Chorea is a pathology of the nervous system. It belongs to the forms of hyperkinesis. The disease is accompanied by problems with the subcortical nodes of the brain. It can be identified by characteristic signs: episodic, aimless, chaotic twitches in the arms and legs, sometimes in the torso. In the later stages, there are mental and intellectual disorders, problems with coordination and self-care. In children, chorea is often mistaken for normal increased activity. In fact, at the first manifestations of pathology, you need to seek qualified help.
The term "chorea"
The term is used in two cases. It can denote independent diseases that belong to this group (for example, chorea minor or Huntington's chorea). It is also used to describe some of the syndromes that occur in various diseases. The word "chorea" itself is taken from the Greek language, where it denotes a certain type of dance. This explains the first symptoms of the disease, whichmanifest in abrupt, uncoordinated, jerky, fast movements of the trunk and limbs. The patient's behavior is really like an involuntary, disorderly dance. Once upon a time, the chorea was called the "dance of St. Vitus." This name is associated with an ancient belief about Saint Vitus, who actively spread Christianity, for which he was tortured by Roman soldiers. After his death, a legend became popular that if someone performs an active dance near his grave on his birthday, he will receive a charge of vivacity and energy for a whole year.
Reasons
The causes of the disease can have various origins, but most often chorea is caused by hereditary factors or genetic disorders.
Less common is chorea caused by infectious diseases such as tuberculous meningitis, viral encephalitis, neurosyphilis, borreliosis, whooping cough. The occurrence of chorea can also be associated with metabolic disorders (if there is Fabry disease, hyperglycemia, hyperthyroidism or Wilson-Konovalov disease), intoxications (poisoning with oral contraceptives, mercury, lithium, digoxin, antipsychotics, levodopa), autoimmune pathologies (such as antiphospholipid syndrome, systemic lupus erythematosus, multiple sclerosis). Among the causes that provoke the onset of the disease, doctors also name damage to the structure of the brain. These include tumor formations, hypoxic encephalopathy, craniocerebral injuries, strokes.
Common signs
Chorea is a disease that is manifested by random, chaotic, short-term bodily movements. In some cases, these movements can be taken as normal, adequate, characteristic of a he althy person, alarmed by something. In others, they resemble an emotional, uncontrollable dance. Jerking twitches can be unilateral or bilateral, but they lack any synchronicity.
Depending on what form of pathology is detected, there are manifestations of chorea. If a person has slightly pronounced choreic hyperkinesis, then in his behavior there is slight motor anxiety, accompanied by motor disinhibition, increased emotionality, some antics, fussy movements, inadequate gestures.
Clearly expressed forms of chorea are manifested in the form of peculiar movements of the "devil on a string". A pronounced form of choreic hyperkinesis distorts walking movements, speech and facial expressions. The patient's gait is not only strange, but also somewhat "clownish". Chorea, the symptoms of which are extremely severe, does not allow any movement at all. Patients with a severe form of pathology are dependent on their environment, as they cannot move around and serve themselves fully in everyday life.
Huntington's Chorea
This pathology in most cases manifests itself at the age of 35 to 45 years. Chorea, the symptoms of which, in addition to typical hyperkinesis, also carry personality disorders and dementia (decreased intelligence), develops gradually, sometimes it is even difficult to trace the moment when it beganshow up. The first violent movements, as a rule, occur on the face. They can be confused with random motor automatisms (this is sticking out the tongue, frowning, licking the lips, opening the mouth). The progression of the disease is manifested in the development of hyperkinesis in the trunk and limbs. The severe form is characterized by deterioration in speech, memory, swallowing processes, self-service level and ends with dementia. The start for Huntington's chorea are mental disorders in the form of hallucinatory-paranoid, affective and behavioral disorders.
Neuroacanthocytosis
Patients with neuroacanthocytosis suffer not only from choreic hyperkinesis, but also from acanthocytosis (the shape of red blood cells changes). A distinctive feature in this pathology is atrophy (weakness in the muscles of the upper and lower extremities). Among other characteristics of the disease, there are: oral hyperkinesis with chewing movements, twitching of the lips, protrusion of the tongue and other grimaces. With neuroacanthocytosis, the patient involuntarily bites his tongue, lips and the inner surface of the cheeks to the blood. Dementia and epileptic seizures are complicating factors in this disease.
