Attending physician)	Department of Neurology, Xuanwu Hospital, Capital Medical University
Wang Yuping	(Chief physician)	Department of Neurology, Xuanwu Hospital, Capital Medical University

Tourette syndrome (TS) is also known as Gilles de la tourette syndrome, multiple tics-coprolalia syndrome, and was described in detail by French doctor Georges Gilles de la Tourette in 1885. The disease can occur in various regions and all races around the world. Epidemiological investigations have found certain differences between regions and races. The prevalence of blacks is low and the prevalence of Jews is high. Eastern Europeans have the disease. The annual incidence is 0.5 to 1 person per 100,000 population.

Western Medicine Name

Tourette syndrome

English name

Tourette syndrome

Affiliated Department

Internal Medicine-Neurology

Main cause

genetic factors

Contagious

Non-contagious

The cause and mechanism of tourette syndrome

The pathogenesis is unknown, and genetic factors may be the cause, which is believed to be related to the following factors:

1. In the striatum, dopamine (DA) hyperconduction or DA receptor (DR) hypersensitivity can effectively control tics with DR antagonists. Conversely, amphetamine can significantly improve tics.

2. The content of DA metabolite high vanillic acid (HVA) was significantly reduced and the degree of reduction was related to the severity of symptoms. Plasma tryptophan levels and the 5-HT metabolite 5-hydroxytryptophan (5-HTP) content in the cerebrospinal fluid were significantly reduced. The degree of tics was related to changes in 5-HT metabolite 5-hydroxyindoleacetic acid (5-HIAA) concentration. This indicates that 5-HT metabolism is abnormal in TS. It has been reported that the cAMP concentration in the cerebral cortex was reduced during autopsy of the patient, suggesting that neurological dysfunction may occur secondary to neurotransmission.

3 Over-nutrition in the early stages of brain development. Due to the over-nutrition of excitatory amino acids and sex hormones, excitatory neurons continue to depolarize. Excitatory amino acids and sex hormones may cause intracellular calcium overload, and some parts of the basal ganglia and limbic system The abnormal increase of neurons and excessive derivation of neuronal synapses produce a series of clinical symptoms. Most of TS occurs before puberty, and there are more men than women, suggesting that it is mainly related to the role of androgens.

Tourette syndrome clinical manifestations

Onset occurs between the ages of 2 and 15 years, with an average of 7 years, with more men than women.

tourette syndrome motor tics

It is the main clinical symptom in the early stage of this disease. It usually starts on the face and manifests as rapid and repeated irregular twitching of eyes and facial muscles, such as blinking, nose twitching, and "ghosting". Later, there are sports twitches in other parts, such as throwing The head, nod, and neck quickly and shortly stretch and shrug. Symptoms can gradually develop to the upper limbs, torso, or lower limbs. Short, violent involuntary movements of the limbs or torso such as upper limb throwing, kicking, kneeling, Bend your knees, press your feet or bend your torso, twist them, etc. Some patients even hit people (self or others) for no purpose, touching themselves or other people's bodies, and strangely imitating the actions of others. The frequency of tics varies from dense to dense. Frequent people can reach dozens or even hundreds of times a day.

tourette syndrome vocal tics

The incidence rate is 79% to 98.5%. It usually appears in the course of 1 to 2 years. It can be a simple vocal tics or a weird sound, which is expressed as a dry cough like throat, such as "hehe", "ahh ", Or making unbearable sounds such as dog barks, grunts, and" tongues ". Nasal convulsions are frequent nasal sprays, asthma, choking sounds, etc. When involuntary twitches appear cursing, they are considered to be ill-defined. Most patients have good self-awareness, but it is difficult to control themselves. Vocal tics often occur in conversations with people, especially in pauses in speech. Patients can sometimes suppress twitches for a short period of time, while agitation and tension make them worse and disappear during sleep. When patients are affected by emotions or related to personality and sex, tics occur particularly frequently. In order to cover the symptoms of vocal tics, 1/3 of the patients often change the language of their expression in some special ways. The result is counterproductive, making the spoken language appear indistinct and uneven in strength. Some patients are afraid that their semantic expression will be unclear and there will be language duplication, resulting in clinically repetitive language. The twitch that appears at the beginning of the disease may be related to uncomfortable thinking feelings, and later progresses to the characteristic of isolated occurrence without any conscious participation.

tourette syndrome behavior disorder

Seen in 30% to 60% of cases, the lighter show restlessness, oversensitivity, irritability or behavioral withdrawal; the severer show compulsive behavior that is difficult to get rid of, often repeating an action irresistibly, such as washing hands, counting Digital and check door locks. Some patients show upset, hyperactivity, emotional instability, and restlessness, known as attention deficit hyperactivity disorder (ADHD). There are also some patients with destructive behaviors, showing sudden impulsive behaviors that cannot be controlled, such as excessive provocations, and even violent and self-injurious behaviors. For example, biting your lips and cheeks, hitting hard objects with your head, etc. About 75% of patients have learning problems, and their learning ability declines. They are particularly prone to perceptual difficulties such as reading, writing, writing, and even fail to complete normal academic studies. Learning problems are related to ADHD.

tourette syndrome examination

CT / MRI of the skull shows that some patients have slight structural abnormalities in the brain tissue, such as asymmetric basal ganglia, larger left caudate nucleus than the right, and slightly enlarged lateral ventricle. MRI sometimes shows bilateral caudate and bean-shaped nuclei. The average volume is smaller than normal. Single-photon emission tomography (SPECT) showed that some patients had reduced cerebral blood flow perfusion in the temporal, frontal, and basal ganglia. Positron emission tomography (PET) also found that the brain's glucose utilization was reduced in the corresponding areas. The EEG examination can show high amplitude slow wave, spike wave, slow spike comprehensive wave, etc. The abnormal rate of dynamic EEG can reach 50%, but it is not specific for diagnosis.

