Segmental dissociative sensory disorder

Introduction Syringomyelia is often characterized by segmental dissociative sensory disturbances. Due to a variety of reasons, a tubular cavity is formed in the spinal cord, called syringomyelia, and there is often gliosis around the cavity. The incidence of this disease is relatively slow, the clinical manifestations of the affected spinal cord segmental nerve damage symptoms, characterized by pain, temperature loss and disappearance, and deep sensory preservation of the sensory disturbance, combined with dyskinesia and nerve damage Nutritional disorders.

Cause

The exact cause is still unclear and can be divided into congenital dysplasia and secondary syringomyelia, the latter being rare.

1. Congenital spinal canal insufficiency: This disease is often accompanied by other congenital anomalies such as spina bifida, cervical ribs, scoliosis, and occipital deformity.

2. Abnormal blood circulation of the spinal cord causes ischemia, necrosis and softening of the spinal cord, forming a cavity.

3. Mechanical factors: due to congenital factors, the fourth ventricle outlet obstruction, cerebrospinal fluid from the fourth ventricle to the subarachnoid space is blocked, the cerebrospinal fluid pulse wave down the central tube of the spinal cord, causing the central tube to expand, and break through the central wall to form a cavity .

4. Others, such as spinal cord cystic changes, traumatic myelopathy, radiation-induced myelopathy, spinal cord infarction softening, intraspinal hemorrhage, necrotizing myelitis.

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The age of onset is 31 to 50 years old, and children and the elderly are rare. More men than women, had a family history report. There are three aspects of the clinical manifestations of syringomyelia, and the degree of symptoms is closely related to the development of the cavity. The general course of disease progresses slowly, and the early symptoms are mostly segmental, affecting the upper limb first. When the cavity is further enlarged, the gray matter in the marrow and the white matter conduction bundle outside it are also involved, and the conduction beam dysfunction occurs below the cavity. Therefore, the symptoms of early patients are more limited and mild, and late symptoms are widespread and even paraplegic.

1. The sensory symptoms are located in the cervical and upper thoracic segments of the spinal cord according to the cavity, and are located on one side or in the center. The segmental sensory disturbance of the unilateral upper limb and the upper thoracic segment is often characterized by segmental dissociative sensory disturbance. Pain, temperature loss or disappear, deep feeling exists. This symptom can also be bilateral.

2. Sports symptoms The neck and thoracic cavity affect the anterior horn of the spinal cord, and there are symptoms of flaccid partial spasm on one or both sides of the upper limb. It is characterized by muscle weakness and decreased muscle tone, especially in the two-handed intermuscular muscle and interosseous muscle atrophy. In severe cases, the claw-shaped hand deformity appears. When the descending root of the trigeminal nerve is affected, the central side feels like central pain and temperature sensation, and the facial separation sensation is so-called "onion-like distribution" with weak chewing muscle strength. If the vestibular cerebellar conduction bundle is involved, dizziness, nausea, vomiting, gait instability, and nystagmus may occur. On one or both sides of the lower extremity, the upper part of the motor was paralyzed, the muscle tension was hyperthyroidism, the abdominal wall reflex disappeared and the Babinski sign was positive. Late cases are more severe.

3. Autonomic nerve damage symptoms involve the sympathetic spinal cord center of the spinal cord (neck 8 cervical and thoracic 1 chest) lateral angle, and Horner syndrome appears. The lesions may damage the corresponding segments, the limbs and the trunk skin may have abnormal secretions, and hyperhidrosis or hypohidrosis is the only sign of abnormal secretion. Less sweat can be confined to one side of the body, called "half side sweating", and more often seen on one side of the upper body, or one side of the upper limb or half of the face. Usually corneal reflexes can also weaken or disappear, because neurotrophic keratitis can cause bilateral corneal perforation. Another strange phenomenon of sweating is the increase in perspiration after cold, accompanied by a decrease in temperature, excessive fingertips, keratinization of the nails, atrophy, and loss of luster. Because of the pain and temperature loss, it is prone to burns, bumps and trauma. Patients with advanced disease have dysfunction of the stool and recurrent urinary tract infections.

Differential diagnosis

The disease should be identified with the following diseases:

1. Spinal cord tumors Extramedullary and intramedullary tumors can cause localized muscle atrophy and segmental sensory disturbance. In tumor cases, astrocytomas or ependymoma secreted from the gray matter of the spinal cord accumulate in the tumor. The diameter of the spinal cord is widened above and below, and the posterior column of the spine and neurological symptoms can be similar to syringomyelia, especially in the lower cervical spinal cord. However, the progression of tumor cases is faster, root pain is common, and nutritional disorders are rare. The protein in the early cerebrospinal fluid is increased and can be distinguished from the disease. CT and MRI can be identified for difficult cases.

2. Cervical vertebrae joint disease can cause upper limb muscle atrophy and long beam signs, but root pain is common, and segmental sensory disturbance with obvious lesion level is rare. Cervical radiography, spinal angiography if necessary, and cervical CT or MRI can help confirm the diagnosis.

3. Cervical ribs can cause localized atrophy of the small muscles of the hand and sensory disturbances, with or without evidence of compression of the subclavian artery, and because of the neck ribs often associated with syringomyelia, the diagnosis can be confused. However, the sensory disturbance caused by the neck rib is usually limited to the ulnar side of the hand and forearm. The tactile dysfunction is more serious than the pain dysfunction. The upper arm tendon reflex is not affected, and there is no long beam sign. When it can be identified, the cervical spine is taken. It also helps to establish a diagnosis.

4. Ulnar nerve palsy can produce localized atrophy of the interosseous muscle and the middle two sacral muscles. However, the sensory impairment is relatively minor and limited, and the sense of touch and pain are equally affected. The nerves in the back of the elbow usually have tenderness.

5. Leprosy can cause feelings to disappear, upper limb muscles atrophy, finger ulcers. However, there are thickening of the median, ulnar and radial nerves and brachial plexus, and there may be scattered depigmentation on the trunk.

6. Syphilis can be suspected of syringomyelia in two ways. In rare proliferative dural meningitis, upper limb sensory disturbances, atrophy, and weakness and lower limb pyramidal tract signs may occur, but myelography may show subarachnoid obstruction and progression is more rapid than syringomyelia. The syphilis of the spinal cord can show signs of intramedullary tumors, but the progression of the disease is rapidly disrupted and the syphilis seropositive.

7. Amyotrophic lateral sclerosis is not easily confused with syringomyelia because it does not cause paresthesia or loss of sensation.

8. Puncture injury or fracture displacement can sometimes cause intramedullary hemorrhage, which is concentrated in the same plane of the spinal cord as syringomyelia, but the history of injury and the evidence of spinal injury in X-ray films are sufficient to provide a basis for identification.

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