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Syringomyelia – Symptoms, Diagnosis And Treatment

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Syringomyelia is a chronic, progressive, degenerative disease of the spinal cord, characterized by the presence of a cystic degeneration (a cavity called syrinx) in the center of the spinal cord. Clinically, syringomyelia presents as main symptoms brachial muscle atrophy and dissociated sensory loss. The cavity is usually present in the cervical spine, but it can spread to the bulb, more rare to the pons or can extend to thoracic and lumbar segments of the spinal cord.

The exact cause of the disease is still unclear, but is assumed to be the result of incomplete closure of embryonic central canal, syringomyelia being included in the concept of status disraphicus. This hypothesis is supported by association of syringomyelia with other dysraphism aspects such as spina bifida, meningocele, myelomeningocele or chest malformations.

Another hypothesis sustain that the syrinx is the result of increased cerebrospinal fluid pressure due to delayed opening of Luscka and Magendie orifices of the fourth ventricle or due to the presence of obstructive factors near the foramen magnum, such as the herniation of cerebral tonsils (in Arnold -Chiari malformation).



Syringomyelia Symptoms

The disease occurs predominantly in men aged between 25 and 40 years. Rarely, clinical manifestations occur in childhood or adolescence. Syringomyelia onset is usually insidious, patient presenting painless burns of the arm or hand muscle atrophies are present.

Neurological impairment consist of a sensory lesional syndrome and a motor lesional syndrome. Sensory lesional syndrome consists of a characteristic sensory impairment called dissociated sensory loss, in which pain and temperature sensibility sensations are lost, while light touch, vibration, and position senses are preserved. Pain and temperature sensation may be impaired in either or both arms, or in a shawllike distribution across the shoulders and upper torso anteriorly and posteriorly. Due to the fact that sensory root of cranial nerve V (trigeminal) descends in the cervical region of the spinal cord, facial sensory impairment may be present.

Sensory lesional syndrome is produced by the anterior expansion of syrinx, interrupting the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in loss of these sensations in radicular bands characteristic for injured neuromeres.

Syrinx extension into the anterior horns of the spinal cord cause motor lesional syndrome, characterized by damages of motor neurons (lower motor neuron), leading to diffuse muscle atrophy that begins in the hands, conferring different aspects of the hand and progresses proximally including the forearms and shoulder girdles. Particular aspects of the hand are:

  • Simian hand, when the thumb goes in the same plan with the rest of the fingers, caused by atrophies that affect muscle from thenar and hypothenar eminence;
  • Claw hand, when first phalanges are in hyperextension and the rest of the phalanges are in flexion, caused by muscle atrophies that affect interossei and lumbrical muscles;
  • Preacher hand- hand remain in extension, due to long and deep flexors atrophies;
  • Skeletal hand, when atrophies affect all muscle groups, including extensors.

Trophic disturbances occur frequently and are represented by painless ulcers of the hand and osteolytic painless arthropathy, most commonly in the shoulder. Hypertrophy of  the hand, is characteristic for syringomyelia and is called cheiromegaly.

When the cavity enlarges and involve the posterior columns of spinal cord, position and vibration senses in the feet are lost and astereognosis may be noted in the hands. Horner syndrome may also appear, caused by impairment of sympathetic neurons in the intermediolateral cell column.

Syrinx may extend from medulla to the cerebral bulb, causing syringobulbia.  Syringobulbia, sometimes may precede spinal cord damage. The bulbar symptoms are unilateral and consist of dysphagia, dysphonia, dysarthria, nystagmus, pharyngeal and palatal weakness, vocal cord paralysis, asymmetric weakness and atrophy of the tongue, and sensory loss involving primarily pain and temperature senses in the distribution of the trigeminal nerve. Rearly, may occur diplopia (double vision), vertigo and pain in the trigeminal nerve territory.

Syringomyelia Diagnosis

The initial evaluation of a patient suspected of having syringomyelia includes a comprehensive history and physical examination, because information obtained from physical examination will guide the imaging studies which are the paraclinical examinations that establish an accurate diagnosis.

Imaging studies include plain radiography which is not useful in highlighting the cavity, but can detect cervical canal dilation and cranio-vertebral anomalies that may be associated with syringomyelia.

CT with contrast is useful in diagnosis because it can demonstrate the presence of syrinx.

Magnetic resonance imaging (MRI) of the brain and spinal cord, performed in the sagittal plane, highlight with high accurcy the medullary cavity and the associated anomalies.

Syringomyelia MRI

Syringomyelia MRI

Syringomyelia Treatment

Patients with syringomyelia do not benefit for a specific medical treatment. However, the disease has a chronic, stable clinical course. Identifying the underlying cause of syrinx formation is very important. Surgical treatment most likely will be necessary and is represented by:
  • Suboccipital and cervical decompression;
  • Laminectomy and syringotomy (dorsolateral myelotomy;
  • Syringoperitoneal shunt;
  • Fourth ventriculostomy;
  • Terminal ventriculostomy.

Neurorehabilitative care is important in preservation of remaining neurological functions and prevention of complications of quadriparesis such as infection and decubitus ulcers is mandatory.