Clumsy hand rotation
Introduction
Introduction Both hands are awkward because of the awkward clinical symptoms of the hands in the rotation test caused by ADHD, hereditary ataxia polyneuritis, ataxia, and progressive hypertrophic interstitial neuritis. The normal movement of the human body is the balance and coordination of the movement in the cerebral cortex motor area, the basal nucleus of the cortex, the vestibular labyrinth system, the deep sense and the vision, and is called the mutual aid movement. The lesions of these structures lead to coordination disorders called ataxia.
Cause
Cause
(a) cerebellar ataxia
1, cerebellar sacral lesions: common in the cerebellar sacral tumor, children with medulloblastoma, astrocytoma, ependymoma, adult metastases more common.
2, cerebellar hemisphere damage: common in tumors, metastases. Tuberculoma or abscess and vascular disease.
3, the whole cerebellar ataxia: common in cerebellar degeneration and atrophy.
(2) Deep feeling disorder ataxia
1, peripheral neuropathy: common in polyneuritis, lead, arsenic, mercury poisoning, alcoholism, metabolic diseases.
2, posterior root lesions: common in metastatic tumors.
3, posterior cord lesions: common in the spinal cord hernia combined degeneration. Alcoholism, spinal cord compression, etc.
4, thalamic lesions: common in cerebrovascular disease.
5, parietal lesions: common in cerebrovascular disease.
(C) cerebral ataxia
It is common in cerebrovascular diseases, tumors, inflammation, trauma, and degenerative diseases in the frontal, parietal, lobular, occipital, and filthy parts of the brain.
(four) vestibular ataxia
Common in acute labyrinthitis, inner ear hemorrhage, acute lesions of the vestibular nerve or vestibular nucleus.
mechanism
(a) cerebellar ataxia
The cerebellum is located in the posterior cranial fossa, on the dorsal side of the pons and the medulla, and is the fourth ventricle. It is connected to the midbrain, pons, and medulla by three pairs of feet. The cerebellum is called the binding arm, which is mainly composed of the telecentric fibers from the cerebellum. The midbrain part is the bridge arm, which is composed of fibers from the pons nucleus. The cerebellum is mainly a rope-like body composed of fibers from the spinal cord and the medulla into the cerebellum. According to the occurrence of cerebellum, physiological function and fiber connection, the cerebellum is divided into three leaves:
1. The small knot of the pompon: It is the oldest part of the cerebellum. It is called the primitive cerebellum or the ancient cerebellum. It receives the fibers from the vestibular nerve and the vestibular nucleus. It is the integrated center of balance and regulation. When it is damaged, it causes the combination of the trunk and the lower limbs. Disorder.
2, the anterior lobe: in front of the cerebellum, the part before the first fissure, belongs to the old cerebellum in phylogenetics, mainly receives the anterior and posterior bundles of the spinal cerebellum. This bundle transmits deep sensation, its function is to regulate muscle tone and maintain body posture. .
3. Posterior: The part after the first fissure, most of the posterior lobe is a newly-occurring structure called the new cerebellum, which receives cerebellar conduction from the cortical pons, and is mainly involved in the regulation of delicate free movement from the cerebral cortex.
In addition to receiving proprioceptive impulses, the cerebellum also accepts the impulses of external sensation, hearing, vision, and visceral sensation. Therefore, the cerebellum not only affects exercise, but also affects feeling and brain function. Therefore, the most important manifestation of cerebellar lesions is ataxia. When standing, the body leans forward or shakes sideways. When sitting, the trunk is also swaying and unstable. When walking, you can't walk straight, and suddenly the left and right gait is drunk. Finger nose test, finger ear test, grasp test, rotation test, rebound test, knee-high test, intentional tremor, nystagmus may have a positive finding.
(2) Deep feeling disorder ataxia
The deep sensory conduction path is as follows:
Muscle, tendon, joint, peripheral nerve, spinal cord, posterior cord, posterior cord, thin bundle (lower branch), thin bundle nucleus, medullary cross, wedge bundle (upper limb), thin bundle nucleus, thalamic cortical bundle, internal capsule occipital, central posterior Go back to 2/3 and the parietal area.
