Athetosis
Introduction
Introduction to Hand and Foot Mob Hand and athesis (athetosis), also known as finger movement, or variability (mobilespasm), is characterized by increased distal and distal migratory muscle tone and reduced movement, slow as a creepy, peristaltic peristalsis, and muscle tone The obstacle is similar, not an independent disease unit, which is a relatively slow, untargeted, continuous involuntary motor syndrome in the fingers, toes, tongue or other parts of the body. basic knowledge The proportion of sickness: 0.01-0.03% Susceptible people: no special people Mode of infection: non-infectious Complications: stunting, mental retardation
Cause
Causes of hand and foot hyperactivity disorder
Causes:
Common causes of atherososis are: 1 hereditary or familial: mostly autosomal recessive, rare; 2 cerebrovascular accident; 3 intracranial infection; 4 drugs; 5 cerebral palsy: caused by various causes Cerebral palsy can occur in children with dyskinesia; 6 high-grade cervical spinal cord disease has different causes, such as paroxysmal dystonic choreoathetosis (PDC), which is a rare hereditary dyskinesia disease. Sexual exercise-derived dances (paroxysmal kinesigenic choreoathetosis (PKC)), a rare dyskinesia disease, often induced by exercise, sometimes associated with diffuse or focal brain injury, and also considered to be a type of epilepsy Form, because of similar causes of seizures, anti-epileptic treatment is effective.
Pathogenesis:
Genetic factors (25%):
Mostly autosomal recessive inheritance, rare, a seizure dystonia dance in Japan, the family gene is located at 2q31 ~ 36, and can be further located between D2S164 and D2S377, this region encodes the PDC gene, At present, a new X-linked hereditary disease can be caused to cause dance and hand-foot movement, which is located in Xp11, accompanied by intelligent developmental delay, behavioral abnormalities, etc.; other minority hereditary neurological diseases, such as leukodystrophy, spinal cord cerebellum Arrhythmia, hepatolenticular degeneration and familial hypoproteinemia, can also be associated with hand and foot akines.
Cerebrovascular accident (20%):
Congenital infant Heubner return arterial occlusion can lead to contralateral limb movements of the hand and foot. Adult acute thalamus, globus infarction can cause movements of the hand and foot, and autopsy can reveal the lacunar-like state of the posterior limb of the internal capsule.
Intracranial infection (15%):
Creutzfeldt-Jakob disease caused by lentivirus may have dementia, myoclonus and bilateral hand movements, MRI showed bilateral caudate nucleus, atrophy of the putamen and high signal of T2WI; herpes simplex virus, Mycoplasma pneumoniae, Toxoplasma gondii Intracranial infection with AIDS can also cause acromegaly.
Drugs (10%):
Cocaine, amphetamine and other addicts may experience dance hand and foot akines, long-term use of antipsychotic drugs such as phenothiazine, haloperidol and other acute dystonia or tardive dyskinesia, can also show hand and foot Xu Motility.
Cerebral palsy (5%):
Various causes of cerebral palsy may occur in hand and foot hyperkinesia, such as perinatal hypoxic encephalopathy, premature labor, birth injury, bilirubin encephalopathy, cerebral cortical dysplasia, brain penetrating malformation, bilirubin encephalopathy, etc., and adult heartbeat Stop, poisoning leads to hypoxic encephalopathy.
Pathological changes:
Mainly involved in bilateral caudate nucleus, putamen and hypothalamus, neuronal degeneration, disappearance, gliosis, medullary fiber bundles significantly increased, irregular distribution, bundled or reticular arrangement, myelin staining is plaque, Like marble, called "status marmoratus", it can be seen that the reduction of Nissl body disappears, the striatum shrinks, the thalamus, the globus pallidus, the substantia nigra, the internal capsule and the cerebral cortex can also be degenerated, and both sides of the hand and foot acrodynamics The lateral neurons of the bilateral globus pallidus showed a PES staining positive Bielschowsky body deposition, which was round and located in the nucleus of the nucleus.
Prevention
Hand and foot hyperactivity disorder prevention
Prevention of hand and foot hyperactivity with a genetic background is more important. Preventive measures include avoiding marriage by close relatives, conducting genetic counseling, genetic testing of carriers, prenatal diagnosis and selective abortion to prevent the birth of children.
Early diagnosis, early treatment, and intensive clinical care are important for improving the quality of life of patients with acromegaly.
Complication
Complications of acromegaly Complications, stunting, mental retardation
Throat muscle, lingual muscle involvement, unclear speech, dysphagia, etc.; various causes of cerebral palsy may appear hand and foot hyperkinesia, such as perinatal hypoxic encephalopathy, premature delivery, birth injury, bilirubin encephalopathy, cerebral cortical dysplasia , brain penetrating malformation, bilirubin encephalopathy, adult cardiac arrest, poisoning, etc. lead to hypoxic encephalopathy, etc., so there are primary cerebral palsy manifestations; cerebral palsy children still have developmental delay, mental retardation, walking or talking Delayed performance.
