eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases
Syringomyelia: Differential Diagnoses & Workup
Updated: Sep 24, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
Arnold-Chiari malformations
Cervical rib
Craniovertebral junction anomalies
Increased intracranial pressure
Intrinsic tumors of the spinal cord
Brainstem syndromes
Cervical disk syndromes
Workup
Laboratory Studies
- Cerebrospinal fluid analysis (not performed because of the risk of herniation)
- CSF pressure sometimes is elevated. A complete subarachnoid block may be noted.
- Cell count is rarely more than 10/mm3.
- Mild elevation of the CSF protein content occurs in half of these cases.
- In cases of subarachnoid block, CSF protein may exceed 100 mg/dL.
Imaging Studies
- Plain x-ray
- Plain films cannot detect the syrinx directly.
- Cervical canal commonly is widened, and the pedicles may be eroded.
- Flexion and extension films exclude bony instability.
- Basilar impression or craniovertebral anomalies may be demonstrated.
- Computerized tomography scan - Assists in detailed assessment and is especially useful in evaluation of bony spinal canal components
- Myelography
- Myelography is performed in special situations when MRI cannot be used.
- Widening of the cord and complete subarachnoid block may be observed.
- CT myelography
- Myelogram combined with immediate and delayed high-resolution CT scan also can be performed.
- Delayed CT scans are obtained 4-24 hours after the initial testing and can demonstrate cyst filling.
- Magnetic resonance imaging
- Imaging of the entire rostrocaudal extension of the cyst or cysts is important. Gadolinium-enhanced images are indicated if a tumor is suspected. Gadolinium-enhanced images are helpful in differentiating between scar or disk material associated with a syrinx, especially in postoperative or posttraumatic cases.
- MRI examination should include sagittal and transverse views in T1 and T2 images (see Media file 1). Proton density scans also can be helpful.
- Magnetic resonance angiography - Can be especially helpful in cases of syringomyelia associated with vascular lesions
- Cine phase-contrast MRI - Used to analyze CSF flow dynamics near the spinal cord cyst
- Real-time ultrasonography - Rarely utilized for imaging syringomyelia since the development of MRI; ultrasonography for this purpose is technically more feasible in young children or in thin patients.
Other Tests
- In neurophysiological assessment by somatosensory evoked potentials (SSEPs), low-amplitude or delayed responses are present in myelopathy.
- Neurophysiological assessment by motor evoked response may be more sensitive than SSEPs in the evaluation of spinal cord dysfunction.
Procedures
- The initial evaluation of patients suspected of having a spinal cord syrinx includes a comprehensive history and physical examination.
- Information obtained from examinations guides the imaging studies. Essential tests include plain radiographic series with dynamic views and high-resolution CT scan to assess the bony spinal canal.
- The most sensitive imaging test for soft tissue is an MRI scan. Gadolinium-enhanced images are also helpful in differentiating between tumor, scar, and disk material, especially in postoperative or posttraumatic cases.
Histologic Findings
The syringomyelic cavity, or syrinx, forms most commonly in the lower cervical region, particularly at the base of the posterior horn and extending into the central gray matter and anterior commissure of the cord.
Histopathologic findings include (1) cavitation of spinal cord gray matter, (2) syrinx continuous with or adjacent to the central canal, and (3) an inner layer of gliotic tissue.
In association with the syrinx, other pathological conditions such as tumors, vascular anomalies, or infective processes also may be evident.
More on Syringomyelia |
| Overview: Syringomyelia |
Differential Diagnoses & Workup: Syringomyelia |
| Treatment & Medication: Syringomyelia |
| Follow-up: Syringomyelia |
| Multimedia: Syringomyelia |
| References |
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References
Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. Jun 1965;28:247-59. [Medline].
Williams B. Progress in syringomyelia. Neurol Res. Sep 1986;8(3):130-45. [Medline].
Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg. Jan 1994;80(1):3-15. [Medline].
Attal N, Parker F, Tadié M, et al. Effects of surgery on the sensory deficits of syringomyelia and predictors of outcome: a long term prospective study. J Neurol Neurosurg Psychiatry. Jul 2004;75(7):1025-30. [Medline].
Boman K, Iivanainen M. Prognosis of syringomyelia. Acta Neurol Scand. 1967;43(1):61-8. [Medline].
Carroll AM, Brackenridge P. Post-traumatic syringomyelia: a review of the cases presenting in a regional spinal injuries unit in the north east of England over a 5-year period. Spine. May 15 2005;30(10):1206-10. [Medline].