A deep sense of the conduction path, any part of the damage can occur ataxia. The characteristics are that the ataxia is not obvious when blinking, and the deep eye is obviously enhanced with deep sensory disturbance (joint position sense, vibration party and sports party reduction or disappearance), closed eyes are difficult to stand positive, and wash basin sign is positive. In the early stage, there may be unstable roads, especially in dark places, where the ataxia is obvious. When walking, the foot is thrown forward, and the heel is forced to land (super step) to widen the base of the two feet. When the upper limbs extended and closed their eyes, the two upper limbs consciously fell, and the fingers were in a playing position. Checking the movement of the limbs ataxia is obvious, the knee gum test is not accurate, the finger test of the upper limbs, the finger test is not accurate. The static balance disorder is also obvious. For example, when the supine position is raised, the two feet are lifted up, and the two feet are kept still, and the shaking is unstable, and the eyes are more obvious when the eyes are closed.
(C) cerebral ataxia
Ataxia can occur in the frontal lobe, parietal lobe, lobes, occipital lobe, and abdominal cavity. Frontal lobe ataxia is caused by damage to the frontal pons cerebellar tract. It is characterized by standing or walking. High-level lesions should be considered when there is a disability in the lower extremities. Parietal ataxia is often accompanied by deep sensory disturbances, and the central lobular lesions in the parietal lobe exhibit cerebellar symptoms and urinary dysfunction. The collar leaf ataxia can be accompanied by other signs of the collar leaf.
(four) vestibular ataxia
Mainly based on balance obstacles, it is characterized by balance obstacles during exercise and at rest. May be accompanied by dizziness, nystagmus, vestibular labyrinth symptoms. Mistaken to test positive, closed eyes difficult to sign positive. This type of ataxia is shaken after a period of time after closing the eye, and gradually increases, and the direction of the dump is consistent with the direction of the eye movement. Found in acute labyrinthitis, inner ear hemorrhage, acute lesions of the vestibular nerve or vestibular nucleus.
Examine
an examination
Related inspection
Brain CT examination of brain MRI
First, medical history
1, ataxia: pay attention to the onset of disease and disease course, general acute onset of ataxia and episodes, vestibular system lesions and vertigo epilepsy is more likely. The onset is more urgent, and those who deteriorate in a short period of time are more likely to have acute cerebellar lesions, central nervous system inflammation and brain trauma after treatment. Patients with more acute onset and rapid deterioration, sometimes life-threatening cerebrovascular disease, brain trauma, especially cerebellar hemorrhage. Alcoholism and vitamin deficiency-induced ataxia can improve ataxia after improving nutritional status. Arrhythmia with remission and recurrence is more common with multiple sclerosis.
2, age and family history: There is a great reference in the diagnosis of ataxia. Childhood is congenital cerebellar hypoplasia, hereditary diseases, childhood acute cerebellar ataxia, encephalitis and so on. Adolescent onset can be seen in juvenile spinal cord hereditary ataxia, hereditary ataxia, polyneuritis, osteomuscular atrophy, hypertrophic interstitial neuropathy, syringomyelia. Young and healthy people can be seen in dentate nucleus red atrophy, olive bridge cerebral degeneration, subacute combined degeneration, telangiectasia and ataxia. Middle-aged and elderly people are more common in cerebellar atrophy, vertebral-basal artery insufficiency, cerebellar hemorrhage, cerebrovascular disease and so on. Some of the ataxia disorders include genetic factors such as congenital cerebellar hypoplasia, childhood acute cerebellar ataxia, and juvenile spinal cord hereditary ataxia. Hereditary ataxia polyneuritis vertebral muscle atrophy ataxia, large interstitial neuropathy, dentate nucleus redness atrophy, olive bridge cerebellar degeneration, telangiectasia ataxia.
Second, physical examination
Correct and free exercise requires a lot of muscles, including active muscle, synergistic muscle, orange anti-muscle and fixed muscle to complete.
1, finger nose test: patients first stretch the upper extremity, then use the tip of the index finger to touch the tip of the nose, repeated in different directions, speed, blinking, closed eyes, and contrast on both sides. In the case of ataxia, the behavior is light and heavy, and the speed is different. If you misunderstand or adjust, you can target 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 eye-opening movement is barrier-free, but when the eyes are closed, there is a clear ataxia.
2. Test with the knee gallbladder: The patient is supine, and the following three actions are performed in sequence: one side of the lower limb is lifted and straightened, and the heel of the raised side is placed on the knee of the lower limb of the contralateral side, and then the heel is placed Sliding down the leading edge of the winning bone, and strive for accurate coherence of movement. Cerebellar damage caused by poor positioning and intentional tremor when lifting the leg and touching the knee, often swaying when moving down; when the sensory ataxia occurs, the patient's heel often cannot find the knee, and when moving down, the swing is uncertain and often cannot and The femur remains in contact.
