What Is Myelomalacia?

Syringomyelia is a chronic, progressive disease of the spinal cord. The etiology is not very clear, and its lesions are characterized by the formation of a tubular cavity in the spinal cord (mainly gray matter) and the proliferation of glial (non-neural cells). Often occurs in the spinal cord of the neck. When the disease involves the medulla oblongata, it is called medulla oblongata.

Basic Information

English name: syringomyelia
Visiting department: Neurosurgery, spine surgery
Multiple groups: 31 to 50 year old men

Common locations: spinal cord
Common causes: Due to congenital dysplasia or spinal cord disease
Common symptoms: Segmental paresthesia, limb dyskinesia, Horner syndrome
Contagious: no

Causes of syringomyelia

The exact etiology is unknown and can be divided into congenital dysplasia and secondary syringomyelia, the latter of which is rare.

1. Congenital atresia of spinal cord and neural tube

This disease is often accompanied by spina bifida, cervical ribs, scoliosis, ring occipital deformity and other congenital abnormalities support this view.

2. Abnormal spinal blood circulation

Causes spinal cord ischemia, necrosis, softening, and formation of cavities.

3. Mechanical factors

Due to congenital factors, the fourth ventricle exit obstruction, cerebrospinal fluid flow from the fourth ventricle to the subarachnoid space is blocked, the cerebrospinal fluid pulsating waves hit the central tube of the spinal cord downward, causing the central tube to expand and break through the central tube wall to form a cavity.

4. Other

Such as cystic changes of spinal cord tumors, traumatic myelopathy, radiation myelopathy, spinal cord infarcts, softened intraspinal hemorrhage, and necrotizing myelitis.

Clinical manifestations of syringomyelia

The age of onset is 31 to 50 years. Children and the elderly are rare. There are more men than women. They have a family history. The clinical manifestations of syringomyelia have three aspects. The degree of symptoms has a great relationship with the development of cavities. Generally, the progress of the disease is slow. Symptoms that appear in the early stages are mostly distributed in segments, affecting the upper limbs first. When the cavity further expands, the gray matter inside the pulp and the white matter outside the conductive bundle are also involved, and the conductive bundle dysfunction occurs below the cavity. The patient's symptoms were more limited and mild, and the advanced symptoms were extensive and even paraplegic.

Sensory symptoms

According to the cavity located in the cervical and upper thoracic segments of the spinal cord, it is biased to one side or centered, and there is a segmental sensory dysfunction of unilateral upper limbs and upper thoracic ganglia, often characterized by segmental detached sensory dysfunction, and pain and hypothermia Or disappear, deep feeling exists, the symptoms can also be bilateral.

2. Motor symptoms

Cervical and thoracic cavity affects the anterior horn of the spinal cord, flaccid partial paralysis of one or both sides of the upper limbs, manifested by muscle weakness and decreased muscle tension, especially in the interstitial muscles of the two hands, and the interosseous muscle atrophy is the most obvious. Deformed hand deformities, when the descending root of the trigeminal nerve is affected, ipsilateral sensations of central pain, temperature dysfunction, and lack of facial separation sensation form a so-called "onion-like distribution" with weak chewing muscles. If the vestibular cerebellum conducts Involved in the bundle, dizziness, nausea, vomiting, gait instability, and nystagmus can occur, while upper motor partial paralysis of one or both lower limbs occurs, hypertonicity, disappearance of abdominal wall reflexes and Babinski sign are positive, and paralysis is more common in advanced cases Heavier.

3. Symptoms of Autonomic Nerve Damage

The cavity involves the sympathetic spinal cord center of the spinal cord (neck 8 cervical spine and thoracic 1 spinal cord), Horner syndrome appears, lesions damage the corresponding segment, and limb and trunk skin may have abnormal secretion, hyperhidrosis or hypohidrosis is secretion Anomalous only signs, hypohidrosis can be confined to one side of the body, called "hemihidrosis", and more commonly seen on one side of the upper body, or on one side of the upper limbs or half of the face, usually with corneal reflexes Weakened or disappeared. Neurotrophic keratitis can cause bilateral corneal perforation. Another strange sweating phenomenon is increased perspiration after cold, accompanied by lower temperature, excessive keratinization of fingertips and nails, atrophy, and loss of gloss. Due to the disappearance of pain and temperature sensation, scalding and trauma are prone to occur, and patients with advanced urinary disorders and recurrent urinary tract infections appear.

Syringomyelia

MRI examination: The cavity shows a low signal, the sagittal position appears on the longitudinal axis of the spinal cord, and the cross-section can clearly show the size and shape of the cavity in the plane. MRI is of higher diagnostic value for this disease.

Syringomyelia diagnosis

According to the characteristics of chronic onset and clinical manifestations, there are segmental dissociated sensory disorders, lower motor neuron dyskinesias in the upper limbs, and upper motor neuron dyskinesias in the lower limbs. Many can make a clear diagnosis, combined with imaging Performance can further confirm the diagnosis.

Syringomyelia treatment

General treatment

Using neurotrophic drugs, radiation therapy has been tried in the past, but the effect is not clear. In view of the slow progress of the disease, and often associated with ring occipital malformations and cerebellar subtonsal hernia deformities, and these are considered to be related to the cause, surgery should be taken after a clear diagnosis.

The theoretical basis of surgery is:

(1) Perform decompression at the craniocervical junction area, deal with possible deformities and other pathological factors at the site, eliminate the cause, and prevent the development and deterioration of the lesion;

(2) Do a short hole incision and shunt to reduce the hole and relieve the internal pressure factors to relieve symptoms.

2. Other treatments

Including vitamin B family, vasodilators, nerve cell metabolic function activators, etc., can be applied. According to the condition, physical therapy, physical therapy and acupuncture can be used to promote the recovery of neural function after surgery.