Syringomyelia Clinical Presentation
- Author: Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE; Chief Editor: Selim R Benbadis, MD more...
Syringomyelia usually progresses slowly; the course may extend over many years. The condition may have a more acute course, especially when the brain stem is affected (i.e., syringobulbia). Syringomyelia usually involves the cervical area. Symptomatic presentation depends primarily on the location of the lesion within the neuraxis. Clinical manifestations are discussed in the sections that follow.
Syrinx interrupts the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in loss of these sensations, while light touch, vibration, and position senses are preserved (dissociated sensory loss).
When the cavity enlarges to involve the posterior columns, position and vibration senses in the feet are lost; astereognosis may be noted in the hands.
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.
Dysesthetic pain, a common complaint in syringomyelia, usually involves the neck and shoulders, but may follow a radicular distribution in the arms or trunk. The discomfort, which is sometimes experienced early in the course of the disease, generally is deep and aching and can be severe.
Syrinx extension into the anterior horns of the spinal cord damages motor neurons (lower motor neuron) and causes diffuse muscle atrophy that begins in the hands and progresses proximally to include the forearms and shoulder girdles. Clawhand may develop.
Respiratory insufficiency, which usually is related to changes in position, may occur.
Impaired bowel and bladder functions usually occur as a late manifestation.
Sexual dysfunction may develop in long-standing cases.
Horner syndrome may appear, reflecting damage to the sympathetic neurons in the intermediolateral cell column.
Extension of the syrinx
A syrinx may extend into the medulla, producing a syringobulbia.[6, 7] This syndrome is characterized by dysphagia, nystagmus, pharyngeal and palatal weakness, asymmetric weakness and atrophy of the tongue, and sensory loss involving primarily pain and temperature senses in the distribution of the trigeminal nerve.
Rarely, the syrinx cavity can extend beyond the medulla in the brain stem into the centrum semiovale (syringocephalus).
Lumbar syringomyelia can occur and is characterized by atrophy of the proximal and distal leg muscles with dissociated sensory loss in the lumbar and sacral dermatomes. Lower limb reflexes are reduced or absent. Impairment of sphincter function is common.
Painless ulcers of the hands are frequent. Edema and hyperhidrosis can be due to interruption of central autonomic pathways.
Acute painful enlargement of the shoulder is associated with destruction of the head of the humerus.
A complete physical examination may reveal diminished arm reflexes, which are sometimes present early in the clinical course of syringomyelia.
Lower limb spasticity, which may be asymmetrical, appears with other long-tract signs such as paraparesis, hyperreflexia, and extensor plantar responses.
Rectal examination includes an evaluation of volitional sphincter control and sensory assessment of sacral dermatomes.
Dissociated sensory impairment may be noted.
The syrinx may extend into the brain stem, affecting cranial nerves or cerebellar function.
Brainstem signs are common in syringomyelia associated with Chiari malformations.
Nakanishi K, Uchiyama T, Nakano N, et al. Spinal syringomyelia following subarachnoid hemorrhage. J Clin Neurosci. 2012 Apr. 19(4):594-7. [Medline].
Kim J, Kim CH, Jahng TA, Chung CK. Clinical course of incidental syringomyelia without predisposing pathologies. J Clin Neurosci. 2012 Feb 29. [Medline].
Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. 1965 Jun. 28:247-59. [Medline].
Williams B. Progress in syringomyelia. Neurol Res. 1986 Sep. 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. 1994 Jan. 80(1):3-15. [Medline].
Viswanatha B. Syringomyelia with syringobulbia presenting as vocal fold paralysis. Ear Nose Throat J. 2009 Jul. 88(7):E20. [Medline].
Tubbs RS, Bailey M, Barrow WC, Loukas M, Shoja MM, Oakes WJ. Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia. Childs Nerv Syst. 2009 Jun. 25(6):689-92. [Medline].
Nacir B, Arslan Cebeci S, Cetinkaya E, Karagoz A, Erdem HR. Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomyelia and type I Arnold-Chiari malformation. Rheumatol Int. 2009 Jun 23. [Medline].
