Signs of syringomyelia

The inability to feel pain and temperature differences leads to patients often receiving various injuries in the form of mechanical trauma, burns, which in most cases leads them to the doctor. However, the first symptoms appear much earlier: slight sensitivity disorders are noted in the form of painful areas, numbness, burning, itching, etc. It is noteworthy that the tactile sensitivity of patients is not affected. Often, patients complain of prolonged dull pain in the cervical spine, between the shoulder blades, in the upper limbs and chest. Partial loss of sensitivity in the lower limbs and lower body occurs less often.

Syringomyelia is characterized by vivid neurotrophic disorders, such as roughening of the skin, cyanosis, slow-healing wounds, bone and joint deformation, and bone fragility. Patients note typical symptoms in their hands: the skin becomes dry and rough, the fingers become rough and thick. Numerous skin lesions can be easily seen: from multiple scars of varying sizes to fresh burns, cuts, ulcers, and abscesses. Acute purulent processes such as panaritium often develop .

If the pathology extends to the lateral horns of the upper thoracic spinal cord, then severe wrist coarsening is observed - the so-called cheiromegaly. Violation of articular trophism (usually in the shoulder and elbow area) is manifested by bone melting with the formation of cavity defects. The affected joint increases in size, pain during movement is not observed, but there is a characteristic noise of friction of the articular bones.

As the pathological process develops, the spinal cavity defects increase and spread to the anterior horn area. This is manifested by weakening of the muscles, motor disorders, development of atrophic processes, and the appearance of flaccid paresis of the arms. If syringomyelia affects the cervical spinal cord, Horner's syndrome becomes noticeable, which consists of drooping eyelids, dilated pupils, and sunken eyeballs. If the motor conduction channels are affected, paraparesis of the lower extremities can be observed, and some patients experience urinary disorders.

The formation of a cavity in the brain stem indicates the development of syringobulbia: sensitivity is impaired in the facial area. Over time, speech suffers, swallowing becomes difficult, problems with the respiratory system arise, atrophic processes spread to the soft palate, tongue, and part of the face. Secondary infection is also possible: bronchopneumonia and inflammatory diseases of the urinary tract develop. In severe cases, bulbar paralysis is observed, which can cause respiratory arrest and death of the patient.

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

First signs

During a neurological examination, patients suffering from syringomyelia are found to have the following characteristic signs:

• Loss of pain and temperature sensations of the "jacket" or "half-jacket" type, spreading to the limbs, upper body, and less often to the lumbosacral region and the trigeminal nerve innervation zone. With further development of the disease, proprioceptive disorders may be added, concerning vibration sensations, tactile and muscular-articular sensitivity. Conductive contralateral disorders may also be observed.
• Development of segmental disorders in the form of distal unilateral and bilateral peripheral paresis of the limbs, as well as central disorders such as pyramidal insufficiency, spastic para and monoparesis of the limbs. There is a possibility 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 detected. [ 3 ]
• Symptoms from the autonomic nervous system appear against the background of trophic disorders. Often observed are blue fingers, changes in sweating (increased or complete cessation), swelling of the limbs. Problems are also observed from the regeneration system: damage and ulcers after injuries and burns do not heal for a long time. The bone-articular mechanism is affected, defects and bone deformations are noted, leading to a disorder in the functioning of the limb.
• Damage to the medulla oblongata is accompanied by the appearance of nystagmus and dizziness.
• Most patients experience hydrocephalus, which is characterized by headache, nausea and vomiting, drowsiness, and congestion of the optic nerve heads. [ 4 ]

Sensory disturbances

Pain is a natural reaction of the body to injury. However, with syringomyelia, both pain sensitivity and other types of it are impaired. Literally, the following happens: a limb or other part of the body begins to hurt constantly and intensely, 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, patients suffering from syringomyelia have traces of cuts and burns from hot objects on the skin: the patient does not feel that he touched something hot or sharp, does not pull his hand away, which leads to the appearance of a burn or cut. In medical circles, this condition is called "painful insensitivity" or "anesthesia dolorosa". [ 5 ]

In addition, metabolic processes and tissue trophism in the pathological zone deteriorate: the affected limb or area of the body loses subcutaneous fat, the skin becomes pale-blue, rough, peeling appears, and the nail plates become dull. Edema is possible, including in the joint area. The musculoskeletal mechanism also suffers: muscles atrophy, bones become fragile.

Bulbar disturbances in syringomyelia

Disorders of the glossopharyngeal, vagus and hypoglossal nerves or their motor nuclei occur when syringomyelia spreads to the medulla oblongata. The lingual muscles, soft palate, pharynx, epiglottis and vocal cords are affected. The pathology can be bilateral or unilateral.

Clinically, bulbar disorders appear as follows:

• speech disorders (aphonia, dysarthria – distorted or difficult pronunciation of sounds);
• swallowing disorders (dysphagia, especially regarding swallowing liquid food);
• deviation of the tongue to the left or right, deterioration of its mobility;
• failure of vocal cord closure;
• loss of pharyngeal and palatal reflexes.

With atrophy of the lingual muscles, fibrillary twitching is observed.

Lhermitte's sign in syringomyelia

Patients with loss of sensation in the lower body and legs are characterized by Lhermitte's symptom, which consists of sudden, short-term pain that covers the spine 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, tingling, tension along the axis along the spine and to the upper limbs are felt.

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

Syringomyelia in children

Syringomyelia is rare in childhood. Since the disease is characterized by slow progression, pathological symptoms rarely make themselves known at an early stage of development. The main cause of childhood pathology is a violation of the development of the spinal cord, namely, the incorrect formation of the suture that connects the two halves of the spinal cord, as well as non-closure of the central canal.

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

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

Forms

The classification of syringomyelia suggests several types of pathology:

• Central canal noncommunicating disorder, which 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 Arnold-Chiari malformation type I.
• An extracanal noncommunicating disorder that occurs when the spinal column is damaged or when blood flow in the spinal cord is impaired. A cystic element is formed in the area of damage, which is prone to further spread.
• Central canal communicating disorder, found simultaneously with Dandy-Walker and Arnold-Chiari type II syndromes. Hydrocephalus is also characteristic.

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

• 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.
• Posttraumatic syringomyelia, with the formation of a cavity in the area of damage, increases and develops in the adjacent sections 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.
• An idiopathic disorder whose cause cannot be determined.

Depending on the localization of the pathology, the following are distinguished:

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

Anterior corneal syringomyelia is rarely seen in isolation. Most often, motor disorders are combined with sensory disturbances.

Depending on the spread 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 signs, 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 damage to the cervical spine and causes the appearance of trophic muscle disorders in the upper limbs. Fibrillary muscle twitching is usually weakly expressed.
• Syringomyelia of the lumbar region (or lumbosacral) is accompanied by paresis of the lower extremities, which occurs 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 entire length of the spinal cord, and not just in one section. This form of the disease is the most unfavorable in terms of prognosis and cure.
• Stem and spinal syringomyelia develops when the brainstem is damaged. The patient experiences nystagmus, bulbar disorders (difficulty swallowing, speech, etc.). Facial sensitivity may be impaired.
• Encephalomyelitic syringomyelia (another name is syringoencephaly) is a lesion of the internal capsule of the brain, which causes motor and sensory impairment on the opposite side of the body.