Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes".
Tourette syndrome (TS) is an inherited neurological condition of multiple motor and at least one vocal tic. Although Tourette syndrome is the most common cause of tic disorders, [1] other sporadic, genetic, and neurodegenerative disorders may also exhibit tics. [2] [3]
Conditions that may manifest tics or stereotyped movements include developmental disorders; autism spectrum disorders [4] and stereotypic movement disorder; [1] [5] Sydenham's chorea; idiopathic dystonia; and genetic conditions such as Huntington's disease, neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, Duchenne muscular dystrophy, Wilson's disease, and tuberous sclerosis. Other possibilities include chromosomal disorders such as Down syndrome, Klinefelter syndrome, XYY syndrome and fragile X syndrome. Acquired causes of tics include drug-induced tics, head trauma, encephalitis, stroke, and carbon monoxide poisoning. [2] [6] The symptoms of Lesch–Nyhan syndrome may also be confused with Tourette syndrome. [7]
Tourette syndrome or Tourette's syndrome is a common neurodevelopmental disorder that begins in childhood or adolescence. It is characterized by multiple movement (motor) tics and at least one vocal (phonic) tic. Common tics are blinking, coughing, throat clearing, sniffing, and facial movements. These are typically preceded by an unwanted urge or sensation in the affected muscles, can sometimes be suppressed temporarily, and characteristically change in location, strength, and frequency. Tourette's is at the more severe end of a spectrum of tic disorders. The tics often go unnoticed by casual observers.
Coprolalia is involuntary swearing or the involuntary utterance of obscene words or socially inappropriate and derogatory remarks. Coprolalia comes from the Greek κόπρος (kópros), meaning "dung, feces", and λαλιά (laliā́) "speech", from λαλεῖν (laleîn) "to talk".
A tic is a sudden, repetitive, nonrhythmic motor movement or vocalization involving discrete muscle groups. Tics can be invisible to the observer, such as abdominal tensing or toe crunching. Common motor and phonic tics are, respectively, eye blinking and throat clearing.
Chorea-acanthocytosis, is a rare hereditary disease caused by a mutation in a gene that directs structural proteins in red blood cells. It belongs to a group of four diseases characterized under the name neuroacanthocytosis. When a patient's blood is viewed under a microscope, some of the red blood cells appear thorny. These thorny cells are called acanthocytes.
A stereotypy is a repetitive or ritualistic movement, posture, or utterance. Stereotypies may be simple movements such as body rocking, or complex, such as self-caressing, crossing and uncrossing of legs, and marching in place. They are found especially in people with autism spectrum disorders, but can also be found in intellectual disabilities, tardive dyskinesia and stereotypic movement disorder, yet may also be encountered in neurotypical individuals as well. Studies have shown stereotypies associated with some types of schizophrenia. Frontotemporal dementia is also a common neurological cause of repetitive behaviors and stereotypies. Several causes have been hypothesized for stereotypy, and several treatment options are available.
Hyperkinesia refers to an increase in muscular activity that can result in excessive abnormal movements, excessive normal movements or a combination of both. Hyperkinesia is a state of excessive restlessness which is featured in a large variety of disorders that affect the ability to control motor movement, such as Huntington's disease. It is the opposite of hypokinesia, which refers to decreased bodily movement, as commonly manifested in Parkinson's disease.
Tetrabenazine is a drug for the symptomatic treatment of hyperkinetic movement disorders. It is sold under the brand names Nitoman and Xenazine among others. On August 15, 2008, the U.S. Food and Drug Administration approved the use of tetrabenazine to treat chorea associated with Huntington's disease. Although other drugs had been used "off label," tetrabenazine was the first approved treatment for Huntington's disease in the U.S. The compound has been known since the 1950s.
Tic disorders are defined in the Diagnostic and Statistical Manual of Mental Disorders (DSM) based on type and duration of tics. Tic disorders are defined similarly by the World Health Organization.
Pantothenate kinase-associated neurodegeneration (PKAN), formerly called Hallervorden–Spatz syndrome, is a genetic degenerative disease of the brain that can lead to parkinsonism, dystonia, dementia, and ultimately death. Neurodegeneration in PKAN is accompanied by an excess of iron that progressively builds up in the brain.
Neuroacanthocytosis is a label applied to several genetic neurological conditions in which the blood contains misshapen, spiculated red blood cells called acanthocytes.
Stereotypic movement disorder (SMD) is a motor disorder with onset in childhood involving restrictive and/or repetitive, nonfunctional motor behavior, that markedly interferes with normal activities or results in bodily injury. To be classified as SMD, the behavior in question must not be due to the direct effects of a substance, Autism, or another medical condition. The cause of this disorder is not known.
Tourette syndrome is an inherited neurodevelopmental disorder that begins in childhood or adolescence, characterized by the presence of motor and phonic tics. The management of Tourette syndrome has the goal of managing symptoms to achieve optimum functioning, rather than eliminating symptoms; not all persons with Tourette's require treatment, and there is no cure or universally effective medication. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.
Arthur K. Shapiro, M.D., was a psychiatrist and expert on Tourette syndrome. His "contributions to the understanding of Tourette syndrome completely changed the prevailing view of this disorder"; he has been described as "the father of modern tic disorder research" and is "revered by his colleagues as the first dean of modern Tourette syndrome researchers".
Sensory phenomena are general feelings, urges or bodily sensations. They are present in many conditions including autism spectrum disorders, epilepsy, neuropathy, obsessive–compulsive disorder, pain conditions, tardive syndromes, and tic disorders.
Klazomania refers to compulsive shouting; it has features resembling the complex tics such as echolalia, palilalia and coprolalia seen in tic disorders, but has been seen in people with encephalitis lethargica, alcohol use disorder, and carbon monoxide poisoning. It was first reported by L. Benedek in 1925 in a patient with postencephalitic parkinsonism. Little is known about the condition, and few cases have been reported.
Tourette syndrome is an inherited neurological disorder that begins in childhood or adolescence, characterized by the presence of multiple physical (motor) tics and at least one vocal (phonic) tic.
Chorea gravidarum is a rare type of chorea which presents with involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces. It is a complication of pregnancy which can be associated with eclampsia and its effects upon the basal ganglia. It is not a causal or pathologically distinct entity but a generic term for chorea of any cause starting during pregnancy. It is associated with history of Sydenham's chorea. It mostly occurs in young patients; the average age is 22 years.
Basal ganglia disease is a group of physical problems that occur when the group of nuclei in the brain known as the basal ganglia fail to properly suppress unwanted movements or to properly prime upper motor neuron circuits to initiate motor function. Research indicates that increased output of the basal ganglia inhibits thalamocortical projection neurons. Proper activation or deactivation of these neurons is an integral component for proper movement. If something causes too much basal ganglia output, then the ventral anterior (VA) and ventral lateral (VL) thalamocortical projection neurons become too inhibited, and one cannot initiate voluntary movement. These disorders are known as hypokinetic disorders. However, a disorder leading to abnormally low output of the basal ganglia leads to reduced inhibition, and thus excitation, of the thalamocortical projection neurons which synapse onto the cortex. This situation leads to an inability to suppress unwanted movements. These disorders are known as hyperkinetic disorders.
A premonitory urge is a sensory phenomenon associated with Tourette syndrome and other tic disorders. Premonitory urges are "uncomfortable feelings or sensations preceding tics that usually are relieved by [a particular] movement".