Chorea is a pathology of the nervous system. It refers to 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 twitching in the arms and legs, sometimes in the body. In the later stages, mental and intellectual disorders occur, 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, small chorea or Huntington's chorea). It is also used to describe some syndromes that occur with various types of 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, which are manifested in steep, uncoordinated, jerky, rapid movements of the trunk and limbs. The patient's behavior is really like an involuntary, erratic dance. Once upon a time, the chorea was called the "dance of St. Witt." This name is associated with the ancient belief in St. Witt, who actively spread Christianity, for which he was tortured by Roman soldiers. After his death, the 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.
Causes
The causes of the development of the disease can have a different origin, but most often chorea is caused by hereditary factors or genetic disorders.
Less common is chorea caused by diseases of an infectious nature such as tuberculous meningitis, viral encephalitis, neurosyphilis, borreliosis, whooping cough. The occurrence of chorea can also be associated with metabolic disorders (if Fabry disease, hyperglycemia, hyperthyroidism or Wilson-Konovalov disease occurs), intoxications (poisoning with oral contraceptives, mercury, lithium, digoxin, antipsychotics, levodopa), autoimmune antiphysiologies (such as systemic lupus erythematosus, multiple sclerosis). Among the causes of the onset of the disease, doctors also called damage to the structure of the brain. These include tumor formations, hypoxic encephalopathy, traumatic brain injuries, strokes.
Common symptoms
Chorea is a disease that manifests itself in random, chaotic, short-term bodily movements. In some cases, these movements can be mistaken for normal, adequate, characteristic of a healthy person, something alarmed. In others, they are reminiscent of an emotional, uncontrollable dance. Jerky jerking can be single or double-sided, but they are devoid of at least some kind of synchronism.
Depending on the form of pathology detected, the manifestations of chorea are distinguished. If a person has slightly pronounced choreic hyperkinesis, then in his behavior there is a motor insignificant anxiety, accompanied by motor disinhibition, increased emotionality, certain grimaces, fussy movements, inappropriate gestures.
Clearly expressed forms of chorea manifest themselves in the form of peculiar movements “little devil on a string”. The pronounced form of choreic hyperkinesis distorts movement when walking, speech and facial expressions. The patient’s gait is not only strange, but also to some extent “clownish”. Chorea, the symptoms of which are extremely severe, does not allow any movements to be performed at all. Patients with a severe form of pathology are dependent on their environment, as they cannot move around and serve themselves fully at home.
Huntington Chorea
This pathology in most cases manifests itself between the ages of 35 and 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 began to appear. The first violent movements usually occur on the face. They can be confused with random motor automatisms (this is protruding the tongue, frowning, licking lips, opening the mouth). The progression of the disease is manifested in the development of hyperkinesis in the trunk and extremities. The severe form is characterized by impairment of speech, memory, swallowing processes, the level of self-care and ends in dementia. Huntington's chorea starts with mental disorders in the form of hallucinatory-paranoid, affective and behavioral disorders.
Neuroacanthocytosis
Patients with neuroacanthocytosis suffer not only from choreic hyperkinesis, but also 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, lip twitching, tongue protrusion and other grimaces. With neuroacanthocytosis, the patient involuntarily bites his tongue, lips and inner surface of the cheeks to blood. Complicating factors in this disease are dementia and epileptic seizures.
Lesch-Nayhan disease
Congenital diseases of the nervous system prevent a person from living a full life. Lesch-Nayhan 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 disorders in the functioning of the nervous system. Also, the pathology provokes an increased production of uric acid.
From the first months of life, a child has a developmental delay. Rigidity of the extremities appears 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 a delay in mental development. A baby can harm himself physically by biting his lips or fingers.
Benign Chorea
Benign chorea in children manifests itself in infancy or early childhood. Pathology refers to hereditary diseases. Generalized hyperkinesis with this disease recedes only when the child is sleeping. This kind of pathology differs from Huntington’s chorea in a non-progressive course and normal development of intelligence. At an older age with benign chorea, it is possible to achieve a decrease in hyperkinesis if you contact a medical institution in time.
Secondary forms of chorea
The most common secondary forms of pathology are two: small chorea and pregnant chorea.
The first is also called Sydenham chorea. Exacerbation of streptococcal infection or rheumatism can provoke its occurrence. The mild form is characterized by exaggerated grimaces, expressive gestures, disinhibited movements. More complex forms of choreic hyperkinesis are manifested in the fact that it is difficult for the patient to move around, speak normally, even breathe. With the disease, “tonic” knee and “hardening” reflexes, emotional and affective disorders, and muscle hypertension also arise.
In pregnant women, chorea is a disease that comes back from childhood. That is, those women who have experienced small chorea in their childhood fall into the risk zone. Pathology can occur during the first pregnancy at 2-5 months. In the subsequent course of an “interesting” situation, chorea can also occur. Treatment in such cases is rarely carried out, as the disease disappears by itself after an abortion or after childbirth.
Rheumatic chorea
The onset of the disease occurs against the background of acute 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 often affects children aged 6 to 15 years. In girls, pathology is more common. At the first stage, the disease may not cause concern, as 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 tips of the fingers 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 normal activities, that is, eating, walking, writing.
Rheumatic chorea distorts the face of a child with thousands of grimaces. Severe forms of the disease prevent the patient from standing on his feet, swallowing, talking, while the body is constantly moving. If chorea is diagnosed, treatment should be carried out carefully and urgently.
Diagnostics
Suspicions of chorea arise from strange human behavior. At the 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 the symptoms of chorea appeared, whether the patient was taking medications, or whether he had inflammatory ailments. Next, the specialist must evaluate the involuntary movements. It is also necessary to diagnose and describe the diseases that may accompany chorea, cause serious intellectual disabilities. Symptoms of such diseases do not always occur. Sometimes, in order to detect such a pathology at an early stage, a whole complex 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 the usual full-fledged lifestyle, so her treatment should be timely and comprehensive. The tactics of treatment depend on the causes of the disease. For example, in Wilson-Konovalov’s disease, a patient is prescribed diets with a minimum copper content and drugs whose action is aimed at reducing its absorption.
With Huntington's chorea, antipsychotics and benzodiazepine tranquilizers are prescribed. If chorea is caused by a chronic lack of blood supply to the brain, then drugs that lower blood pressure are needed. With vasculitis, hormonal treatment is performed. If very general 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.