3. Fast rotation test: Quickly pat the opposite side of the hand with one hand; or the forearm quickly rotates the front and back, or the palm and the back of the hand alternately touch the table; when the cerebellum is damaged, the above action is clumsy, rhythm Uneven.
4, rebound test: the patient closed his eyes, one side of the upper limbs forcefully clenched the fist flexion, the doctor suddenly forced the process to pull it apart, the normal fragile protection action, will not touch themselves, cerebellar lesions. Because of the control of the active muscle and orange The poor coordination of anti-muscle often leads to over-action and attacks on yourself. Or maintain the posture of the arms extending forward. The examiner suddenly pushes down its arms separately or simultaneously, and then releases, and the normal person can accurately return to the original position. Patients with cerebellar ataxia do not normally control the coordination of the agonist muscle and the orange-anti-muscle, often causing excessive movement and excessive swinging time. When examining the lower limbs, the calf can be pushed while the patient maintains a 90o bend of the knee, and the meaning is the same as above.
5. Over-finger test: The upper limb of the patient is stretched forward, and the finger is placed on the finger that the examiner is fixed. Then the patient lifts the hand to the vertical position and then descends to the examiner's finger. Always keep your upper limbs straight. Close your eye and check your eyes. When the vestibular ataxia occurs, the lower limbs tend to be lost to the side with the lesion; when the sensory ataxia occurs, the examiner's fingers are often not found when the eyes are closed, but the brain is not fixed in the direction of the skew. In the case of dysregulation, generally only the upper limb is deflected to the outside.
6, toe-finger test: The patient is supine, lifting the big toe to touch the fingers of the lake, the latter often change position, requiring patients to track accurately.
7. Sit-up test: The patient is supine, the two hands are placed on the chest and are not supported and sit up. The normal person can only press the lower limbs and flexure without leaving the bed surface. The pith and the trunk of the cerebellar lesion are flexed at the same time. Lift up, called the joint flexion sign.
Third, auxiliary inspection
1, cerebellar ataxia: brain CT or MRI should be examined to exclude cerebellar tumors, metastases, tuberculoma or abscess and vascular disease and cerebellar degeneration and atrophy.
2, deep sensory ataxia: If the localized lesion is located in the peripheral nerve, the EMG and somatosensory evoked potential should be examined; if considering the posterior root lesion or the posterior cord lesion, the EMG of the EMG, the evoked potential, and the lesion should be examined. Cerebrospinal fluid examination, or myelography. It is best to check brain CT or MRI when considering the thalamus or parietal lobe.
3, cerebral ataxia: cerebrovascular disease, tumor, inflammation, trauma, degenerative diseases, etc., should check brain CT or MRI, EEG and so on.
4, vestibular ataxia: can be examined electrical audiometry, auditory evoked potentials, vestibular function tests.
Diagnosis
Differential diagnosis
(A) juvenile myeloid hereditary ataxia (Friedreich blood for ataxia)
The most common type of hereditary ataxia is usually autosomal recessive, with early onset often accompanied by skeletal deformities. The lesions involved the spinal cord cerebellar tract and corticospinal tract in the spinal cord and lateral cord, and the cerebral cerebral anterior bundle was less involved. Demyelination of the nerve fibers and axonal rupture, the cells of the Clarke column disappeared, and the gliosis proliferated.
1, clinical manifestations: more than 5-18 years old, the average age of 12-13 years old, no difference in gender. Gradually onset, slow development, the earliest symptoms of gait is unstable, gait is registered, the body shakes when standing, and the drunk is like a gait. Closed eyes are difficult to sign positive. The muscle tension is low, the knee tendon reflex disappears, and the disease is microscopically caused by cone beam damage. The condition progresses gradually. The upper limbs are inflexible and clumsy, intentional tremors, cerebellar dysarthria, and speech is ambiguous. The position of the lower limbs and the shaking party disappeared. The nervous system examination found that: 1 limb ataxia is dominated by the lower limbs, walking and standing are obvious. 2 Most patients have nystagmus, horizontal nystagmus is common, but verticality and rotation are visible, usually the most obvious when gazing outward. 3 limb muscle tension is reduced, the lower limbs are obvious, and pathological reflex occurs when the pyramidal tract is damaged. 4 The sensory disturbance is not obvious, and the tremor can be affected. 5 a small number of patients may have primary optic atrophy.
2, auxiliary examination: 1X line flat film has more deformities of the foot and spine. 290% of patients had ECG changes such as T wave inversion, conduction block or QRS wave abnormalities.
