Ataxia
Introduction
Introduction to ataxia The anterior horn cells of the spinal cord receive cerebral cortex, cerebral cortex, cerebral cortex, cerebellum, vestibular labyrinth system, deep sense and other regulation and control of the upward and downward conduction beam, so that the human body maintains a certain posture to properly perform random movement and maintain balance. A lesion occurs in the above part, resulting in a disorder of coordination, called ataxia. Ataxia mainly reflects damage to the cerebellar hemisphere and its relationship with the contralateral frontal cortex, but may also occur in other parts of the lesion, such as acute labyrinth edema produces vestibular ataxia, accompanied by vertigo. Deep sensory disturbances produce sensory ataxia. Involving the limbs, trunk and throat muscles can cause posture, gait and language barriers. basic knowledge The proportion of illness: the incidence rate is about 0.003%-0.005%, more common in alcoholics Susceptible people: no specific population Mode of infection: non-infectious Complications: cervical spondylotic myelopathy
Cause
Cause of ataxia
Causes:
Ataxia is caused by many causes of various parts of the nervous system. Any simple movement must be completed with the participation of the four muscles of the agonist, antagonistic muscle, synergistic muscle and fixed muscle, and depends on the coordination and balance of the nervous system. There are many causes of ataxia. First, you must determine which nature it belongs to, and then consider the various causes. Therefore, deep sensation, vestibular system, cerebellum and brain damage can occur ataxia, called sensory, vestibular, cerebellum and cerebral ataxia, as well as unexplained factors, some with intelligence insufficiency Or dementia.
The coordination and balance of the nervous system includes:
1. Sensory deep feeling reflects the position and direction of movement of various parts of the body to the central nervous system. The causes are:
1 peripheral nerve or radiculopathy;
2 subacute combined degeneration of the spinal cord, skull base malformation, myelopathy, tumor;
3 brain stem vascular diseases such as infarction, hemorrhage, multiple sclerosis, tumor;
4 thalamic parietal pathway or parietal vascular disease, tumor.
2. The vestibular vestibular system conducts balance information to the heart, causing an equilibrium response such as body position, line of sight adjustment, and spatial positioning sensation. The causes are:
1 labyrinthitis, vestibular neuritis, idiopathic bilateral vestibular disease;
2 vertebral-basal artery stenosis or occlusion;
3 days under the curtain tumor.
3. The cerebellum of the cerebellum is the regulating center of exercise. The function of these structures is all done under the unified control of the cerebral cortex. The causes are:
1 hereditary
2 primary or metastatic tumors;
3 vascular such as infarction, bleeding;
4 inflammatory such as acute cerebellitis, abscess;
5 poisoning such as alcohol, food, drugs, harmful gases, etc.;
6 demyelinating;
7 underdeveloped or poor;
8 hereditary;
9 trauma;
10 calcification;
(11) Malformation.
4. The frontal lobe lesion is in the front of the frontal lobe, and the movement disorder is the contralateral side. The causes are:
1 tumor;
2 inflammation;
3 vascular disease.
Prevention
Ataxia prevention
Pay more attention to your diet and pay attention to your daily life. If similar symptoms occur, seek medical attention as soon as possible. Pay attention to proper rest, do not overwork, master the combination of movement and static.
Complication
Ataxia complications Complications Cervical spondylosis spastic cerebral palsy
Cervical spondylotic myelopathy, spastic cerebral palsy.
Symptom
Ataxia symptoms common symptoms uppercase gait instability sensation ataxia after circulation ischemic cerebellar ataxia sensory disturbance limb movement incoordination strong grip reflex intentional tremor vestibular ataxia
1. Cerebellar ataxia (cerebellar: ataxia): the speed, rhythm, amplitude and strength of the random movement, that is, coordination of movement disorders, may also be associated with decreased muscle tone, eye movement disorders and speech disorders.
(1) Postural and gait changes: ankle lesions cause tonic ataxia, unstable standing, gait squatting, walking away from the feet when walking, shaking, and even difficult to sit stable, the upper jaw is damaged forward When dumped, the lower jaw is damaged and tilted backwards. The ataxia of the upper limb is not obvious. The cerebellar hemisphere lesions are skewed or dumped to the affected side when walking.
(2) Incoordination of involuntary movement: cerebellar hemisphere damage leads to ataxia of ipsilateral limbs, manifesting dysmetria and intentional tremor, upper limbs are heavier, and the more the action is closer to the target, the tremor Obviously, the eyeballs are visible to the lesion side, and the large nystagmus is visible. The upper limbs and the hands are the most serious, and the coordination and fine movements cannot be completed. The synergy can not be achieved (asynergy), the quick recovery and the rotation movement are abnormal, and the writing is getting bigger and bigger (upper case disease).
(3) Speech disorder: due to the vocal imbalance of the articulated organs such as the lips, tongue, and throat, the speech is slow, ambiguous, and the sound is intermittent, frustrated or explosive, showing a poetic or fulminant language.
(4) ocular dyskinesia: large ataxia nystagmus occurs in the ataxia of the eye movement muscle, especially when the vestibular contact is involved, the eyes swing back and forth, and even the down-beat nystagmus, the rebound eye Shock and so on.
(5) Reduced muscle tone: visible pendulum-like sputum reflex, seen in acute cerebellar lesions, when the patient's forearm resists resistance contraction, such as sudden withdrawal of external force can not immediately stop contraction, may hit your chest (rebound phenomenon).
2. Cerebral ataxia: The frontal bridge bundle and the sacral occipital bundle are the fibers associated with the brain's frontal, temporal, occipital and cerebellar hemispheres. The lesions can cause ataxia, with mild symptoms and less associated with nystagmus.
