Syringomyelia

Syringobulbia

What is Syringomyelia?

Syringomyelia describes the condition in which a fluid-filled cavity grows within the spinal cord, usually in the neck area. If the cavity forms within the lower brain stem, the condition is referred to as syringobulbia. As the fluid-filled cavity gradually expands, it presses on nerves that serve the hands and arms (syringomyelia in the neck area) or the lower extremities (syringomyelia in the lumbar area). As a result, lack of nerve sensation and muscular atrophy typically occur. In about half of the cases, the disorder is present at birth (congenital). The expansion of the cavity often occurs during the teen or young adult years. Syringomyelia can also be caused by spinal cord trauma or the development of a spinal cord tumor.

How is it diagnosed?

History: The individual may first notice a lack of sensation in the fingers such as a painless burn or cut. A lack of nervous sensation in a cape-like pattern over the shoulders and back is common. Stiffness and weakness of the lower extremities may also develop. In syringobulbia, the individual frequently complains of dizziness and a sensation of spinning (vertigo), jerky eye movements (nystagmus), tongue wasting, uni- or bilateral facial sensory impairment, and speech impairment (dysphagia).

Physical exam: The most prominent signs include decreased or absent deep tendon reflexes in the arm and a lack of sensation (dissociated anesthesia) below the syringomyelia level of the cavity, often in the pattern of a cape or shawl. Symptoms are nearly always present on both sides of the body (bilateral) but may be asymmetrical. Because of the loss of pain and temperature sensation in the hands and inner forearms, painless ulcer may follow a trauma.

Tests: Typically, CT scans, myelography, MRI, and x-rays of the skull and cervical spine are utilized in making a definitive diagnosis. Additionally, electromyography may be used to demonstrate denervation in the upper arm.

How is Syringomyelia treated?

Surgery is used in an attempt to drain the fluid from the cavity, reduce pain, and stop further progression of neurological symptoms. Congenital syringomyelia may require that pieces of cervical vertebrae in the area of the cavity be removed (cervical laminectomy) in an effort to drain the cavity and decompress the spinal cord.

Medications

Information Brand Generic Label Rating
http://www.nmihi.com/b/baclofen.html Lioresal Baclofen On-Label

What might complicate it?

Neurologic deficits, intense pain, and chronic spinal problems are possible complications.

Predicted outcome

Approximately half of the individuals with syringomyelia improve significantly after surgery. The rest, although relieved of some pain, eventually become wheelchair-bound.

Alternatives

Differential diagnoses include neoplasms and vascular malformations.

Appropriate specialists

Neurologist and neurosurgeon.

Last updated 15 December 2011


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