3, diagnosis: The diagnosis of this disease is a slow occurrence of adolescent and progressive ataxia, dysarthria, knee reflexes disappear, pathological reflexes, deep sensory disturbances, skeletal malformations, cardiac signs, autosomal recessive inheritance.
(2) hereditary spastic ataxia
Also known as hereditary cerebellar ataxia. Usually autosomal dominant, mostly in adult onset, accompanied by increased muscle tone and hyperreflexia. Mainly damage the cerebellum, Purkinje cells mostly disappear, white matter demyelination, axonal degeneration. Lesions can affect the pons, medulla, olive nucleus, spinal cord, optic nerve and so on.
1. Clinical manifestations: Most of them start from 25-55 years old. First, there is a slow progress of gait instability, which is easy to fall. It can be accompanied by a gait or a gait. Later, the upper limbs were also affected, and the hands were clumsy and intentional tremors, so that the fine movements could not be completed, the dysarthria could be completed, and the speech could have an outbreak language. Pyramidal tract signs appear in the lower extremities, such as increased muscle tone, hyperreflexia and pathological reflexes. Many patients are associated with optic atrophy, retinal degeneration, extraocular muscle activity disorder, and drooping eyelids. Ocular tremor may appear very late, without skeletal deformities.
2, auxiliary examination: 1CT and MRI scan: cerebellum and brain stem atrophy. 2 gas cerebral angiography: see the subarachnoid space and cerebellum under the air increased, suggesting that the cerebellum and brain stem atrophy.
3, diagnosis of hereditary ataxia diagnosis is adult onset, slow onset and slow progression of ataxia, lower limbs with pyramidal tract signs, CT and MRI scan can be seen in cerebellar atrophy, autosomal dominant inheritance.
(c) hereditary spastic paraplegia
The disease is a type of hereditary ataxia, which is an autosomal dominant inheritance. Mainly the axonal degeneration and demyelination of the bilateral corticospinal tract in the spinal cord, with the largest thoracic segment. Spinal cord cerebellar bundles, thin bundles, anterior horns, giant pyramidal cells, basal ganglia, brainstem, cerebellum, optic nerve, etc. may also be altered.
1, clinical manifestations: more than 10 years old onset or a few 20-30 years old onset, the earliest stiff and inflexible legs, lower limb muscle rigidity and ankle joint flexor weakness and scissors gait. Due to the weakness and paralysis of the flexor of the medullary joint, the sick child felt difficult to go upstairs. The examination revealed that the lower extremities had high muscle tension, weakened muscles, hyperreflexia of the knee, positive pathological reflex, and no sensory disturbance. The onset of the disease progressed slowly, and the upper limbs were also affected, resulting in a lighter pyramidal sign. Involved in medulla oblongata, dysphagia, and strong crying and strong laughter. In the advanced stage, there may be mild dysfunction of sphincter function. There may be primary optic atrophy and retinitis pigmentosa.
2, diagnosis: childhood onset, slow progress of lower limb pyramidal tract sign, scissors gait, mild coordination disorder, with a clear family history.
3, special type 1 hereditary spastic paraplegia with ocular and extrapyramidal symptoms (Ferguson-Critchley syndrome): manifested in the limbs of the cone, the eye symptoms are mainly nystagmus, lateral and vertical fixation restricted, false Sexual ball paralysis. Extrapyramidal lesions show tough limbs, involuntary movements, facial expressions, and may have a forward gait. 2Kjellin syndrome: spastic paraplegia begins to occur around the age of 25, and small muscles in both hands and legs undergo progressive atrophy, decreased intelligence, and central retinal degeneration. 3Troyer syndrome: early onset in children, spastic paraplegia with distal muscle atrophy, short stature, unable to walk until 20-30 years old, a small number of patients involuntarily crying, dysarthria. 4Mast syndrome: 11-20 years old, mainly with spastic paraplegia and Alzheimer's disease. 5Sjugren-larsson syndrome: spastic paraplegia, congenital ichthyosis, mental retardation.
(4) Ataxia telangiectasia
The disease is a primary immunodeficiency disease involving the nerves, blood vessels, skin, reticuloendothelial system, endocrine, and the like. It is autosomal recessive, and this effect is lost due to thymic dysplasia in children. The main pathological changes were diffuse cerebellar cortical atrophy, markedly reduced cells, and thin bundles of spinal cord and demyelination of the spinal cerebellar tract. The thymus is significantly reduced or missing.
