Signs of syringomyelia

The inability to feel pain and temperature difference leads to the fact that patients often receive various injuries in the form of mechanical injuries, burns, which in most cases leads them to a doctor. However, the first symptoms appear much earlier: there are slight disturbances in sensitivity such as painful areas, numbness, burning, itching, etc. It is noteworthy that the tactile sensitivity of patients is not affected. Often, patients complain of prolonged dull soreness in the cervical spine, between the shoulder blades, in the upper limbs and chest. Partial loss of sensation in the lower limbs and lower body is less common.

With syringomyelia , bright neuro-trophic disorders are found, such as coarsening of the skin, cyanosis, long-term healing wounds, bone and articular deformity, and bone fragility. Patients notice typical symptoms of the hands: the skin becomes dry and rough, the fingers coarse and thicken. You can easily see numerous lesions on the skin: from multiple scars of various sizes to fresh burns, cuts, ulcers and abscesses. Not uncommon - the development of acute purulent processes of the panaritium type.

If the pathology extends to the lateral horns of the upper thoracic spinal regions, then there is a strong carpal coarsening - the so-called cheiromegaly. Violation of articular trophism (usually in the shoulder and elbow region) is manifested by bone fusion with the formation of cavity defects. The affected joint increases in size, there is no pain during movement, but there is a characteristic rubbing noise of the articular bones.

As the pathological process develops, the spinal cavity defects increase, spread to the area of the anterior horns. This is manifested by weakening of the muscles, movement disorders, the development of atrophic processes, the appearance of flaccid paresis of the hands. If syringomyelia affects the cervical spinal region, then Horner's syndrome becomes noticeable, which consists in drooping of the eyelid, dilated pupils, and retraction of the eyeball. If the motor conductive channels are affected, then paraparesis of the lower extremities can be observed, in some patients urinary disorders are noted.

The formation of a cavity in the brain stem indicates the development of syringobulbia: sensitivity is impaired in the face. Over time, speech suffers, swallowing becomes difficult, problems with the respiratory system arise, atrophic processes spread to the soft palate, tongue, part of the face. Secondary infection is not excluded: bronchopneumonia, inflammatory diseases of the urinary tract develops. In severe cases, bulbar paralysis is noted, which can cause respiratory arrest and death of the patient.

The clinical course of the disease progresses from months to years with an early rapid deterioration, which gradually slows down. There is a linear relationship between cyst morphology, symptom duration, and severity. [1], [2]

First signs

During a neurological examination in patients with syringomyelia, the following characteristic signs are found:

• Loss of pain and temperature sensations of the "jacket" or "half-jacket" type, spreading to the region of the extremities, the upper part of the body, less often to the lumbosacral region and the zone of innervation of the trigeminal nerve. With the further development of the disease, it is possible to add proprioceptive disorders related to vibration sensations, tactile and muscular-articular sensitivity. Conductive contralateral disturbances may also occur.
• The development of segmental disorders in the form of distal one and two-sided peripheral paresis of the extremities, as well as central disorders such as pyramidal insufficiency, spastic couple and monoparesis of the extremities. There is a likelihood of twitching in the affected muscles. If the medulla oblongata is involved in the process, disorders associated with paresis of the tongue, pharyngeal zone, vocal cords, and soft palate are found. [3]
• Symptoms from the autonomic nervous system appear against the background of trophic disorders. Blue fingers, changes in sweating (increased or complete cessation), swelling of the extremities are often observed. Problems are also observed on the part of the regeneration system: injuries and ulcers after injuries and burns do not heal for a long time. The osteoarticular mechanism is affected, defects, bone deformities are noted, leading to a disorder of the limb's working capacity.
• The defeat of the medulla oblongata is accompanied by the appearance of nystagmus, dizziness.
• Most patients have hydrocephalus, which is characterized by headache, nausea with vomiting, drowsiness, and congested optic discs.  [4]

Sensitive disorders

Pain is the body's natural response to injury. However, with syringomyelia, pain sensitivity and its other types are impaired. The following literally happens: a limb or other part of the body begins to constantly and intensely hurt, but at the same time the person does not feel pain from external stimuli. The body does not react if it is cut, pricked, burned: the patient simply does not feel it. Often, in patients suffering from syringomyelia, traces of cuts and burns with hot objects are found on the skin: the patient does not feel that he has touched a hot or sharp one, does not pull his hand, which leads to a burn or cut. In medical circles, this condition is called "painful numbness" or "dolorosis anesthesia." [5]

In addition, metabolic processes deteriorate, trophism of tissues in the pathological zone: the affected limb or part of the body lose subcutaneous fat, the skin becomes pale cyanotic, rough, peeling appears, the nail plates fade. Swelling is possible, including in the area of the joints. The musculoskeletal mechanism also suffers: muscles atrophy, bones become fragile.

