What Is Sensory Ataxia?

Spinal ataxia. Caused by deep sensory impairment caused by posterior root posterior cord injury of the spinal cord. Vision can be compensated, so Romberg's sign is positive, and the test of the knee and tibia is unstable, and it is accompanied by disturbance of lower limb position and vibration. Found in spinal cord disease, peripheral neurosis, Friedrich's disorder.

Sensory ataxia

Spinal ataxia. Caused by deep sensory impairment caused by posterior root posterior cord injury of the spinal cord. Vision can be compensated, so Romberg's sign is positive, and the test of the knee and tibia is unstable, and it is accompanied by disturbance of lower limb position and vibration. Found in spinal cord disease, peripheral neurosis, Friedrich's disorder.

What is the cause of sensory ataxia?

Degenerative, secondary atrophy of nerve cells and posterior root fibers in the spine can cause this disease. The disease is an autosomal recessive inheritance, and the onset usually begins in infancy or adolescence, and it increases with age.

How to diagnose sensory ataxia

It is characterized by slow walking, legs spread, swinging from side to side, faltering, positive heel and tibia test, and weakening or disappearing of deep feeling of both lower limbs and knee tendon and Achilles tendon reflex. Babinski sign was positive on both sides. Often causes severe scoliosis or kyphosis and arched feet. There are different degrees of paralysis and bladder and rectal dysfunction in the late stage.

Sensory ataxiaSensory ataxia is easily confused with what symptoms

(1) Adolescent spinal cord hereditary ataxia: It is the most common type of hereditary ataxia, usually with autosomal recessive inheritance, and early onset is often accompanied by skeletal deformities. Clinical manifestations: Onset in adolescence, slow development, earliest symptoms of unstable gait, staggering gait, shaking body when standing, drunk like gait. Closed eyes are difficult to establish a positive sign. Muscle tension is low and knee-ankle reflexes disappear. The condition gradually progressed. The movements of the upper limbs were inflexible and awkward, intentional tremor, cerebellar dysphonia, and blurred speech. The sense of position and vibration of the lower limbs disappeared. Nervous system examination found that: limb ataxia was mainly in the lower limbs, and walking and standing were obvious. Most patients have nystagmus, and horizontal nystagmus is more common, but verticality and rotation can be seen, usually the most obvious when staring outward. The muscle tension of the limbs is reduced, the lower limbs are obvious, and pathological reflexes occur when the pyramidal tract is damaged. The sensory disturbance is not obvious, and the tremor can be affected. A few patients may have primary optic nerve atrophy. Auxiliary examination: Plain radiographs often have deformities of the feet and spine. There may be changes in the electrocardiogram such as T wave inversion, conduction block or QRS wave abnormality.

(2) Hereditary spastic ataxia: Also called hereditary cerebellar ataxia. It is usually autosomal dominant, and most of them start in adulthood, accompanied by increased muscle tone and hyperkinesis. Clinical manifestations: Slowly progressing gait instability first, easy fall, may appear to be a gait or convulsive gait. Later, the upper limbs were also affected, with clumsy hands and intentional tremor that could not complete fine movements, dysphonia, and violent speech in speech. There are pyramidal signs in the lower limbs, such as increased muscle tone, hyperfemoral reflexes, and pathological reflexes. Many patients are accompanied by optic nerve atrophy, retinal degeneration, extraocular muscle dysfunction, and drooping eyelids. Nystagmus may appear very late, without skeletal deformities.

Auxiliary examination: CT and MRI scans: cerebellum and brain stem atrophy. Pneumoencephalography: Seeing increased gas in the subarachnoid space and the cerebellum, suggesting that the cerebellum and brainstem atrophy.

(Three) hereditary spastic paraplegia: This disease is a type of hereditary ataxia, which is an autosomal dominant inheritance. Clinical manifestations: Earliest scissor gait for stiff and inflexible legs, stiffness in lower limb muscles and weakness in ankle dorsiflexus. Due to the weakness and cramps of the flexor muscles of the medullary joint, the sick child felt difficulty in going upstairs. Examination revealed that the lower limbs had high muscle tension, weakened muscle strength, hyperreflexia of the knee and ankle, positive pathological reflex, and no sensory disturbance. The disease progressed slowly, and later the upper limbs were also affected, with a lighter pyramidal sign. Spastic dysarthria occurs when the medulla is involved, difficulty swallowing, and crying and laughing. Slight dysfunction of sphincter function may occur in advanced stage. May have primary optic nerve atrophy and retinal pigment degeneration.

