Physical incoordination

Introduction In the absence of muscle strength, the coordinated movement of limb movements is dysfunctional, unstable and uncoordinated, called ataxia. Ataxia telangiectasia is a neurocutaneous syndrome characterized by progressive cerebellar ataxia, ocular telangiectasia, and repeated upper respiratory tract infections. Girls have more morbidity than boys. The most obvious change in this disease is vasodilation of the bulbar conjunctiva, followed by telangiectasia of the eyelid skin, followed by telangiectasia of the face, ear and neck. Sometimes the skin can also be seen with changes in coffee milk spots, pigmentation spots, and the like. The neurological symptoms first manifested as cerebellar ataxia, which began to manifest when walking, 18 to 24 months after birth began to appear symptoms, but also started to become sick after 5 years of age, sick children appear inconsistent movement, walking gait Stable, inaccurate with the finger nose, etc., to 12 to 15 years old to develop completely unable to walk. As the disease progresses, the intelligence gradually diminishes. Children with this disease often have a history of repeated upper respiratory tract infections, or recurrent pneumonia and sinusitis. Blood immunoglobulin (lgA) was reduced.

Cause

Any simple movement of the body must be completed by the participation of the four groups of active muscles, antagonist muscles, synergistic muscles, and fixed muscles, and depends on the coordination and balance of the nervous system. The thin bundle and the wedge bundle of the posterior cord of the spinal cord penetrate the entire length of the spinal cord, and the thin beam transmits the deep feeling of the lower part of the trunk and the lower limbs, and the wedge beam transmits the deep feeling of the upper part of the trunk and the upper limbs. The fibers emanating from the posterior cord are crossed at the medulla, through the interverted thalamus to the cerebral cortex, the posterior bundle transmits muscles, the deep sense of the joints and tendons, the position of the limbs in space, the impulses of the force and range of limb movement, and parts Feeling and two points to identify feelings. The front spin system conducts balance information to the center of the heart, causing an equilibrium response.

The cerebellum is an important center for maintaining body balance, mutual aid exercise, and muscle tone. The function of these structures is all done under the unified control of the cerebral cortex. Ataxia can occur in deep sensation, anterior rotation system, brain and upper brain lesions, which are called sensory, anterior, cerebellum and cerebral ataxia.

1. Sensory ataxia: Ataxia is relieved when blinking, and is aggravated when closing eyes, accompanied by positional sensation, and the sense of vibration is reduced or disappeared. Due to the deep feeling of the lower limbs, the lower limbs are more common, so the instability of standing and gait instability are the main performance. The patient has difficulty walking at night, and the body is easy to fall in the direction of the wash basin when washing the face (the wash basin sign is positive). When walking, the eyes are gazing at the ground, the foot is too high, the step is wide, and the ground is too heavy, like a cross-threshold, so it is called a cross-threshold gait. Closed eyes are difficult to sign positive, finger nose test, and knee test are not correct.

2. Cerebellar ataxia: The cerebellum and its afferent fibrotic lesions can cause ataxia, which is characterized by the balance of the trunk and the instability of the standing, and the ataxia of the limbs. For dyskinesia, coordination failure, exercise initiation and termination delay or continuity disorder.

Cerebellar ataxia is not affected by blinking, closed eyes or illumination, without sensory disturbances, nystagmus, dysarthria, nephropathy and special cerebellum gait, that is, the two feet are separated when walking, and the steps are different. The gait is unstable and easy to dump. The ataxia is extremely obvious in the finger-nose test. It can be seen that the upper limb has a curved swing and intentional tremor, and the muscle tension is reduced or disappeared, the joint movement is excessive, the quick-reaction movement disorder, and the muscle rebound phenomenon.

3. Vestibular ataxia: caused by damage to the vestibular system, mainly due to balance disorders. It is characterized by a balance disorder between static and exercise. It has the same points as cerebellar ataxia, such as wide base of the feet when standing, unstable body, dumping to the side or rear, and skew when walking. However, there are generally obvious dizziness, nystagmus and abnormal vestibular function tests.