Bulbar disorders in syringomyelia

Dysfunction of the glossopharyngeal, vagus and hypoglossal nerves, or their motor nuclei, occurs when syringomyelia spreads to the region of the medulla oblongata. In this case, the lingual muscles, soft palate, pharynx, epiglottis and vocal cords suffer. Pathology is bilateral or unilateral.

Clinically bulbar disorders are as follows:

• speech disorders (aphonia, dysarthria - distorted or difficult pronunciation of sounds);
• swallowing disorders (dysphagia, especially with regard to swallowing liquid food);
• deviations of the tongue to the left or right, deterioration of its mobility;
• non-closure of the vocal cords;
• loss of the pharyngeal and palatine reflex.

With atrophy of the lingual muscles, fibrillar twitching is noted.

Lermitte's symptom with syringomyelia

For patients with loss of sensitivity in the lower torso and legs, Lermitte's symptom is characteristic, which consists in a sudden short-term pain that covers the spinal column from top to bottom, like an electric shock.

Such a manifestation is considered one of the acute symptoms of sensory disorders. For the patient, such episodic short-term pain is extremely unpleasant. At the same time, there is a tingling sensation, tension along the axis along the spine and to the upper limbs.

The symptom occurs against the background of mechanical irritation, which can occur with a sharp bend of the neck, as well as during sneezing or coughing. Pathology is observed in about 15% of patients.

Syringomyelia in children

Syringomyelia is rare in childhood. Since the disease is characterized by a slow increase, pathological symptoms rarely make themselves felt at an early stage of formation. The main cause of pediatric pathology is a violation of the development of the spinal cord, namely, the improper formation of the suture that connects the two halves of the spinal cord, as well as the cleft of the central canal.

For childhood syringomyelia, less pronounced sensory and pain disorders are characteristic, in contrast to the same disease in adults. However, children are more at risk of developing scoliosis, which is more favorable in terms of surgical correction. In some cases, childhood syringomyelia can heal on its own. [6]

The disease is never the same in different patients. For some patients, pathology reveals itself only as mild symptoms, with their subsequent stabilization throughout the year. In others, the disease can progress dramatically, complicated by disorders or loss of important body functions, which entails a significant deterioration in the quality of life. Familial cases of the disease are also known, which often require surgical treatment.

Forms

The classification of syringomyelia involves several types of pathology:

• A central channel non-communicating disorder that is considered the most common. Its appearance can occur simultaneously with the deterioration of the patency of the spinal canal in the subarachnoid space, or with type I Arnold-Chiari malformation.
• An extracanal non-communicating disorder that occurs when the spinal column is damaged, or when blood flow in the spinal body is impaired. In the area of damage, a cystic element is formed, prone to further spread.
• Central canal communicating disorder found simultaneously with Dandy-Walker and Arnold-Chiari II syndromes. Hydrocephalus is also characteristic.

Since 1974, there has been another similar classification of the disease:

• The communicating disorder, with penetration into the subarachnoid space of the spinal column, develops as a result of pathological changes in the area of the craniovertebral junction or the base of the skull.
• Post-traumatic syringomyelia, with the formation of a cavity in the area of damage, increases and develops in the adjacent parts of the spinal column. Pathological signs appear at a late stage, after a fairly long period of time, when the victim, it would seem, has fully recovered.
• A disorder that develops as a result of arachnopathy or arachnoiditis.
• Cysts that appear as a result of tumor processes in the spinal cord.
• A disorder associated with non-neoplastic processes that cause increased pressure on the spinal cord.
• Idiopathic disorder, the cause of which cannot be established.

Depending on the localization of the pathology, there are:

• posterior (sensitive);
• anterior (motor);
• lateral horn (vegetative trophic);
• mixed syringomyelia.

Anterior syringomyelia is rarely found in isolation. Most often, motor disorders are combined with sensitivity disorders.

Depending on the distribution of the disorder along the spinal axis, the following types of disease are distinguished:

• Syringomyelia of the cervical spine - develops most often and has characteristic features such as loss of sensitivity in the arms and trunk (the affected areas are designated as a "jacket" or "half jacket".
• Syringomyelia of the thoracic spine is often combined with lesions of the cervical spine and causes trophic muscle disorders in the upper extremities. Fibrillar muscle twitching is usually mild.
• Syringomyelia of the lumbar spine (or lumbosacral) is accompanied by paresis of the lower extremities, which happens relatively rarely (about 10%) and is most often caused by tumor or inflammatory processes in the spine.
• Total syringomyelia occurs in 10% of cases and is characterized by the appearance of pathological cavities throughout the spinal cord, and not only in any one section. This form of the disease is the most unfavorable in terms of prognosis and cure.
• Stem and spinal syringomyelia develops when the brain stem is affected. The patient has nystagmus, bulbar disorders (difficulty swallowing, speaking, etc.). Violation of facial sensitivity is possible.
• Encephalomyelitis syringomyelia (another name is syringoencephaly) is a lesion of the internal capsule of the brain, in which a motor and sensory disturbance occurs on the opposite side of the body.