(D) Ataxia telangiectasia: This disease is a primary immunodeficiency disease involving nerves, blood vessels, skin, reticular endothelial system, and endocrine. Genus Autosomal Recessive. Clinical manifestations: The child's gait is obvious, and his legs are wide. Then intentional tremor appeared in the upper limbs. Unlike juvenile spinal cord genetic ataxia, there is no sensory disturbance, and it is difficult to close the eyes with negative signs. Most children are accompanied by hand, foot and asthma. As the age increases, the extrapyramidal ADHD becomes more obvious. The movement of the eyeballs to the sides in the same direction is slow and intermittent, often accompanied by blinks and head swings. At the end of the movement, nystagmus and cerebellar articulation disorders occur. After puberty, most patients develop symptoms of spinal cord injury, deep sensation disappear, and pathological signs are positive. Capillary telangiectasis occurs in the exposed area of the bulbar conjunctiva, which affects all conjunctiva, eyelids, nasal bridge, and ears, neck, elbow, and armpits with age. Early onset changes in skin and hair are noticeable. Subcutaneous fat disappears early in infancy, and the facial skin often shrinks and clings to the bones. May be accompanied by chronic seborrheic dermatitis, punctate pigmentation and hypopigmentation, and repeated respiratory infections are one of the prominent symptoms of this disease. After rhinitis, sinusitis, chronic bronchitis, and pneumonia can cause extensive fibrosis in the lungs for a long time, and clubbing fingers and pulmonary insufficiency occur. Almost all the sick children have sexual dysfunction and usually do not have secondary sexual characteristics. About three-quarters of the patients have dwarfism. X-ray films can often find all the symptoms of paranasal sinusitis and chronic bronchitis and pneumonia, and sometimes malignant lymphoma can cause enlarged mediastinal shadows. Electrocardiograms are mostly normal, the selectivity of immunoglobulins IgA and IgE in serum is lacking, and lymphocytes in peripheral blood are reduced. Alpha-fetoprotein is significantly elevated, reflecting liver dysplasia. Chromosomal abnormalities.

(E) Olive Cerebellar Atrophy (OPCA)

The disease is divided into two types of hereditary and sporadic cases, and there are many types of clinical, Meniel type is the most common and most typical of hereditary. The disease is autosomal dominant and recessive, the former is more. The clinical manifestations are hereditary ataxia of middle-aged onset. It started with cerebellar difficulty walking, and later affected the upper limbs and presented with dysarthria. Sometimes quivering of the head and trunk can occur. Usually no nystagmus, normal muscle strength and reflexes, intentional tremor, poor range discrimination. Involuntary movements such as dance movements, hand and foot movements, tremor paralysis syndrome. Some patients have supranuclear or nuclear ophthalmoplegia, optic nerve atrophy, retinal pigment degeneration, nystagmus are rare, pathological reflexes, deep sensory disturbances, and urinary incontinence. A few have dementia. Pneumoencephalography and CT or MRI scans show atrophy of the cerebellum and brainstem. Brainstem evoked potentials are also helpful in diagnosis.

(6) Cerebellar olive atrophy: This disease is also known as primary cerebellar parenchymal degeneration. It is an autosomal dominant inheritance. A few patients have an autosomal recessive inheritance. Early clinical manifestations of gait instability, staggered walking, two feet separated. Later, it affects the fine movements of the hand, the handwriting becomes bad, the speech stutters, or there is a bard language. Low muscle tone, intentional tremor, finger and nose, knee and tibia tests are not allowed. In some cases, nystagmus appeared later. Bladder sphincter disorders are also common. A few patients have decreased intelligence, normal vision, and no sensory disturbances. Air-brain angiography, CT, or MRI showed a widening of the vermiform sulcus and normal fourth ventricle.

(7) Myoclonic cerebellar coordination disorder: autosomal recessive inheritance. Also called "dentate nucleus atrophy." Clinical manifestations of myoclonus, cerebellar dysfunction, with or without epilepsy. It can start with directional tremor of a limb, dysarthria, poor discrimination, and inability to rotate. Limb ataxia is more pronounced than trunk ataxia. The upper limbs are heavier than the lower limbs. In severe cases, fluttering tremors appear when both hands are straight forward.

(8) Hereditary ataxia-cataract-dwarf-mental retardation syndrome: It is a rare genetic disease. Most are autosomal recessive. Patients with clinical manifestations after birth or infancy are called infants, and adults with onset of disease are called adult. There are three characteristic symptoms of this disease: cataract, cerebellar ataxia, and mental retardation. Cataracts are bilateral. Cerebellar dysfunction is manifested as articulation disorders, ataxia of the trunk and limbs, nystagmus, and low muscle tone. Older children often have a positive cone tract sign. Stunted sexual function, foot eversion, scoliosis, finger (toe) deformity, etc.

How to prevent sensory ataxia

Ataxia currently has no specific therapy. In addition to general supportive therapy, acupuncture can be used for treatment, physical therapy and limb function training. Various B vitamins, citicoline, intramuscular injection, oral lecithin, etc. Advanced patients should pay attention to prevent various infections.