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. 2007 Mar. 6(3):216-21. [Medline].
Cardoso M, Keating RF. Neurosurgical management of spinal dysraphism and neurogenic scoliosis. Spine (Phila Pa 1976). 2009 Aug 1. 34(17):1775-82. [Medline].
Kiran NA, Kasliwal MK, Suri A, Mahapatra AK. Giant posterior fossa arachnoid cyst associated with syringomyelia. Clin Neurol Neurosurg. 2010 Feb 4. [Medline].
Lucchetta M, Cagnin A, Calderone M, Manara R, Rotilio A, Briani C. Syringomyelia associated with Chiari I malformation. Neurol Sci. 2009 Sep 19. [Medline].
Prat R, Galeano I. Pain improvement in patients with syringomyelia and Chiari I malformation treated with suboccipital decompression and tonsillar coagulation. J Clin Neurosci. 2009 Apr. 16(4):531-4. [Medline].
Falci SP, Indeck C, Lammertse DP. Posttraumatic spinal cord tethering and syringomyelia: surgical treatment and long-term outcome. J Neurosurg Spine. 2009 Oct. 11(4):445-60. [Medline].
Sixt C, Riether F, Will BE, Tatagiba MS, Roser F. Evaluation of quality of life parameters in patients who have syringomyelia. J Clin Neurosci. 2009 Oct 7. [Medline].
Attal N, Parker F, Tadie 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. 2004 Jul. 75(7):1025-30. [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. 2005 May 15. 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. 2003 Mar. 74(3):344-7. [Medline].
Colombo A, Cislaghi MG. Familial syringomyelia: case report and review of the literature. Ital J Neurol Sci. 1993 Dec. 14(9):637-9. [Medline].
Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. 2006 Oct. 29(4):251-63; discussion 264. [Medline].
Gruber DP, Crone KR. Neuroendoscopy. Grossman RG, Loftus CM, eds. Principles of Neurosurgery. 2nd ed. Philadelphia: Lippincott-Raven; 1998. 757-62.
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. 2005 Apr. 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. Herkowitz HN, Garfin SR, Balderston RA, et al, eds. The Spine. 4th ed. Philadelphia: WB Saunders Company; 1999. 2: 1431-59.
Mancall EL. Syringomyelia. Rowland LP, ed. Merritt's Textbook of Neurology. 8th ed. Philadelphia: Lea & Febiger; 1989: 687-91.
Milhorat TH, Capocelli AL Jr, Kotzen RM. Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. 1997 Nov. 41(5):1102-10. [Medline].
Milhorat TH, Kotzen RM, Mu HT, et al. Dysesthetic pain in patients with syringomyelia. Neurosurgery. 1996 May. 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. 2005 Feb 15. 18(2):ECP2. [Medline].
Oakes WJ. Chiari malformation and syringomyelia. Rengachary SS, Wilkins RA, eds. Principles of Neurosurgery. St. Louis, Mo: Wolfe; 1994. 9.1-9.17.
Penagaricano JA, Linskey ME, Ratanatharathorn V. Accelerated cerebral vasculopathy after radiation therapy to the brain. Neurol India. 2004 Dec. 52(4):482-6. [Medline].
Rhoton AL, Hamilton AJ. Chiari malformation and syringomyelia. Benzel EC, ed. Spine Surgery: Techniques, Complication Avoidance, and Management. Boston: Churchill-Livingstone; 1999. 2: 793-812.
Rusbridge C, Greitz D, Iskandar BJ. Syringomyelia: current concepts in pathogenesis, diagnosis, and treatment. J Vet Intern Med. 2006 May-Jun. 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. 2002 May 28. 58(10):1576-7; author reply 1577. [Medline].
Wisoff JH. Chiari Malformations and Hydromyelia. Tindall GT, Cooper PR, Barrow D, eds. The Practice of Neurosurgery. Baltimore: Williams & Wilkins; 1995. 3: 2743-53.