Bilateral atherosclerosis
Introduction
Introduction 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, with a slow, creepy, wriggle-like peristalsis. Similar to dystonia, it is not an independent disease unit. It is a relatively slow, untargeted, continuous involuntary motor syndrome in the fingers, toes, tongue, or other parts of the body. 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 medium and small amplitude exercise, common in patients with cerebral palsy. (2) Dance hand and foot hyperactivity disorder (choreoathetosis): a dance-like movement with a large range of hand and foot akinesia, seen in familial episodes of acrobatics, non-progressive familial dance, and acrodynamics. (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.
Cause
Cause
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 appear hand and foot hyperactivity; 6 high cervical spinal cord lesions.
Different types of causes, such as paroxysmal dystonic choreoathetosis (PDC), are rare hereditary dyskinesias. Paroxysmal kinesigenic choreoathetosis (PKC) is a rare dyskinesia disease that is often induced by exercise and sometimes associated with diffuse or focal brain injury. It is also considered to be epilepsy. In one form, anti-epileptic treatment is effective because of similar causes of seizures.
(two) pathogenesis
1. Hereditary or familial: mostly autosomal recessive inheritance, rare, a paroxysmal dystonia dance in Japan, the family gene is located at 2q31 ~ 36, and can be further located in D2S164 and D2S377 In the meantime, this region encodes the PDC gene, and a new X-linked hereditary disease has been found to cause dance acromegaly, localized in Xp11, accompanied by intelligent developmental delay, behavioral abnormalities, etc.; other minority hereditary neurological diseases, such as the brain White matter malnutrition, spinocerebellar ataxia, hepatolenticular degeneration and familial hypoproteinemia can also be associated with acromegaly.
2. Cerebrovascular accident: Congenital infant Heubner return arterial occlusion can lead to contralateral limb movements of the hand and foot. Adult acute thalamus and globus infarction can cause movements of the hand and foot. The autopsy can show the lacunar-like state of the posterior limb of the internal capsule.
3. Intracranial infection: Creutzfeldt-Jakob disease caused by lentivirus may have dementia, myoclonus and bilateral hand movements, MRI showed bilateral caudate nucleus, atrophy of the putamen and T2WI high signal; herpes simplex virus Intracranial infections of Mycoplasma pneumoniae, Toxoplasma gondii and AIDS can also cause acromegaly.
4. Drugs: cocaine, amphetamine and other addicts may appear to dance hand and foot hyperactivity, long-term use of antipsychotic drugs such as phenothiazine, haloperidol caused by acute dystonia or tardive dyskinesia, also Can show hand and foot akines.
5. Cerebral palsy: 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, etc. And sudden cardiac arrest in adults, poisoning caused by hypoxic encephalopathy.
6. High cervical spinal cord lesions: such as cervical demyelinating lesions, in addition to causing deep sensory loss, there may also be pseudo-hand and acrodynamics or fingering movements.
Pathological changes: mainly involved bilateral caudate nucleus, putamen and hypothalamus, neuronal degeneration, disappearance, gliosis, medullary fiber bundles significantly increased, irregular distribution, bundled or reticular arrangement, myelin staining It is plaque-like, like marble, called "status marmoratus", showing that the Nissl body disappears and the striatum shrinks. Thalamus, globus pallidus, substantia nigra, internal sac and cerebral cortex can also be degenerated. In the bilateral lateral globus pallidus neurons of both sides of the hand and foot hyperactivity, PES staining positive Bielschowsky body deposition was observed, which was round and located in the nucleus of the nucleus.
Examine
an examination
Related inspection
Involuntary exercise check CT examination
Hand and foot hyperactivity disorder has involuntary movements in the special posture of the hands and feet, so the diagnosis is not difficult. The dance-like movements of chorea appear in the trunk and head and face of the limbs. They are wide-ranging and more rapid than involuntary movements. They are beating-like, and the involuntary movements different from the intrinsic are mainly limited to the hands and feet. But the intrinsic sometimes coexists with chorea, called dance-chorea-athetosis.
Diagnosis
Differential diagnosis
1, the disease should be distinguished from pseudo-hand and foot akines. The latter is caused by the loss of position of the limb with a combination of frontal, posterior and lateral column damage or peripheral nerve damage.
2, very slow hand and foot Xu movement caused abnormal posture is quite similar to twisting sputum, the latter mainly invades the proximal limb, neck muscle and trunk muscle, and the typical performance is torsion with the trunk 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 a wide range of involuntary movements in the limbs, trunk and face, showing coarse, variable and rapid beating.
Hand and foot hyperactivity disorder has involuntary movements in the special posture of the hands and feet, so the diagnosis is not difficult. The dance-like movements of chorea appear in the trunk and head and face of the limbs. They are wide-ranging and more rapid than involuntary movements. They are beating-like, and the involuntary movements different from the intrinsic are mainly limited to the hands and feet. But the intrinsic sometimes coexists with chorea, called dance-chorea-athetosis.
