Ataxia

Introduction Movement disorders, also known as extrapyramidal diseases, mainly manifest as voluntary motor regulation dysfunction, muscle strength, sensory and cerebellar function are not affected. This group of diseases stems from basal ganglia dysfunction, usually divided into two categories: increased muscle tone - decreased exercise and decreased muscle tone - excessive exercise. The former is characterized by poor exercise and the latter is characterized by abnormal involuntary movement.

Cause

(1) Causes of the disease:

The regulation of motor function can be accomplished by the close cooperation of the pyramidal system, basal ganglia and cerebellum. These three are not independent and independent systems, but are functionally an inseparable whole. Dyskine disorders (ie, extrapyramidal diseases) are mainly caused by basal ganglia dysfunction.

(2) Pathogenesis:

The basal ganglia has complex fiber connections and mainly constitutes three important neural circuits: 1 cortical-cortical loop: cerebral cortex - caudate putamen - medial globus pallidus - thalamus - cerebral cortex; 2 substantia nigra-striatum loop : black matter and caudate nucleus, caudal nucleus between the fibers; 3 striatum - globus globule loop: caudate nucleus, caudate nucleus - lateral globus pallidus - subthalamic nucleus - medial globus pallidus.

There is a direct pathway in the cortical-cortical loop (striatum-medial globus pallidus/black matter reticular) and indirect pathway (striatum-lateral globus pallidus-thalamic nucleus-medial globus pallidus/black matter reticular The loop is the anatomical basis for the basal nucleus to achieve motor regulation. The balance of activity between the two pathways is critical to achieving normal motor function.

Degeneration of the nigrostriatal striatum DA pathway leads to excessive basal ganglia export, and the thalamic-cortical feedback activity is excessively inhibited, which weakens cortical motor function facilitation and produces less motility diseases such as Parkinson's disease. Degeneration of striatum neurons leads to a decrease in basal ganglia output, and thalamic-cortical feedback is too powerful for cortical motor function, resulting in hyperactive diseases such as Huntington's disease. Therefore, basal nuclear transmitter biochemical abnormalities and loop activity disorders are the main pathological basis for the development of various dyskinesia symptoms. The treatment of dyskinesias, regardless of the principles of drug or surgical treatment, is based on the correction of transmitter abnormalities and loop activity disorders.

an examination

Related inspection

Exercise function check CT examination of bone and joint and soft tissue

The clinical manifestations of basal nucleus diseases are mainly:

1. Involuntary action.

2. The movement is missing or slow and innocent.

3. Poor posture and muscle tension.

Indirect pathways cause hyperactivity and stiffness, and indirect pathways cause insufficient chorea and throwing; direct pathway hyperfunction leads to dystonia, acromegaly or tics, and insufficient direct function leads to slow movement .

1. History:

(1) Age of onset: often can cause the cause, such as infant or early onset may be cerebral hypoxia, birth injury, bilirubin encephalopathy or genetic factors, tremor in juvenile may be hepatolenticular degeneration; also help Judging the prognosis, such as the primary torsion of the onset of childhood is far more severe than the onset of adult disease; on the contrary, the onset of late onset dyskinesia is more stubborn than the young.

(2) onset of disease: often can indicate the cause, such as acute onset of children or adolescents dystonia may suggest adverse drug reactions, slow onset is mostly primary torsion spasm, hepatolenticular degeneration, etc.; acute onset Severe chorea or eccentric throwing may suggest a vascular cause, and a slow insidious onset may be a neurodegenerative disease.

(3) Course of disease: It is also helpful for diagnosis. For example, small chorea usually relieves within 6 months of onset, which is different from other choreas that start from childhood.

(4) Drugs such as phenothiazines and butyrylbenzenes can cause movement disorders.

(5) Certain diseases such as rheumatic fever, thyroid disease, systemic lupus erythematosus, polycythemia vera, etc. may be accompanied by dance-like movements.

(6) Family history: Diagnostic significance, such as Huntington's disease, benign hereditary chorea, idiopathic tremor, torsion spasm, tics-slang syndrome, etc. have a genetic background.

2. Physical examination: can understand the characteristics of dyskinesia symptoms, clear whether there are other signs and symptoms of the nervous system, such as static tremor, lead tube-like or gear-like muscle rigidity suggest Parkinson's disease, corneal KF ring prompts Wilson's disease, Huntington's disease In addition to movement disorders such as hepatolenticular degeneration, often accompanied by mental and intellectual damage.

Differential diagnosis

Extrapyramidal diseases should be distinguished from motor dysfunction caused by diseases of the pyramidal system. The main clinical features of the latter are sputum (reduced muscle strength), which is generally not difficult to identify. In addition, the identification of different types of extrapyramidal diseases is more important.

The clinical manifestations of basal nucleus diseases are mainly:

1. Involuntary action.

2. The movement is missing or slow and innocent.

3. Poor posture and muscle tension.

Indirect pathways cause hyperactivity and stiffness, and indirect pathways cause insufficient chorea and throwing; direct pathway hyperfunction leads to dystonia, acromegaly or tics, and insufficient direct function leads to slow movement .

1. History:

(1) Age of onset: often can cause the cause, such as infant or early onset may be cerebral hypoxia, birth injury, bilirubin encephalopathy or genetic factors, tremor in juvenile may be hepatolenticular degeneration; also help Judging the prognosis, such as the primary torsion of the onset of childhood is far more severe than the onset of adult disease; on the contrary, the onset of late onset dyskinesia is more stubborn than the young.

(2) onset of disease: often can indicate the cause, such as acute onset of children or adolescents dystonia may suggest adverse drug reactions, slow onset is mostly primary torsion spasm, hepatolenticular degeneration, etc.; acute onset Severe chorea or eccentric throwing may suggest a vascular cause, and a slow insidious onset may be a neurodegenerative disease.

(3) Course of disease: It is also helpful for diagnosis. For example, small chorea usually relieves within 6 months of onset, which is different from other choreas that start from childhood.

(4) Drugs such as phenothiazines and butyrylbenzenes can cause movement disorders.

(5) Certain diseases such as rheumatic fever, thyroid disease, systemic lupus erythematosus, polycythemia vera, etc. may be accompanied by dance-like movements.

(6) Family history: Diagnostic significance, such as Huntington's disease, benign hereditary chorea, idiopathic tremor, torsion spasm, tics-slang syndrome, etc. have a genetic background.

2. Physical examination: can understand the characteristics of dyskinesia symptoms, clear whether there are other signs and symptoms of the nervous system, such as static tremor, lead tube-like or gear-like muscle rigidity suggest Parkinson's disease, corneal KF ring prompts Wilson's disease, Huntington's disease In addition to movement disorders such as hepatolenticular degeneration, often accompanied by mental and intellectual damage.

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