Syringomyelia - is a kind of progressive myelopathy. In the case of syringomyelia syringomyelic cavity formed in the spinal cord central portion (central channel). Spinal cord syringomyelia - is the congenital anomaly. Sometimes syringomyelia may form after:
- spinal cord injury,
- primary intraspinal (intramedullary) tumors,
- extrinsic spinal cord's central part necrosis,
- spinal arachnoiditis,
- hemorrhachis or necrotizing myelitis.
In the case of congenital nature of syringomyelia, disease manifestation begins with lesion of the middle portions at the cervical level, and then extends upward to the medulla oblongata and downward to the level of the lumbar spinal cord. Often syringomyelic cavity disposed eccentrically, causing in patients unilateral neurological conductive disorders symptoms or tendon and periosteal reflexes asymmetry.
In many cases of syringomyelia in patients also observed combination of the head and neck structures congenital anomalies (craniovertebral anomalies):
- Arnold-Chiari anomaly,
- basilar Impression (platybasia),
- median aperture (of Magendie) atresia,
- Dandy-Walker cyst.
On cervical spine MRI shown two syringomyelic cavity, filled with liquor (indicated by arrows).
The main clinical syringomyelia symptoms resemble of the spinal cords upper cervical segments central lesions syndrome. Neurological symptoms in syringomyelia will depend on the length of syringomyelic cavity and concomitant abnormalities. Syringomyelia typical neurologic manifestations of include:
- dissociated type sensation loss in a patient (lost pain and temperature sensitivity, but saved tactile and vibration sensitivity) on the neck posterior surface, shoulders and upper limbs ("capes" type) with the possible hands involvement;
- lower neck , shoulder girdle muscles, hands and palms muscles atrophy, tendon and periosteal reflexes asymmetric loss;
- thoracic spine upper level deformity in the form kyphoscoliosis.
Most often, the symptoms in patients with syringomyelia occur asymmetrically in the form of a unilateral sensitivity reduction. In some patients may be reduced face pain sensitivity, which is caused by lesions of the spinal nucleus of the trigeminal nerve paths at the upper cervical spinal cord segments level. Headache and neck pain provocation by cough occurs in a patient with syringomyelia combined with congenital anomaly (Arnold-Chiari malformation).
In cases of idiopathic syringomyelia, neurological symptoms formed in teenagers or young people and progress is uneven. Often neurological symptoms subsequently ceases progressing for several years. Some patients with syringomyelia not become disabled, but most of them are not able to take care of themselves and move independently. The loss of pain sensation (analgesia) in patients with syringomyelia contributes to the appearance of injuries, burns and ulcers on the fingertips.
On cervical spine MRI is defined syringomyelia cavity at the C6 vertebral body level (arrow).
At advanced stages of syringomyelia can develop neurogenic arthropathy (joint of Charcot) shoulder, elbow and knee joints. Marked weakness in the lower extremities or increased reflexes (hyperreflexia) indicate about concomitant cervical-cranial (craniovertebral) junction anomalies.
Siringobulbia - is a kind of syringomyelia. Syringomyelic cavities extend to the medulla oblongata level and sometimes pons because of siringobulbia. In siringobulbia syringomyelic cavity usually supersede tegmentum in its lateral parts. In patients with siringobulbia occurs the soft palate and vocal cords paralysis, dysarthria, nystagmus, dizziness, tongue atrophy and Horner's syndrome.
Slow growth of syringomyelic cavity leads to subarachnoid space narrowing or complete blockade around the spinal cord. Syringomyelic cavity may be separated from the spinal cords central channel, but is typically connected thereto.
Syringomyelia diagnosis based on clinical signs and confirmed by finding of expanded cervical spinal cord at myelography, as well as on the results of the spinal cord CT scans, produced a few hours later after water-soluble contrast injection into the subarachnoid space. Best of all cystic cavities is visible on spinal cords MR imaging. Because of other potential congenital anomalies in patients is necessary cervico-occipital junction additional similarly study.
Neurosurgical treatment (operation) of syringomyelia is directed to syringomyelic cavity decompression. Neurosurgeons performed decompression of syringomyelic cavity in patients with syringomyelia for prevent of further spinal cord progressive destruction and for spinal canal decompression in the case of spinal cord extension. In the case of spinal cord enlargement on cervical level with Arnold-Chiari abnormality combination is indicated operation (laminectomy and suboccipital decompression), which expand bony window and eliminates spinal cord, cerebellum and medulla compression by occipital bone and vertebral arch.
Operation laminectomy is performing in case of syringomyelia for spinal cord decompress, which removes its compression.
If you have any questions on the diagnosis or treatment of syringomyelia, you can ask them to our neurosurgeon or neurologist: (499) 130–08–09
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