sensory ataxia
Introduction
Introduction Spinal ataxia is caused by deep sensory disturbances caused by posterior root cord lesions in the spinal cord. Vision can be compensated, so the Longbo sign is positive, and the knee test is not stable, accompanied by lower limb position and vibration. Found in spinal cord lesions, peripheral nervous system, Flidry disorders.
Cause
Cause
Causes:
Degenerative and secondary atrophy of nerve cells and posterior root fibers in the spine can cause the disease. The disease is autosomal recessive, and the onset begins in young children or adolescents, and increases with age. It was characterized by slow walking, split legs, swinging left and right, drunk, and positive knee test. The deep sensation of both lower extremities and knee or achilles tendon reflexes weakened or disappeared, and the babinski signs on both sides were positive. Often caused by severe scoliosis or kyphosis and arched foot, advanced degree of sputum and bladder, rectal dysfunction.
Examine
an examination
Related inspection
Finger nose test holding cup test rotation
Clinical manifestations:
It is characterized by slow walking, split legs, swinging left and right, intoxication, positive knee test, deep sensation of both lower extremities and weakened or disappeared knee tendon and Achilles tendon reflex. Both sides of the babinski sign positive.
Often caused by severe scoliosis or kyphosis and arched feet. In the advanced stage, there are different degrees of sputum and bladder and rectal dysfunction. Increased muscle tone, hyperreflexia and pathological reflex. 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.
Diagnosis
Differential diagnosis
Differential diagnosis:
(A) juvenile spinal cord hereditary ataxia: the most common type of hereditary ataxia, usually autosomal recessive inheritance, often accompanied by skeletal deformities in early onset. 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. The nervous system examination found:
1 limb ataxia is dominated by the lower limbs, walking and standing clearly.
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. Auxiliary inspection:
The 1X line plain film has many deformities of the foot and the spine.
2 can have ECG changes such as T wave inversion, conduction block or QRS wave abnormality.
(B) 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.
Auxiliary inspection:
1CT and MRI scans: 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) Hereditary spastic paraplegia: This 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.
(D) ataxia telangiectasia: this disease is a primary immunodeficiency disease involving the nerves, blood vessels, skin, reticuloendothelial system, endocrine and so on. 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. Cerebellar and brain stem atrophy can be seen by gas brain angiography and CT or MRI scan. Brain stem evoked potentials are also helpful in diagnosis.
(6) Cerebellum olive atrophy: This disease, also known as primary cerebellar parenchymal degeneration, is an autosomal dominant inheritance, 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: autosomal recessive inheritance. 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: 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.
Sensory ataxia is characterized by slow walking, split legs, swinging left and right, drunkenness, positive knee test, deep sensation of both lower extremities and weakened or disappeared knee tendon and Achilles tendon reflexes. Both sides of the babinski sign positive. Often caused by severe scoliosis or kyphosis and arched feet. In the advanced stage, there are different degrees of sputum and bladder and rectal dysfunction.