Lesch-Nyhan disease
Congenital diseases of the nervous system prevent a person from living a full life. Lesch-Nyhan disease also belongs to such pathologies. The main reason for the development of pathology is a hereditary deficiency of hypoxanthine-guanine phosphoribosyltransferase. This chorea in children is accompanied by severe disturbances in the functioning of the nervoussystems. Also, pathology provokes increased production of uric acid.
From the first months of life, the child has a developmental delay. Rigidity of the limbs manifests itself from the third month. From the second year of life, the baby has facial grimaces, which are accompanied by signs of lesions of the pyramidal tract and mental retardation. A toddler can harm himself physically by biting his lips or fingers.
Benign chorea
Benign chorea in children appears in infancy or early childhood. Pathology refers to hereditary diseases. Generalized hyperkinesis in this disease recedes only when the child is sleeping. This type of pathology differs from Huntington's chorea in a non-progressive course and in the normal development of the intellect. At an older age, with benign chorea, you can achieve a decrease in hyperkinesis if you contact a medical facility in time.
Secondary forms of chorea
The most common secondary forms of pathology are two: minor chorea and chorea pregnant.
The first is also called Sydenham's chorea. Exacerbations of streptococcal infection or rheumatism can provoke its occurrence. The mild form is characterized by exaggerated grimaces, expressive gestures, and disinhibited movements. More complex forms of choreic hyperkinesis are manifested in the fact that it is difficult for the patient to move, speak normally, even breathe. With the disease, there are also “tonic” knee and “freezing” reflexes, emotional and affective disorders, musclehypertension.
In pregnant women, chorea is a disease that comes back from childhood. That is, those women who encountered minor chorea in childhood fall into the risk zone. Pathology can manifest itself during the first pregnancy at 2-5 months. With the subsequent course of the "interesting" situation, chorea may also occur. Treatment in such cases is rarely carried out, since the disease disappears on its own after an abortion or after childbirth.
Chorea rheumatica
The onset of the disease occurs against the background of exacerbated rheumatism or endocarditis, which is accompanied by damage to the heart valves. Many cases of the onset of the disease are not associated with this factor, and the disease manifests itself on its own.
Rheumatic chorea most commonly affects children between 6 and 15 years of age. In girls, the pathology is more common. At the first stage, the disease may not cause concern, since the symptoms are limited to grimaces, and parents take it as a joke. The process continues with small involuntary movements of the limbs, starting from the fingertips and gradually spreading throughout the body. A week later, a period of manifestation begins, when the child's movements become fast and sweeping. This condition prevents the baby from continuing his normal activities, that is, eating, walking, writing.
Rheumatic chorea distorts the child's face with thousands of grimaces. Severe forms of the disease prevent the patient from standing, swallowing, speaking, while the body is constantly moving. If chorea is diagnosed,treatment must be carried out carefully and urgently.
Diagnosis
Suspicions of chorea arise from the strange behavior of a person. During a preliminary examination, the doctor should conduct a conversation with the patient or with his relatives. In particular, it is important to find out if someone else in the family had this disease, how long ago the symptoms of chorea appeared, whether the patient took medications, or had inflammatory ailments. Next, the specialist must assess the involuntary movements. It is also necessary to diagnose and describe diseases that may accompany chorea and cause serious intellectual deviations. Symptoms of such diseases do not always appear. Sometimes, in order to detect such a pathology at an early stage, a whole range of examinations is carried out. A blood test will show the level of copper in the blood and the presence or absence of signs of inflammatory reactions. In addition, computed tomography, magnetic resonance imaging, electroencephalography are performed.
Treatment
Chorea is a pathology that does not allow the patient to lead a normal, full-fledged lifestyle, so its treatment should be timely and comprehensive. The tactics of treatment directly depends on the causes of the disease. For example, in Wilson-Konovalov disease, the patient is prescribed diets with a minimum content of copper and drugs whose action is aimed at reducing its absorption.
In case of Huntington's chorea, antipsychotics and benzodiazepine tranquilizers are prescribed. If choreacaused by chronic insufficiency of the blood supply to the brain, then drugs are needed that reduce the level of blood pressure. With vasculitis, hormonal treatment is performed. If very generalized symptoms appear, treatment should be comprehensive.
It is possible to reduce the intensity of involuntary movements with the help of surgical intervention, when the ventrolateral nuclei of the thalamus are destroyed. With chorea, you need to take drugs that improve the functioning and nutrition of the brain, as well as B vitamins.
