Syringomyelia

What is a Syringomyelia?

The term “Syringomyelia” refers to a neurological disorder where a fluid-filled cyst (syrinx) develops within the spinal cord ¹. The enlargement and elongation of the cyst over time can damage the spinal cord and results in pain, weakness, and stiffness in the back, shoulders, and extremities. This condition also causes the loss of the ability to feel extremes of cold or hot, especially in the hands. People with syringomyelia often develop bilateral loss of pain and temperature sensation in the chest and arms.

Syringomyelia mainly develops in two forms: congenital and acquired. Acquired syringomyelia occurs due to serious physical trauma to the body such as road traffic accidents. Classification of syringomyelia also includes communicating and non-communicating forms. Communicating form develops due to lesions on the foramen magnum and the non-communicating form occurs due to other spinal cord diseases. A congenital form of syringomyelia relates with the malformation of the brain known as Arnold–Chiari malformation or Chiari type 1 malformation (CM1) ².

Syringomyelia can develop as a complication of trauma, tumor, meningitis, hydrocephalus, hemorrhage, arachnoiditis, etc. Symptoms of syringomyelia usually develop slowly over time. Symptoms of Chiari malformation generally begin between ages 25 and 40. Straining or coughing may trigger the symptoms of syringomyelia in many cases. Signs and symptoms of syringomyelia comprise muscle weakness, muscle atrophy, loss of reflexes, headache, loss of sensation, spinal curvature (scoliosis), severe pain in the back, shoulders, arms, and legs.

A study reported the prevalence of syringomyelia at 8.4 cases per 100,000 people with symptoms that usually started in young adulthood ³. In some people, syringomyelia leads to serious complications. Others produce no symptoms. Possible complications of syringomyelia may include paralysis, abnormal curve of the spine, motor difficulties, chronic pain, etc.

How do Neurosurgeons Diagnose Syringomyelia?

The initial diagnosis of syringomyelia includes a physical examination and pain patterns of patients. Physicians now use magnetic resonance imaging (MRI) to diagnose syringomyelia. This technique uses radio waves and a strong magnetic field to produce detailed images of your spine and spinal cord. Other imaging tests may include ⁴:

• X-ray: To show the alignment of the brain and spinal cord.
• Electromyography (EMG): Uses electrical impulses to see whether the nerve functions normally.
• Computed tomography (CT) scan: Produces 3D images to show more detail of the spine and reveal the presence of tumors and other abnormalities such as hydrocephalus.
• Myelogram: This uses radiographs and requires a contrast medium that injects into the subarachnoid space.

How do Neurosurgical Physicians Treat Syringomyelia?

Treatment of syringomyelia depends on the progression of the disorder and how abruptly the affects the patients. Patients with mild or no symptoms may recover without treatment. The neurologist will recommend medications or surgery when symptoms negatively affect the life. The medical team should comprise neurosurgeons, nerve specialists, and physical therapists while treating syringomyelia. The treatment protocol may include ⁵:

• Medications: Medicines such as gabapentin helps to decrease the painful sensation of the shoulders and arms that frequently occur with syringomyelia.
• Physical therapy: This includes stretching and light exercises that can reduce pressure on your nerves and relieve minor pains. Patients may require passive mobilizations, occupational therapy, or even speech therapy.

Surgery helps to correct the underlying cause of the syrinx and relieve the pressure on your spinal cord. In the case of CM1, surgeons suggest to expand the base of the skull and the covering of the brain. This will take the pressure off the spinal cord and brain. Surgery resolves the syringomyelia in maximum cases. Contact your doctor to select the best option for you. Since Syringomyelia can recur after surgery. So, regular examinations including periodic MRIs, requires to assess the outcome of the surgery.

References

1. Roy, A. K., Slimack, N. P. & Ganju, A. Idiopathic syringomyelia: Retrospective case series, comprehensive review, and update on management. Neurosurg. Focus 31, 1–9 (2011).
2. Elliott, N. S. J., Bertram, C. D., Martin, B. A. & Brodbelt, A. R. Syringomyelia: A review of the biomechanics. J. Fluids Struct. 40, 1–24 (2013).
3. Sharma, M., Coppa, N. & Sandhu, F. A. Syringomyelia: A Review. Semin. Spine Surg. 18, 180–184 (2006).
4. Chen, J. K. et al. Acute idiopathic syringomyelia: A case report. Kaohsiung J. Med. Sci. 20, 404–409 (2004).
5. Kleindienst, A., Laut, F. M., Roeckelein, V., Buchfelder, M. & Dodoo-Schittko, F. Treatment of posttraumatic syringomyelia: evidence from a systematic review. Acta Neurochir. (Wien). 162, 2541–2556 (2020).