Syringomyelia Clinical Presentation

  • Author: Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Apr 10, 2012
 

History

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 (ie, syringobulbia). Syringomyelia usually involves the cervical area. Symptomatic presentation depends primarily on the location of the lesion within the neuraxis. Clinical manifestations include the following:

  • Sensory
    • Dissociated sensory loss: 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.
    • 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.
  • Motor
    • 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.
  • Autonomic
    • 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.
  • Other manifestations
    • Painless ulcers of the hands are frequent. Edema and hyperhidrosis can be due to interruption of central autonomic pathways.
    • Neurogenic arthropathies (Charcot joints) may affect the shoulder, elbow, or wrist.[8] Scoliosis is seen sometimes.[9, 10]
    • Acute painful enlargement of the shoulder is associated with destruction of the head of the humerus.
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Physical

  • Arm reflexes are diminished early in the clinical course.
  • 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.
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Causes

Etiology of syringomyelia often is associated with craniovertebral junction abnormalities.

  • Bony abnormalities
    • Small posterior fossa
    • Platybasia and basilar invagination
    • Assimilation of the atlas
  • Soft-tissue masses of abnormal nature
    • Tumors (eg, meningioma at foramen magnum)
    • Inflammatory masses
  • Neural tissue
    • Cerebellar tonsils and vermis herniation
    • Chiari malformation
  • Membranous abnormalities
    • Arachnoid cysts[11] , rhombic roof, or vascularized membranes
    • Posthemorrhagic or postinflammatory membranes
  • Other etiologies not associated with craniovertebral abnormalities
    • Arachnoid scarring related to spinal trauma
    • Arachnoid scarring related to meningeal inflammation
    • Arachnoid scarring related to surgical trauma
    • Subarachnoid space stenosis due to spinal neoplasm or vascular malformation
    • Subarachnoid space stenosis, with possible scarring, related to disk and osteophytic disease
    • Idiopathic
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Contributor Information and Disclosures
Author

Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE,  Associate Professor, Department of Neurosurgery, Suez Canal University; Co-Director, Center of Research and Development in Medical Education and Health Services Suez Canal University Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Ayman Ali Galhom, MD, PhD  Lecturer (Associated Professor), Department of Neurosurgery, Suez Canal University Faculty of Medicine, Egypt

Ayman Ali Galhom, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Franklin C Wagner, Jr, MD  Former Chief, Division of Spine and Spinal Cord Surgery, Former Professor, Department of Neurosurgery, University of Illinois at Chicago College of Medicine

Franklin C Wagner, Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Surgery of Trauma, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Sigma Xi, Society for Neuroscience, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Christopher Luzzio, MD  Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

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Sagittal T1-weighted image showing a thoracic syrinx.
 
 
 
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