Diagnosis and differential diagnosis of tourette syndrome

Diagnosis of tourette syndrome

The diagnosis of this disease can refer to the diagnostic standards of the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) in the United States: (1) Sometimes there are multiple motor tics and one or more vocal tics during the disease, but not necessarily at the same time Occurrence (twitches are sudden, rapid, recurrent, arrhythmic, stereotyped movements or vocalizations); (2) tics occur multiple times a day, with seizures or intermittent occurrences almost daily over a period of more than 1 year. During this period, there have been no seizures for more than 3 months; (3) onset before the age of 18; (4) the disease is not due to drugs (such as stimulants) or common diseases (such as Huntington's disease or viral infection after encephalitis) ) Caused by direct physiological effects.

differential diagnosis of tourette syndrome

1. Habitual spasm: It is more common in boys 5 to 10 years of age. For bad habits or imitating the behavior of others, they generally have no symptoms of sound spasm, no behavior disorder, single and limited performance, and can disappear on their own.

2. Minor chorea: It is also seen in children, but the symptoms are dance-like movements, uncoordinated voluntary movements, decreased muscle tone, and decreased muscle strength. There is generally no vocal tics, and laboratory evidence of rheumatism.

tourette syndrome disease treatment

Drug therapy combined with psychological counseling is an effective measure to treat this disease. The main drugs are haloperidol, sulpiride, thiopiride, risperidone, etc., which should be started from a small dose and gradually increased to an effective dose. After the symptoms are controlled, they should be gradually reduced and maintained for a period of time (3 months or longer). ), Can make many children return to normal. Other medicines include permidazine, clonidine, tetrabenazine, sodium valproate, clonazepam, and tricyclic antidepressants or selective serotonin reuptake inhibitors.

tourette syndrome haloperidol

Start with a small dose of 0.25mg daily, 2 or 3 times a day after a few days, and gradually increase to symptom relief. Most patients can achieve better results at 2mg or slightly higher daily doses, especially for the control of tic symptoms better. The maintenance dose should last for a period of time (usually 1 to 3 months). The dosage of haloperidol should be determined according to the specific situation of the patient. Different individuals respond to it differently. The dosage of the Chinese is significantly lower than the recommended dosage abroad. Large-dose applications are prone to adverse reactions in the extrapyramidal system, such as increased muscle tone, slow movement, and salivation. Antan can be added to counteract its adverse effects.

tourette syndrome clonidine

In addition to having a significant effect on tics, it is also effective on ADHD associated with tics. The starting dose was 0.05 mg before bedtime, and the dose was increased by 0.05 mg every few days until the tics were satisfactorily controlled without significant adverse reactions. Most patients can get to 0.1mg 3 times a day. When clonidine is not effective with a single medication, the addition of fluphenazine or clonazepam may help control symptoms. Common adverse reactions are fatigue, dry mouth, allergies, dizziness, headache, and insomnia. When the effect of the medicine is not ideal, it should be gradually reduced when switching to other medicines, reducing 0.05mg every few days to avoid the potential withdrawal phenomenon of the medicine such as tachycardia and hypertension.

tourette syndrome

It has similar effects to haloperidol, but has fewer adverse reactions. Start with a small dose of 50 to 100 mg / day, orally in 3 divided doses, and gradually increase the dose until the symptoms improve.

4 Sulpiride: 0.1 ~ 0.2g, 3 times a day, effective for tics. In addition, pimozide (piperazine) can also be used, starting at 0.5 mg / day, and gradually increasing the amount to 8-9 mg / day.

tourette syndrome sodium valproate

By inhibiting GABA transaminase and glutamate decarboxylase, increasing GABA content in the brain has a certain therapeutic effect on tics. The general oral dose is 30 to 40 mg / kg daily.

tourette syndrome risperidone

As a first-line drug for treating schizophrenia, recent authors believe that risperidone is expected to replace or partially replace haloperidol and clonidine and become an effective drug for the treatment of TS. The dose starts from 0.25mg / day, and gradually increases, and it is increased every 2 days, each time increasing by 0.25 ~ 0.5mg / day, the therapeutic dose is 1 ~ 3mg / day. 1/3 to 1/2 of the therapeutic dose is used as a maintenance dose.

Authors have reported that other drugs that can improve tics are clonazepam, tetrabenazine, and calcium channel antagonists.

tourette syndrome with compulsive behavior that is difficult to get rid of

On the basis of anti-ticulent drug treatment, morning fluoxetine 20mg can be added. Clomipramine, fluvoxamine, and naloxone are equally effective in treating obsessive-compulsive ideas and behaviors.

Tourette syndrome psychological and behavioral therapy

Applying psychological and behavioral methods such as hypnosis therapy, relaxation training, and biofeedback therapy to treat TS has a certain effect.

tourette syndrome other

Patients with blepharospasm or painful dystonia of the neck can receive a significant improvement in tics with local injection of botulinum toxin type A. ^[1]

tourette syndrome prognosis

Although this disease is a chronic disease, the prognosis is good. Most patients can use drugs to control the onset, and about 3% of patients can resolve on their own. It is very beneficial to the prognosis of TS by cooperating with psychological counseling to reduce the psychological burden and correct bad behaviors while controlling the tic symptoms in time. The impact of family and society plays a very important role in the outcome of the patient's illness. It is also a problem that cannot be ignored to improve the parent's education and reduce various negative pressures that patients may encounter in social interaction. Many clinical review conclusions show that while pharmacological treatment, mobilize the patient's own initiative, so that doctors and patients can communicate and understand each other, and establish a good doctor-patient relationship. Achieved satisfactory results.