Chang HS, Nakagawa H. Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. J Neurol Neurosurg Psychiatry. Mar 2003;74(3):344-7. [Medline].
Colombo A, Cislaghi MG. Familial syringomyelia: case report and review of the literature. Ital J Neurol Sci. Dec 1993;14(9):637-9. [Medline].
Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. Oct 2006;29(4):251-63; discussion 264. [Medline].
Gruber DP, Crone KR. Neuroendoscopy. In: Grossman RG, Loftus CM, eds. Principles of Neurosurgery. 2nd ed. Philadelphia: Lippincott-Raven; 1998:757-62.
Hopkins A. Clinical Neurology: A Modern Approach. 1993. New York: Oxford University Press; 342-4.
Huewel N, Perneczky A, Urban V, Fries G. Neuroendoscopic technique for the operative treatment of septated syringomyelia. Acta Neurochir Suppl (Wien). 1992;54:59-62. [Medline].
Kaminsky SB, Clark CR, Traynelis VC. Operative treatment of cervical spondylotic myelopathy and radiculopathy. A comparison of laminectomy and laminoplasty at five year average follow-up. Iowa Orthop J. 2004;24:95-105. [Medline].
Koyanagi I, Iwasaki Y, Hida K, Houkin K. Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis. Surg Neurol. Apr 2005;63(4):350-5; discussion 355-6. [Medline].
Lin JW, Lin MS, Lin CM, Tseng CH, Tsai SH, Kan IH. Idiopathic syringomyelia: case report and review of the literature. Acta Neurochir Suppl. 2006;99:117-20. [Medline].
Madsen III PW, Green BA, Bowen BC. Syringomyelia. In: Herkowitz HN, Garfin SR, Balderston RA, et al, eds. The Spine. 2. 4th ed. Philadelphia: WB Saunders Company; 1999:1431-59.
Mancall EL. Syringomyelia. In: Rowland LP, ed. Merritt's Textbook of Neurology. 1989. 8th ed. Philadelphia: Lea & Febiger; 687-91.
Milhorat TH, Capocelli AL Jr, Kotzen RM. Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. Nov 1997;41(5):1102-10. [Medline].
Milhorat TH, Kotzen RM, Mu HT, et al. Dysesthetic pain in patients with syringomyelia. Neurosurgery. May 1996;38(5):940-6; discussion 946-7. [Medline].
Mueller D, Oro' JJ. Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia. Neurosurg Focus. Feb 15 2005;18(2):ECP2. [Medline].
Oakes WJ. Chiari malformation and syringomyelia. In: Rengachary SS, Wilkins RA, eds. Principles of Neurosurgery. St. Louis, Mo: Wolfe; 1994:9.1-9.17.
Ono A, Suetsuna F, Ueyama K, Yokoyama T, Aburakawa S, Numasawa T. Surgical outcomes in adult patients with syringomyelia associated with Chiari malformation type I: the relationship between scoliosis and neurological findings. J Neurosurg Spine. Mar 2007;6(3):216-21. [Medline].
Peñagarícano JA, Linskey ME, Ratanatharathorn V. Accelerated cerebral vasculopathy after radiation therapy to the brain. Neurol India. Dec 2004;52(4):482-6. [Medline].
Rhoton AL, Hamilton AJ. Chiari malformation and syringomyelia. In: Benzel EC, ed. Spine Surgery: Techniques, Complication Avoidance, and Management. 2. Boston: Churchill-Livingstone; 1999:793-812.
Rusbridge C, Greitz D, Iskandar BJ. Syringomyelia: current concepts in pathogenesis, diagnosis, and treatment. J Vet Intern Med. May-Jun 2006;20(3):469-79. [Medline].
Simon RP, Aminoff MJ, Greenberg DA. Clinical Neurology. 4th ed. Norwalk, Conn: Appleton-Lange; 1999:220-1.
Sudo K, Miyazaki Y, Tajima Y. Spontaneous resolution of idiopathic syringomyelia. Neurology. May 28 2002;58(10):1576-7; author reply 1577. [Medline].
Wisoff JH. Chiari Malformations and Hydromyelia. In: Tindall GT, Cooper PR, Barrow D, eds. The Practice of Neurosurgery. 3. Baltimore: Williams & Wilkins; 1995:2743-53.
Wisoff JH, Epstein F. Management of hydromyelia. Neurosurgery. Oct 1989;25(4):562-71. [Medline].
Further Reading
Keywords
syringomyelia, hydromyelia, syrinx, syringohydromyelia, syringocephalus, syringobulbia
Differential Diagnoses & Workup: Syringomyelia