(1) frontal lobe ataxia: seen in the frontal lobe or frontal bridge cerebellar stenosis, similar to cerebellar ataxia, such as postural balance disorder, gait instability, backward or sideways, contralateral limbs Arrhythmia, increased muscle tone, hyperreflexia and pathological signs, with frontal lobe symptoms such as mental symptoms, strong grip reflexes. : (2) parietal ataxia: different degrees of ataxia in the contralateral limbs, obvious when the eyes are closed, deep sensory disturbances are not obvious or transient, bilateral lower lobe posterior lateral lesions appear double lower limbs Sexual ataxia and urinary dysfunction.
(3) temporal lobe ataxia: lighter, showing transient balance disorder, not easy to find early.
3. Sensory ataxia: For the spinal cord injury, the patient can not distinguish the position of the limb and the direction of movement, and the sensory ataxia is unstable, such as standing, not knowing the distance, the depth of the foot, the feeling of cotton, often visually the ground. Walking, it is difficult to walk in the dark, check for vibration, joint position loss and closed eyes (Romberg) sign positive.
4. Vestibular ataxia : vestibular lesions cause spatial dysfunction, mainly due to balance disorders, unstable standing, dumping to the disease side while walking, unable to walk in a straight line, changing the symptoms of the head, and the normal movement of the limbs Often accompanied by severe dizziness, vomiting and nystagmus, vestibular function test inner ear temperature change (hot and cold water) test or rotation test reaction decreased or disappeared, the lesion is closer to the inner ear lost, the more common ataxia.
Examine
Ataxia check
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.Diagnosis
Ataxia diagnosis and identification
diagnosis
Diagnosis is based on medical history, clinical manifestations, and examinations.
Differential diagnosis
(a) juvenile myeloid hereditary ataxia
The most common type of hereditary ataxia is usually autosomal recessive, with early onset often accompanied by skeletal deformities. Clinical manifestations: adolescent onset, slow development, the first symptoms of gait is unstable, gait squat, body shaking when standing, drunk like gait. Closed eyes are difficult to sign positive. The muscle tension is low and the knee reflex disappears. The condition progresses gradually. The upper limbs are inflexible and clumsy, intentional tremors, cerebellar dysarthria, and speech is ambiguous. The positional sense of the lower limbs and the sense of vibration disappear.
(2) hereditary spastic ataxia
Also known as hereditary cerebellar ataxia. Usually autosomal dominant, mostly in adult onset, accompanied by increased muscle tone and hyperreflexia. Clinical manifestations: First, there is a slow progression of gait instability, easy to fall, can be a gait or a combined 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.
(c) hereditary spastic paraplegia
The disease is a type of hereditary ataxia, which is an autosomal dominant inheritance. Clinical manifestations: Scab gait appeared at the earliest stiffness and inflexibility of the legs, weakness of the lower extremities and weakness of the ankle joints. 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.
(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. Clinical manifestations: The child'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. Telangiectasia occurs in the exposed area of the bulbar conjunctiva and affects all conjunctiva, eyelids, bridge of the nose and cheeks, neck, elbow fossa and armpits as they 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. It can be accompanied by chronic seborrheic dermatitis, punctate pigmentation and hypopigmentation. Repeated respiratory infection is one of the prominent symptoms of this disease. After rhinitis, sinusitis, chronic bronchitis, pneumonia, long-term pulmonary fibrosis, clubbing and pulmonary insufficiency. Almost all of the sick children have sexual developmental disorders, and usually do not have secondary sexual characteristics. About three-quarters of patients have dwarfism. X-ray films can often be found in all cases of paranasal sinusitis and chronic bronchitis and pneumonia, sometimes seen in malignant lymphoma caused by widening of 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. Abnormal chromosomal examination.
(5) Olive 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 clinical manifestations are hereditary ataxia with 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.
(6) Cerebellar olive atrophy
The disease, also known as primary cerebellar parenchymal degeneration, is autosomal dominant, and a small number of patients are autosomal recessive. In the early stage of clinical manifestation, the gait was unstable, walking and squatting, and the two feet were separated. In the future, the fine movements of the hands will be affected, the writing will be bad, the speech will be stuttering, or there will be a poetic language. Low muscle tone, intentional tremor, finger nose and knee squat test is not allowed. Ocular tremor occurred in some cases later. Bladder sphincter disorders are also more common, a small number of patients with mental decline, normal vision, no sensory disturbance. Gas cerebral angiography, CT or MRI showed widening of the sulcus and normal ventricle.
(7) Myoclonic cerebellar coordination disorder
It is autosomal recessive. Also known as "dental nucleus red core atrophy." Clinical manifestations of myoclonus, cerebellar dysfunction, with or without epileptic seizures. It can be a directional tremor of a limb, a dysarthria, a poorly resolved distance, and a rotational movement. Limb ataxia is more pronounced than torso ataxia. The upper limbs are heavier than the lower limbs, and in severe cases, the two hands are stretched forward when they are fluttering.
(8) Hereditary ataxia-cataract-a gnome-intellectual deficit syndrome
It is a rare genetic disease. Mostly autosomal recessive inheritance. Clinical manifestations of symptoms after birth or infancy are called infants. Adults are called adult type. There are three characteristic symptoms of this disease: cataract, cerebellar ataxia, and mental retardation. Cataracts are bilateral. Cerebellar dysfunction is characterized by dysarthria, torso and limb ataxia, nystagmus, and low muscle tone. Older children often have positive pyramidal tract signs. Sexual function developmental delay, foot valgus, posterior scoliosis, finger (toe) deformity, etc.
