Chronic progressive chorea

Introduction to chronic progressive chorea Chronic progressive chorea is a slow-progressive cerebral cortex and striatum degenerative disease with autosomal dominant inheritance. Clinically, it is clinically characterized by dance-like movements, mental disorders and progressive dementia. basic knowledge The proportion of sickness: 0.01% Susceptible people: more common in the elderly Mode of transmission: mother-to-child transmission Complications: progressive lipodystrophy progressive muscular dystrophy deafness hereditary spastic paraplegia fibromatosis

Cause of chronic progressive chorea

Cause:

Is an autosomal dominant genetic disease, the gene is located on the autosome 4p16.3, the gene product is Huntingtin, the pathology is atrophy of the caudate nucleus, followed by the putamen, basal white ball, neuronal degeneration and loss, among which The loss of -aminobutyric acid (GABA) neurons and cholinergic neurons was markedly reduced, and the loss of dopamine neurons was relatively small, and somatostatin neurons were preserved.

Chronic progressive chorea prevention

The disease is an autosomal dominant genetic disease with no effective preventive measures and usually dies 10-20 years after onset.

The prevention of genetic diseases mainly focuses on the following aspects: environmental protection, detection of genetic carriers, genetic counseling, marriage guidance and selective abortion, and prevention before symptoms appear. Neonatal screening is an effective method for preventing and treating certain genetic diseases after birth. It is usually carried out on paper with cord blood or heel blood. The selected conditions should consider the following conditions: 1 high incidence; 2 serious consequences of death, disability, and stupidity; 3 more accurate and practical screening methods; 4 screening of diseases with methods; 5 Economic Benefits Marriage guidance and birth guidance for patients with genetic diseases and their relatives, if necessary, ligation surgery or termination of pregnancy, can prevent the birth of children, reduce the corresponding disease-causing genes in the population.

Chronic progressive chorea complications Complications , progressive lipodystrophy, progressive muscular dystrophy, deafness, hereditary spastic paraplegia

A few reports that the disease can be combined with other diseases, such as progressive muscular dystrophy, deafness, spastic paraplegia, neurofibromatosis, acute osteoarthritis.

Chronic progressive chorea symptoms Common symptoms Dementia Ataxia Mental disorder Irritable uneasiness Episode involuntary movement Depression Abnormal Large dance-like movements irritating irritability

1, the disease usually 30 to 40 years old, gradually increased, family history, occasional sporadic cases, early visible irritability, depression and antisocial behavior and other mental disorders, advanced progressive dementia; dyskinesia initially manifested obvious I was restless and uneasy, and gradually developed into an unusually large dance-like movement.

2, a small number of cases of atypical symptoms (Westphal variant), showing progressive muscle rigidity and decreased exercise, no dance-like movements, mostly in childhood, epilepsy & cerebellar ataxia is also a common feature of adolescents, With dementia and family history.

Chronic progressive chorea examination

1, CT and MRI examination often confirmed in the confirmed cases of cerebral cortex and caudate nucleus atrophy.

2. Positron emission tomography (PET) showed a significant decrease in glucose metabolism in the striatum.

3, EEG can have diffuse abnormalities.

Diagnosis and identification of chronic progressive chorea

Diagnosis can be based on medical history, clinical symptoms, and laboratory findings.

Differential diagnosis

1. A rare benign chorea is an autosomal dominant inheritance in childhood, with only mild muscle weakness, no intellectual changes, and no progression.

2, senile chorea occurs in the elderly, can be caused by stroke, acute onset, can present partial dance chorea.

3, L-dopa, phenothiazines and contraceptive-induced chorea, have a history of medication, and are not accompanied by dementia.

4, atherosclerotic cortical encephalopathy occurs in the elderly, dementia is very light, often accompanied by arteriosclerosis diabetes, may have hemiplegia, muscle atrophy or vertebral body bundles and other nerve damage, brain CT and MRI may have lateral ventricle vaginal Quality damage can be identified only with dementia in the sporadic cases of this disease.

5, vascular dementia has a history of stroke, severe hemiplegia and other signs of neurological damage, can be identified with the disease only scattered patients with dementia.

6, senile dementia occurs in elderly patients, with intelligent barriers as outstanding performance, no hyperactivity in the performance, PET with 18F-deoxyglucose is mainly in the extensive metabolic disorder of the cortex without striatum of the caudate nucleus damage.

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