4. Hereditary ataxia: Chronic diseases of the central nervous system, the cause is unknown, mostly family history, autosomal recessive or dominant inheritance, and occasional sexual inheritance. Pathological changes are mainly caused by spinal cord, cerebellum, and brain stem degeneration. Peripheral nerves, optic nerves, brain and cerebellum can also be affected. Clinical manifestations of ataxia and poor discrimination are the main manifestations.

an examination

Related inspection

Electromyography of the bones and joints of the extremities

Physical examination

1. Finger nose test: When the ataxia is at a loss, the action is light and heavy, and the speed is not the same. If you misunderstand or adjust, you can point to the target. When the cerebellar hemisphere lesions are manifested, the more the ataxia is closer to the target, the more obvious the ataxia is, and the poor distance can often exceed the target. In the case of sensory ataxia, the blink of an eye is a barrier to movement, but when the eyes are closed, there is a clear ataxia.

2, knee squat test: cerebellar damage caused by poor positioning and intentional tremor when lifting legs and knees, often swaying when moving down; when sensory ataxia, the patient's heel often can not find the knee, move down The swing is uncertain.

3, rapid rotation test: cerebellum damage when the action is clumsy, uneven rhythm.

4, rebound test: patients with cerebellar lesions often lead to excessive movement and attack themselves.

5, over-finger test: vestibular ataxia, when the upper limbs decline, the side of the disease is lost; when the sensory ataxia, the eyes of the examiner are often not found when closing the eyes.

6. Toe-finger test: The patient is lying on his back and lifting the big toe to reach the extended finger.

7. Sit-up test: The pith and the trunk of the patient with cerebellar damage are flexed at the same time, and the lower limbs are lifted up, which is called combined flexion sign.

Auxiliary inspection

1, cerebellar ataxia should be examined brain CT or MRI to exclude cerebellar tumors, metastases, tuberculoma or abscess and vascular disease and cerebellar degeneration and atrophy.

2, deep sensory ataxia such as localized lesions located in the peripheral nerve should be examined EMG, somatosensory evoked potential; such as in the posterior root lesion or posterior cord lesion should be examined EMG, evoked potential, MRI of the lesion, cerebrospinal fluid Check, or myelography. It is best to check brain CT or MRI when considering the thalamus or parietal lobe.

3, cerebral ataxia should be checked for brain CT or MRI, EEG and so on.

4, vestibular ataxia can be checked for electrical audiometry, auditory evoked potentials, vestibular function tests.

Differential diagnosis

First, sensory ataxia

(a) peripheral neuropathy

Clinically, it is common in various causes of polyneuritis, such as toxic, metabolic, hereditary polyneuritis. Mainly manifested as the symmetry of the distal limbs, sensation of movement and nutrition, decreased muscle tone, loss of tendon reflex, muscle tenderness and so on. The main feature of the ataxia is the ataxia of the extremities, the lower extremities are heavier than the upper limbs, the distal end is heavier than the proximal end, and the eyes are aggravated when the eyes are closed. This type has deep sensory disturbances, no Argyll-Robertson's pupil, no sphincter disorders, and the above three points can be differentiated from posterior bundle or spinal cord.

(two) posterior root lesions

Patients with multiple radiculitis may have ataxia, accompanied by paresthesia, symptoms of terminal type sensory disturbance, myalgia, decreased muscle tone, loss of tendon reflex and increased protein in cerebrospinal fluid. The spinal cord tendon damages the posterior root and the posterior bundle, and typical sensory ataxia occurs. There may be lightning-like pain, body tract sensation, sphincter dysfunction, Argyll-Robertson's pupil, knee, Achilles tendon reflex reduced or disappeared, and blood and cerebrospinal fluid Huakang reaction positive.

