What Is Involved in the Management of Ataxia in Children?

The maintenance of human posture and the completion of voluntary movements are closely related to the brain, basal nodules, cerebellum, vestibular system, and deep sensation. The damage of these systems will lead to poor coordination of movement, impaired balance, etc. These symptoms and signs are called ataxia. It is divided into four types: sensory ataxia, vestibular ataxia, cerebellar ataxia, and hereditary ataxia. Because of its many causes, the same drug treats different causes of ataxia with very different effects. At present, there is no effective medicine or method for the treatment of this disease in the clinic.

The maintenance of human posture and the completion of voluntary movements are closely related to the brain, basal nodules, cerebellum, vestibular system, and deep sensation. The damage of these systems will lead to poor coordination of movement, impaired balance, etc. These symptoms and signs are called ataxia. It is divided into four types: sensory ataxia, vestibular ataxia, cerebellar ataxia, and hereditary ataxia. Because of its many causes, the same drug treats different causes of ataxia with very different effects. At present, there is no effective medicine or method for the treatment of this disease in the clinic.

Chinese name

Ataxia

Foreign name

Marie ataxia

Ataxia I. Etiology and related diseases

The causes of ataxia include cerebellar ataxia, deep sensory ataxia, cerebral ataxia, and vestibular ataxia.

Cerebellar ataxia

(1) Cerebellar vermiform lesions: It is common in cerebellar vermiform tumors. It is more common in children with medulloblastoma, astrocytoma and ependymal tumor, and in adults with metastatic tumors.

(2) Cerebellar hemisphere damage: common in tumors, metastases, tuberculomas or abscesses, and vascular diseases.

(3) Total cerebellar ataxia: common in cerebellar degeneration and atrophy.

2. Deep sensory ataxia

(1) Peripheral neuropathy: common in polyneuritis, lead, arsenic, and mercury poisoning, alcoholism, and metabolic diseases.

(2) posterior root lesions: common in metastatic tumors.

(3) Posterior cord disease: common in spinal cord degeneration, alcoholism, spinal cord compression and so on.

(4) Thalamic lesions: common in cerebrovascular diseases.

(5) Parietal lobe lesions: common in cerebrovascular diseases and tumors.

3. Cerebral ataxia

It is common in cerebrovascular diseases, tumors, inflammation, trauma, degenerative diseases, etc. in the frontal lobe, parietal lobe, temporal lobe, occipital lobe, and corpus callosum.

4. Vestibular ataxia

Common in acute labyrinthitis, inner ear hemorrhage, acute lesions of the vestibular nerve or vestibular nucleus.

Common diseases that cause ataxia include temporal lobe tumors, vascular reticuloma, hemolytic uremic syndrome, hereditary ataxia, small chorea, chronic progressive chorea, Creutzfeldt-Jakob disease, sedative and hypnotic poisoning , Lead poisoning, botulism, etc.

There are some rare diseases, such as juvenile spinal hereditary ataxia, hereditary spastic ataxia, hereditary spastic paraplegia, ataxia capillary dilatation, atrophy of the pontine cerebellum, atrophy of the cerebellum olive, muscle Clonic cerebellar coordination disorder. And some rare diseases, such as hereditary ataxia, cataract, dwarfism, mental retardation syndrome.

Ataxia 2. Differential diagnosis

The differential diagnosis check method is: observe the patient's daily movements, such as whether the body movements are accurately coordinated during eating, dressing or undressing, unbuttoning, taking things, standing, walking, etc. If the movements are inaccurate and uncoordinated, they should be Think of ataxia. In order to accurately determine the extent, the following physical examination can be performed:

1 Ataxia 1. Physical examination

(1) Finger-nose test : Ask the patient to straighten his upper limbs first, and then touch his nose with his index finger. During the test, first open the eyes and then close the eyes to do this action. If the movement of one side limb is slow and awkward, the fingers will sway or tremble when they move, and they cannot accurately touch the tip of the nose, it is positive.

(2) Rotation exercise test : The patient is instructed to perform a quick flip exercise with both hands. If the movement of one side of the limb is slow and awkward, the test is positive.

(3) Heel and tibia test : The patient lies on his back and asks him to raise one lower limb, put the heel on the knee of the opposite lower limb, and slide it down the front of the tibia. If the heel cannot be placed on the knee accurately, also Can not slide straight down the tibia, which is positive for the test.

(4) Tracing test : When the patient is lying on his back during the examination, he is instructed to draw triangles, circles, or squares in the space with his feet. If he cannot complete this exercise, the test is positive.

(5) Ang white test : Ask the patient to stand upright, with both feet close together, with both hands stretched forward. When the eyes are opened and closed, pay attention to observe whether there is a tendency to sway or fall. If there is a phenomenon of swinging or dumping when the eyes are closed, the test is positive, which is also known as the difficulty of closing the eyes.

2 Ataxia 2, auxiliary examination

(1) Cerebellar ataxia

CT or MRI of the brain should be checked to exclude cerebellar tumors, metastases, tuberculomas or abscesses, vascular disease, and cerebellar degeneration and atrophy.

(2) Deep sensory ataxia

If the localized lesion is located in the peripheral nerve, the electromyography and somatosensory evoked potential should be checked; if the posterior root lesion or posterior cord lesion is considered, the electromyography, evoked potential, MRI of the lesion site, cerebrospinal fluid examination, or myelography should be examined. Considering CT or MRI of the brain is best examined in the thalamus or parietal lobe.

(3) Cerebral ataxia

Cerebrovascular diseases, tumors, inflammation, trauma, degenerative diseases are more common, and brain CT or MRI, EEG, etc. should be checked.

(4) Vestibular ataxia

Can check electrical audiometry, auditory evoked potentials, vestibular function tests, etc.

Ataxia III. Principles of treatment

The effect of the same drug on ataxia with different causes is very different, and there is no specific drug treatment for ataxia. The main treatment is primary disease, and corresponding symptomatic treatment is given. There are also acupuncture treatments.