eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Syringomyelia: Differential Diagnoses & Workup

Author: Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE, Assistant Professor, Department of Neurosurgery, Suez Canal University; Co-Director, Center of Research and Development in Medical Education and Health Services Suez Canal University Hospital
Coauthor(s): Ayman Ali Galhom, MD, PhD, Lecturer (Associated Professor), Department of Neurosurgery, Suez Canal University Faculty of Medicine, Egypt; 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
Contributor Information and Disclosures

Updated: Sep 24, 2008

Differential Diagnoses

Acute Inflammatory Demyelinating Polyradiculoneuropathy
Medulloblastoma
Amyotrophic Lateral Sclerosis
Meningioma
Ankylosing Spondylitis
Metastatic Disease to the Spine and Related Structures
Arteriovenous Malformations
Motor Evoked Potentials
Atlantoaxial Instability in Individuals with Down Syndrome
Multiple Sclerosis
Brainstem Gliomas
Neural Tube Defects
Central Pontine Myelinolysis
Neurological History and Physical Examination
Cervical Spondylosis: Diagnosis and Management
Spinal Cord Hemorrhage
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Spinal Cord Infarction
Diabetic Neuropathy
Spinal Cord Trauma and Related Diseases
Ependymoma
Spinal Epidural Abscess
Hydrocephalus
Spinal Muscular Atrophy
Limb-Girdle Muscular Dystrophy

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

References

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Further Reading

Keywords

syringomyelia, hydromyelia, syrinx, syringohydromyelia, syringocephalus, syringobulbia

Contributor Information and Disclosures

Author

Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE, Assistant 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.

Medical Editor

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison
Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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: Nothing to disclose.

 
 
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