(three) posterior bundle disease

Sensory ataxia can occur in various causes of damage to the posterior spinal cord, such as subacute combined degeneration, spinal cord tumors, and spinal cord hereditary ataxia. It is characterized by the separation of feelings, that is, the sense of touch, the sense of temperature and pain are not damaged, and the sense of position, pressure and vibration are reduced or disappeared. Subacute combined degenerative patients often have pyramidal tract damage and have lower extremity muscle weakness, hyperreflexia and pathological signs, and may also have multiple neuritis manifestations and pernicious anemia. Gastric juice analysis often has free acid reduction. Tumors in the posterior spinal cord often have radicular pain, and then symptoms of sensory ataxia gradually occur, often accompanied by conduction beam type shallow sensory dysfunction and pyramidal tract signs, spinal canal obstruction symptoms, and increased protein in cerebrospinal fluid.

(four) brain stem lesions

Sensory ataxia can occur when the posterior bundle or its nucleus or the medial collaterals of the pons and midbrain are damaged. The medullary lesion has its ataxia on the ipsilateral side. The ataxia of the pons and central lesions is contralateral. It is characterized by symptoms of cranial nerve damage on the ipsilateral side of the lesion, and most of them are associated with cerebellar ataxia.

(5) Thalamic lesions

The causes of thalamic ataxia can be vascular, tumor and trauma. In addition to the contralateral half-sensual ataxia, there may be spontaneous pain and shallow sensory disturbance in the contralateral half, and the ataxia is more important than the lower limb. Because the middle abdomen nucleus of the thalamus is associated with the cerebellum, the cerebellar lesions are accompanied by cerebellar symptoms. Thalamic lesions cause deep sensory disturbances. It can be seen that the movements of the hands and feet are obvious, especially in the hands, that is, the so-called thalamic uneasiness, when the eyes are closed, the hand can not maintain a certain posture and the finger is fingering movement, which is caused by the positional dysfunction of the hand. (false hand and foot Xu move). (6) Parietal lesions of parietal lobe lesions caused by sensory ataxia can be found in the contralateral limbs, or part of the limbs, such as hands or fingers. The cause can be vascular, tumor, and the like. There is no parallel relationship between ataxia caused by parietal lesions and deep sensory disturbances, that is, ataxia is obvious and deep sensory disturbance is extremely mild. The deep sensory disturbance of parietal lobe lesions is mainly spatially oriented sensory disturbance.

Second, cerebellar ataxia

(a) cerebellar sac lesion

The cerebellar sacral lesions mainly cause balance disorders, which show torsional ataxia, standing and walking instability, and the limbs of the mutual aid movement is near normal or completely normal, called cerebellar sacral syndrome. Acute progressive cerebellar palpebral lesions are common in tumors, especially in children, such as medulloblastoma, astrocytoma, and ependymoma. In adults, metastatic tumors are more common, and the clinical features are progressive intracranial hypertension and torsade atony. The performance is most obvious when the patient is standing and walking. It is usually seen that the body is shaking and falling backwards, especially when turning around, the apparent gait instability is not obvious, and the upper limb ataxia is not obvious, often accompanied by dizziness and decreased muscle tone. Chronic progressive cerebellar palsy, starting from early childhood with progressive cerebellar ataxia, characterized by ocular telangiectasia; adult with progressive cerebellar degeneration, cancerous cerebellar atrophy, alcoholic cerebellar degeneration, etc. The main clinical manifestations are trunk ataxia and speech disorder.

(B) cerebellar hemisphere lesions

Mainly manifested as the ataxia of the limbs, while the balance of the trunk is not obvious. Common cerebellar hemisphere lesions include astrocytoma, metastatic tumors, abscesses, and tuberculosis. Clinically, it is characterized by chronic side-to-sex aphrodisiac and increased intracranial pressure. It is characterized by early symptoms of increased intracranial pressure, coordinated movement of the limbs, movement of benzene, instability, rapid rotation, dysfunction, finger-nose test and knee-shoulder test, and poor limb resolution. Muscle rebound phenomenon. The head and neck are often fixed in a special position. The head is often tilted forward and turned to the side of the disease. Nausea and vomiting can occur when shaking the head or turning the head.