1. Clinical manifestations: The children's gait is obvious and the legs are wide. Intentional tremors appear in the upper limbs. Different from juvenile myeloid hereditary ataxia, there is no sensory disorder, and closed eyes are difficult to sign negative. Most children are associated with acromegaly, and extra-pyramidal hyperactivity is more variable with age. The eyeball actively moves to the two sides in the same direction slowly and intermittently, often accompanied by blinking and head swinging, nystagmus when the movement is terminated, and cerebellar dysarthria. After puberty, most patients developed symptoms of spinal cord injury, deep feeling disappeared, and pathological signs were positive. Capillary vasodilation usually occurs at 3-6 years of age and occurs in the exposed area of the bulbar conjunctiva. It affects all conjunctiva, eyelids, bridge of the nose and cheeks, neck, elbow fossa and armpits with age. Early onset changes in skin and hair are evident. The subcutaneous fat in infancy disappears very early, and the facial skin often shrinks and sticks to the facial bone. Can be associated with chronic seborrheic gingivitis and phlegm dermatitis, punctate pigmentation and hypopigmentation, repeated respiratory infections are one of the prominent symptoms of this disease. After rhinitis, sinusitis, chronic bronchitis, pneumonia, can cause extensive fibrosis of the lungs for a long time, and the occurrence of finger-like fingers and pulmonary insufficiency. Almost all of the sick children have sexual developmental disorders, and usually do not have the first sexual characteristics. About three-quarters of patients have dwarfism.
2, auxiliary examination X-ray film: often can find all the symptoms of sinusitis and chronic bronchitis and pneumonia, sometimes visible malignant lymphoma caused by widening of the mediastinal shadow. Most of the electrocardiogram is normal, the selectivity of immunoglobulins IgA and IgE in serum is lacking, and lymphocytes in the surrounding blood are reduced. Alpha-fetoprotein is significantly elevated, reflecting liver dysplasia. The main change in chromosome examination is t(14q+, 14q-), which is the shift of homologous chromosome 14, and also the chromosome 14 and the 7th, 8th or X chromosome translocation.
3. Diagnosis: Ataxia in infancy, telangiectasia in 3-6 years old, somatic growth, and premature aging of skin. Serum IgA and IgE were significantly reduced. Serum alpha-fetoprotein is elevated. X-ray lateral radiographs showed reduced or absent lymphoid tissue in the nasopharynx.
(5) stimulating body bridge cerebellar atrophy (OPCA)
The disease is divided into two types, hereditary and sporadic cases. There are many types of clinical, and the Meniel type is the most common and most typical of heredity.
The disease is autosomal dominant and recessive, and the former is more. The pathological changes were mainly in the olive body, the basal ganglia of the pons, and the cerebellar hemisphere. The cells disappeared obviously, and the nerve fibers were significantly demyelinated. Spinal cord and spinal cord cerebellar tract are also involved, and facial nucleus, hypoglossal nucleus, red nucleus, substantia nigra, basal ganglia, cerebral cortex, and anterior horn of the spinal cord are also damaged.
1. Clinical manifestations: Hereditary ataxia for middle-aged onset. It started to be difficult for cerebellum to walk, and later affected the upper limbs and showed dysarthria. Static tremors of the head and torso can sometimes occur. Usually no nystagmus, normal muscle strength and reflex, intentional tremor, poor discrimination. There are involuntary movements such as dance movements, hand and foot movements, and tremor paralysis syndrome. Some patients have nuclear or nuclear ophthalmoplegia, optic atrophy, retinitis pigmentosa, nystagmus is rare, pathological reflex, deep sensory disturbance, urinary incontinence. A few have dementia.
2, auxiliary examination: cerebellar angiography and CT or MRI scan can be seen in the cerebellum and brain stem atrophy, MRI examination is better than CT. Brain stem evoked potentials are also helpful in diagnosis.
3. Diagnosis According to clinical manifestations, progressive cerebellar ataxia occurring after adulthood, with extrapyramidal signs, ocular symptoms, spinal cord symptoms, positive family history, combined with CT and MRI diagnosis is not difficult.
(6) Cerebellum Huilan shrinking
The disease, also known as primary cerebellar parenchymal degeneration, is autosomal dominant, and a small number of patients are autosomal recessive. Pathological changes in the cerebellar cortex, Purkinje cells disappeared. Degeneration can be extended to other nuclei of the cerebellum to the cerebellum to effervescent fibers, degeneration retrograde to the olive nucleus, the latter atrophy, gliosis, and demyelination of the fibers between the olive nucleus.
