Chorea-like movements

Introduction Chorus is a rapid irregularity, arrhythmia, and large recurrence of limbs and heads. You can control your movements, such as turning neck, shrugging shoulders, intermittent finger flexion and extension (squeezing milk-like grip), swinging hands, stretching arms, etc. Movement, upper limb weight, gait instability or coarse jumping dance-like gait. When heavy, there may be a rapid and large jumping action from one side to the other (dance-like gait), which increases trust when exercising casually or emotionally. It is relieved when it is quiet, disappears during sleep, and the facial muscles are visible and the muscles of the limbs are low. Common in small chorea, Huntington's chorea and application of neuroleptics. Hemiplegia is limited to the body side of the medical insurance, and is common in stroke and brain tumors.

Cause

The disease is an autosomal dominant genetic disease. Homozygous or heterozygous genes containing diseased genes also show disease, and thus its penetrance is considered to be 100%. The HD related gene IT15 (interesting transcript 15) is located between chromosome 4p16.3 region D4S180 and D4S182, and is about 210Kb long. It is encoded as a polypeptide containing about 3144 amino acids and is named Huntington (Huntington Factor, Ht). There is a polymorphic (CAG)n trinucleotide repeat at the 5' end of its open reading frame. The normal value of repeated copy number n is different in the literature. It is generally considered to be below 34, and the majority is considered to be 11-34. The minimum value is 5 or 6, and the upper limit is 36±. In HD patients, The n value of this variant gene increased significantly, with an amplitude of 42-66 and a median of 45. The lower limit was 37, and the upper limit was above 100, reaching 121. Therefore, it is considered that the (CAG)n trinucleotide repeat length reaches a certain range is the cause of HD.

an examination

Related inspection

Brain Doppler Ultrasound (TCD) EEG examination of involuntary movement examination of abdominal wall reflex brain protein metabolism imaging

According to the characteristic dance movement, behavioral personality change and dementia triad, combined with family history to consider diagnosis. There is a certain difficulty in clinical diagnosis when there is no family history. Genetic testing, neuroimaging and neuropsychological testing can help. Among them, the most diagnostic value of genetic testing will be discussed further later. In the case of dance-like involuntary movements and abnormal movements, it is often necessary to distinguish from a variety of diseases with similar abnormal movements.

Differential diagnosis

(1) Congenital chorea is generally a symptom of cerebral palsy or other brain diseases that may occur before and after birth. The appearance of the chorea is early, and it has already begun before the age of 2 years. All are accompanied by other symptoms such as mental retardation, tremor, hand and foot or sputum.

(2) The survivors of nuclear jaundice and jaundice may have a variety of involuntary movements in the future, including dance-like movements. It can be identified by medical history, intellectual disabilities and other forms of involuntary movements.

(3) Hereditary progressive chorea The incidence of this disease is more than middle age, characterized by family history, dance-like movements and progressive dementia. Occasionally, in childhood, it is accompanied by an increase in tonic muscle tone, and often seizures.

(D) abnormal exercise caused by drugs Some children may cause acute motor abnormalities or delayed disorders after taking strong tranquilizers. According to the history of medication, it can be identified. In addition, the dance-like movements of dyskinesia are also seen with the movements of the hands and feet and dystonia.

(5) Habitual habitual habits are also common diseases of children. Can be seen on the face, tongue, neck, upper limbs or throat. Unlike chorea, the action is stereotyped, that is, repeated contraction in the same muscle or the same muscle group. There was no change in muscle tone, muscle strength, and mutual movement.

(6) Hand, foot and sputum caused by low blood calcium disease, patients often complain of abnormal feelings and even muscle pain. Unlike the Wamu sign of small chorea, the middle-earthing hand is characterized by metacarpophalangeal flexion and thumb adduction. Facial nerve slamming tests and beam arm compression tests can also help with diagnosis.

(7) Tourette syndrome is a repetitive rapid tic disorder of the array of muscles, which is seen in the head and neck. Although only half of them spoke slang, each child had a throat twitch, causing repetitive, explosive, meaningless word noise.

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