Symptom
Symptoms of Hand and Foot Mobility Symptoms Symptoms of the hand
1. Clinical classification:
This syndrome can be a variety of neurological diseases, divided into three types according to clinical manifestations:
(1) bilateral two-handedness (doubleathetosis): characterized by often accompanied by myoclonus and irregular, small amplitude exercise, common in patients with cerebral palsy.
(2) dance hand and foot hyperactivity disorder (choreoathetosis): performance of hand and foot akines with a larger dance-like movement, seen in familial episodes of hand and foot acrodynamics, non-progressive familial dance, hand and foot hyperkinesis.
(3) Unilateral and pseudo-hand and foot movement: Cerebrovascular disease or other causes of deep sensory disturbance, unilateral athetosis and pseudoathetosis are not basal ganglia lesions.
2. Main clinical manifestations:
(1) Congenital acromegaly is usually an involuntary movement after birth, but it can also become obvious after several months of life. Stunting, delay in starting to sit up, walking or speaking, involuntary Exercise has already begun, but it is not obvious at first, until the child can exercise freely. Symptomatic acromegaly can occur at any age, both men and women can be affected by hepatic encephalopathy, phenothiazine, fluoride Hand and foot hyperactivity caused by piperidol or levodopa overdose often develops after adulthood or in old age.
(2) The unique movements of the hand and foot in this disease are the singular and compulsory movements of the hands and feet that are constantly making slow, curved or squatting crawling. These movements are more prominent at the distal end of the extremities, and the lower limbs are affected. When the big toe is often spontaneously dorsiflexed, causing a false Babinski sign, sometimes the face can be affected, the patient often frowns, dressed up as a variety of faces, throat muscles and tongue muscles are involved, then the words are not Clear, difficult to construct, the tongue sometimes retracts when it is extended, and there are obstacles in swallowing. It may be accompanied by twisting or spasmodic torticollis. This involuntary movement may be due to emotional stress, or when the spirit is stimulated, or casual. Increased during exercise, relieved when completely quiet, stopped when falling asleep.
(3) High and low fluctuations in muscle tension, muscle tension is increased when the tendon is increased, and muscle relaxation is normal. Therefore, this disease is also called mobile spasm. About half of patients may have double due to pyramidal beam involvement. Side squat or sputum, especially in the lower limbs, more than half of them have mental retardation and the whole body feels normal.
(4) The disease is generally a chronic disease, and the course of disease can last for several years or decades. A small number of patients can be stopped for a long time without progress, severe movements of the hands and feet, and accompanied by throat muscles, can be early Died of complications.
Hand and foot hyperactivity disorder has involuntary movements of special postures of the hands and feet, so the diagnosis is not difficult. The dance-like movements of chorea appear on the limb trunk and head and face, and the range is wide, and it is more rapid than the involuntary movement, which is beating, different from Involuntary involuntary movements are mainly limited to the hands and feet, but the intrinsic sometimes coexists with chorea, called dance-chorea-athetosis.
Examine
Examination of acromegaly
1, blood, urine, stool routine and electrolyte examination, depending on the cause may have different results.
2, cerebrospinal fluid examination, there is a differential diagnosis.
3, MRI examination of children with cerebral palsy, T2WI more visible bilateral hypothalamus, high signal of nucleus symmetry, and some high signal around the lateral ventricle.
4, familial episodes of dystonia dance, hand-foot movement, EEG and head MRI examinations, no abnormalities, episodes of exercise-induced dance, hand-foot-knee electroencephalogram and head MRI examinations are also no abnormalities, However, SPECT examination showed a decrease in cerebral blood flow in the contralateral basal ganglia at the time of onset.
5, symptomatic hand and foot aphosis caused by other brain diseases, CT, MRI may have related performance.
6, genetic testing has the primary cause of differential diagnosis.
Diagnosis
Diagnosis and differentiation of hand and foot akines
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory findings.
Differential diagnosis
1, the disease should be distinguished from pseudo-hand and foot akinesia, the latter caused by the loss of position of the limb with a frontal lobe, combined damage to the posterior column and lateral column or peripheral nerve damage.
2, very slow hand and foot Xu movement caused abnormal posture similar to twisting sputum, the latter mainly invaded the proximal limb, the neck muscles and the trunk muscles, the typical performance is torsion as the axis.
3, should pay attention to the identification of dance - hand and foot hyperactivity and other clinical types, dance - hand and foot hyperactivity disorder patients with limbs, trunk and face appear in a wide range of involuntary movements, showing large, variable and rapid beating.