The mouth is eaten, the articulation is difficult, the nystagmus is obvious, the gait is squatting while walking, and often falls to the side of the disease. Closed eyes are difficult to sign positive. The muscle tone is reduced and the tendon reflex is slow or disappears. The cerebellar hemisphere lesions on both sides cause ataxia in the limbs. Multiple sclerosis is the multiple myelination and glial scar formation of the central nervous system white matter. In addition to cerebellar ataxia, nystagmus, often limb weakness, paralysis, may be accompanied by symptoms of optic neuritis or retrobulbar optic neuritis. It is characterized by multiple lesions and repeated episodes and remissions.

(C) total cerebellar lesions

The balance of the major lesions of the trunk and limb ataxia. Acute damage is more common in acute cerebellitis and pediatric poisoning (such as phenytoin poisoning, barbiturate poisoning, acute mercury, lead poisoning, etc.). The former has a history of infection before the disease, and the acute onset cerebrospinal fluid has increased leukocytosis, often recovered within 2 to 8 weeks. The latter has a history of exposure. Chronic cerebellar lesions are more common in cerebellar degeneration and atrophic lesions. The common type is hereditary ataxia, which is characterized by a family history, progressive onset and progressive, and can have pyramidal and spinal cord posterior bundle damage. symptom. The early manifestation of cerebellar hypoplasia is when the child begins to reach out and pick up the ataxia, sitting, standing, walking late. It is accompanied by other manifestations of mental retardation such as mental retardation, epilepsy and extrapyramidal symptoms.

(4) Cerebral ganglion lesions

The common cause is tumor, which is more common in acoustic neuroma, accounting for 80-90%. Start with sick side tinnitus, progressive hearing loss or vertigo symptoms. Later ipsilateral local sensory disturbance, facial nerve palsy, cerebellar ataxia and increased intracranial pressure appear. In the late stage, due to brain stem and cranial nerve involvement, speech and dysphagia, contralateral pyramidal tract signs and sensory disturbances occur.

(5) Brain stem lesions

The ataxia can be sensory, cerebellum or vestibular, and is common in the cerebellum. The relationship between the brainstem and the cerebellar hemisphere is more than that of the crotch. Therefore, the cerebellar ataxia caused by brain stem damage is marked by the ataxia of the extremities. Because the compensation is not as good as the cerebellar hemisphere, it lasts for a long time. It is characterized by a variety of symptoms associated with brain stem adjacent structures such as motion, sensory conduction bundles, and cranial nerve damage.

(6) Cerebral lesions

The frontal, temporal and parietal lesions of the brain can have cerebellar ataxia clinically.

1. Frontal ataxia: Ataxia of the contralateral limb can occur during frontal lobe lesions, mainly during standing or walking. It is characterized by increased muscle tone, hyperreflexia, positive pathology, and may have psychiatric symptoms and strong grip reflexes. However, the clinical manifestations of decreased muscle tone, decreased sacral reflex or sputum, and no pathological reflex were observed in patients with cerebellar lesions.

2. temporal lobe ataxia: caused by damage to the balance center in the temporal lobe, or secondary to intracranial pressure and high pressure. The characteristic of temporal lobe ataxia is that the symptoms of ataxia are mild, not easy to find in the early stage, and there are symptoms such as unilateral blindness and aphasia.

3. Parietal ataxia: In addition to deep sensory disturbance and cortical sensory disturbance, the parietal lobe is the high center of the cerebellum and vestibule, so the central lobe of the parietal lobe can cause cerebellar ataxia and size. Obstacles.

Third, vestibular ataxia

(a) peripheral vestibular lesions

The lesion of the vestibular to vestibular nerve of the inner ear is called peripheral vestibular lesion. Acute unilateral peripheral lesions such as Meniere's disease, vestibular neuronitis, and various types of labyrinthitis. It is characterized by acute onset, severe vertigo of rotation, nausea, vomiting, and nystagmus (slow phase to the disease side). Can be accompanied by tinnitus, deafness. There are obvious trunk balance obstacles, the tilting during standing and the deflection test are consistent with the direction of the slow phase of the nystagmus. The tilting direction changes with the change of the head position. When the push neck test is pushed from the healthy side to the disease side, it is easy to fall. When the closed eye is walking in a straight line, it is deflected to the side of the disease like the one with a cerebellar hemisphere lesion. The blinking of the eye is a jagged step due to random correction. When the eyes are closed, the center of the body axis is slowly rotated to the healthy side.

