Cerebellar disorders

Introduction Spinal cerebellar disorders are the main symptoms of movement disorders. Pathologically, the disease is mainly caused by degeneration of the cerebellum and its afferent and efferent pathways. Clinically, it is characterized by limb ataxia and dysarthria. A large number of clinical data reports have shown that most patients with cerebellar atrophy are hereditary, and the condition is chronic, progressive deterioration, and if not effectively controlled, it will soon be life-threatening. Therefore, once it is found that early medication should be used, it can effectively control the disease, improve the original symptoms, improve the quality of life, and delay life. However, there is no cure for it. It is an incurable disease.

Cause

First, the etiology and pathology of Western medicine

The etiology of this disease is unknown, but most of them have a family genetic predisposition. Most of the patients who started from the age of 20 were autosomal recessive, while those who started after the age of 20 were mostly autosomal dominant. After long-term research, many scholars at home and abroad have located the Friedreich ataxia-deficient gene at 9q13~q21, and the OPCA genetic gene at 6p24~p23. At the same time, it was found to be associated with viral infection, immunodeficiency, and lack of biochemical enzymes. There are many factors related to abnormal DNA repair function, but the exact cause is not fully understood.

In terms of pathology, its performance is diverse. Commonly, there are atrophy and degeneration of nerve cells, loss of myelin sheath, mild hyperplasia of glial cells, and extensive degeneration of the cerebellar hemisphere and ankle, cerebellum and lower abundance, Purkinje cells. Disappeared; nerve cells in the posterior column of the spinal cord and Clark column atrophy or disappear, secondary glial cell hyperplasia, posterior root and spinal ganglion degeneration, myelin loss, especially in the lumbar and temporal spinal cord. Degeneration of the brain stem part of the cerebral cortex, basal ganglia, thalamus, and basal ganglia of the pons can also be seen.

Second, the pathogenesis of Chinese medicine

Chinese medicine believes that this group of syndromes is not enough congenital endowment, kidney deficiency or disease prolonged for a long time, lack of blood, brain no filling, tendon dystrophy; or due to emotional disorders, poor air, qi Blood stasis causes the dysfunction of the five internal organs. Among them, the kidney is deficient. Because the kidney is hidden by the essence of the internal organs, and the marrow is injected into the brain, so that the marrow sea is full, and the marrow is more than enough, it is light and powerful, and it is self-sufficient. Insufficient myelin, the brain turns to tinnitus, sputum sputum, nothing to see, slack and sleep. That is to say, kidney essence deficiency can cause dizziness, tinnitus, vertigo, limb weakness, mental exhaustion, unstable walking, and even bedridden. In addition, the disease has a very close relationship with the dysfunction of the liver and spleen. The kidney is the main bone of the essence, the main blood of the liver is hidden, the liver and kidney are both deficient, the bone is in the roots, and the veins are not honored, but the foot is full of veins. Insufficient kidney, kidney yang deficiency, sealing dereliction of duty, can also lead to temper weakness, so see waist and knees, impotence and nocturnal emission, menstrual flow or amenorrhea, tinnitus and deafness, mental euphemism, Shenpi fatigue, less gas lazy, moving Then it is very good, pale, and then stayed in the squat, the frequency of urination, the rest of the leaching, the pulse is too late.

It can also be seen that the yin and anger are prosperous, and the yin and yang are outside. "Nei Jing" cloud: "The kidneys are strong officials, and they are clever." Only when the kidneys function normally, the human body can coordinate smoothly. Although this group of syndromes and liver, spleen, and kidneys are dirty, kidney deficiency is fundamental. Kidney yin deficiency, can not be filled with the marrow, strong and no power, no cleverness, so the action is clumsy, faltering; kidney deficiency and lack of blood, can not restrict the yang, yin deficiency, the sun floating on the virtual The wind is endogenous, and the limbs are fluttering, the body is shaking; the brain is lost, so the thinking is slow, the expression is dull, the intelligence is low, and the spirit is euphemistic.

an examination

Related inspection

Cerebrospinal fluid culture + drug sensitivity test combined with flexion tracing test human body weight balance instrument in situ test

Any child who was healthy in the past, after a cold, diarrhea, especially chickenpox infection, quickly appeared gait instability, like a drunkard drinking too much alcohol, is sitting in a chair and swinging left and right; Taking something is not a hand, but a trembling can't catch something; at the same time, you can find the abnormal movement of the eyeball in the eyelid. When children develop these phenomena, first consider the presence of acute cerebellar ataxia.

Differential diagnosis

Differential diagnosis of cerebellar disorders:

1, Friedreich ataxia: is the prototype of spinal ataxia. It belongs to autosomal recessive inheritance. The relevant gene is located on chromosome 9. Gait instability occurs between the ages of 5 and 15, followed by upper limb ataxia and sputum eating. Intelligence often also declines. If tremor occurs, it is a secondary symptom. The sputum reflex disappears and there is a loss of the feeling of large fiber conduction (vibration and position). Common arched feet, scoliosis and progressive myocardial lesions. Both blood -lipoprotein deficiency (Bassen-Kornzweig syndrome, vitamin E deficiency) and Refsum disease have some clinical manifestations of Friedreich ataxia, but the underlying metabolic disorder is currently unknown.

2, multiple system atrophy: in multiple system atrophy (the olivine bridge cerebellar atrophy), ataxia occurs in young and middle-aged. Additional symptoms include different combinations of rigidity, extrapyramidal symptoms, sensory disturbances, lower motor nerves Meta-symptoms and autonomic dysfunction. Optic atrophy, pigmented retinitis, ocular tendon and dementia can occur in certain families. These syndromes include Menzel dominant genetic disease (with cranial nerve disorders and rigidity); Dejerine-Thomas sporadic or recessive genetic syndrome (with significant Parkinson's syndrome symptoms); Azov-type motor system degeneration (Machado- Joseph's disease); and cerebellar ataxia with autonomic dysfunction (Shy-Drager syndrome).

3. Some systemic diseases with unknown pathogenesis, such as ataxia-telangiectasia, can also produce ataxia. In mitochondrial multisystem diseases, in addition to ataxia, there are different combinations of eye muscle spasm, heart block and myopathy. The activity of several respiratory chain enzymes is reduced, the loss of mitochondrial DNA, and muscle biopsy reveal characteristic broken red fibers.

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