Unilateral chronic damage such as auditory neuralgia, persistent otitis media, etc., generally without trunk balance disorders. Dizziness is rare, if there is non-rotation, there is a slight shaking feeling every time when the body is active or closed. Acute bilateral peripheral vestibular lesions such as streptomycin, kanamycin, gentamicin poisoning, both sides of Meniere's disease, etc., standing and walking instability and other balance obstacles are significant, after the eyes closed, but no autologous Spontaneous deflection, rotatory vertigo, but with a sharp shake or floating feeling. The symptoms of bilateral peripheral vestibular lesions such as streptomycin, kanamycin, and gentamicin poisoning are the same as those of acute, but to a lesser extent. Inducing vestibular functional tests are impaired regardless of acute or chronic peripheral vestibular damage.

(2) Central vestibular damage

The vestibular nucleus and its centrally associated lesion are called central vestibular lesions. When seen in a variety of causes of brainstem lesions, it appears to be backward or backward in the standing position, which is inconsistent with the direction of the nystagmus, and has nothing to do with the head position, and is different from the spontaneous skew direction of the body. Therefore, the central vestibular lesion is characterized by inconsistent vestibular responses and mild symptoms. The induced vestibular function test is unobstructed and can be differentiated from peripheral vestibular lesions.

Fourth, hereditary ataxia

(a) spinal cord type

This type of Friedreich's ataxia is the most common. The main lesions are the posterior cord and lateral cord of the spinal cord, the cerebellar tract of the spinal cord and the chronic degeneration of the pyramidal tract, which are mostly insidious onset from 5 to 15 years old, with slow progress. The earliest symptoms are ataxia of the two lower extremities, unstable walking, gait, easy to fall, and the feet are wide when standing, shaking to both sides. It is difficult to close the eyes because of the damage of the conduction beam after the deep cable. Later, there will be ataxia in the upper limbs, which may have intentional tremor, but the upper limb symptoms are often lighter than the lower limbs. There may also be trunk ataxia. When standing or starting, the body is not stable, and the speech is vague or poetic. The limbs are weak. There may be mild mild atrophy of the tibialis anterior and hand muscles, the constriction is obviously reduced, the knee reflex is weakened or disappeared, the muscle tension is underarm, and the pyramidal tract sign is positive. Most patients have nystagmus, often with posterior spine and arched foot, and can have spina bifida, finger (toe) and so on. There is an abnormal ECG in the early stages of the disease, but only 1/3 of the cases have symptoms or signs of heart disease.

(2) Cerebellar type

The Narie type hereditary spastic ataxia is more common. The pathological changes are mainly in the cerebellum, and the symmetry cerebellar atrophy is seen. The Purkinje cells are shed, while the granule cells are less involved. Most of them are insidious after adulthood, and the progress is slow. The ataxia is the first symptom. It shows the intentional tremor of the upper limbs, the ataxia gait and the difficulty of articulation, and the tonic ataxia. The tension of the lower limbs is increased, and the ataxia- gait is formed, which may have hyperreflexia and pathological reflex. A small number of patients are associated with nystagmus and optic atrophy. No skeletal deformities.

(3) Olive bridge cerebral atrophy (olivo-ponto-cerebellar atrophy)

It is a more common type of hereditary ataxia that begins after middle age. Pathological changes involve the lower olive nucleus, the bridge bottom and the cerebellum. Symptoms include limb ataxia, dysarthria, and tremor of the head trunk. A small number of patients may have soft palate. Increased muscle tone, increased sputum reflex and Babinski sign later. There are often nystagmus and optic atrophy. There are still distal sensory disturbances, eye muscle spasms, rigidity, tremors, mental disorders and mental decline.

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