II. Definitions

  1. Syringe
    1. Fluid-filled cavity
  2. Syringomyelia or Syrinx
    1. Syringe forms at inner portion of spinal cord, forming an expanding central cavity
    2. Chronic progressive spinal cord degeneration
  3. Syringobulbia
    1. Syringomyelia extending into Brainstem

III. Epidemiology

  1. Symptom onset in second and third decade

IV. Pathophysiology

  1. Results from alterations in CSF flow
  2. Cervical spinal cord most often affected

V. Causes

  1. Chiari I Malformation (accounts for 90% of cervical Syringomyelia cases)
  2. Foramen magnum narrowing
  3. Achondroplasia
  4. Intraspinal tumors
  5. Subarachnoid scarring
    1. Traumatic spine injury (e.g. Whiplash) or Hemorrhage
    2. Spinal Infection

VI. Symptoms

  1. Back Pain
  2. Back and Shoulder stiffness
  3. Provocative
    1. Postural Change
    2. Valsalva Maneuver

VII. Signs

  1. Sensory dissociation in neck, arms and upper trunk
    1. Touch and position sense preserved
    2. Pain and Temperature sense lost (Spinothalamic Tract)
  2. Anterior horn cell involvement
    1. Upper extremity changes
      1. Asymmetric Muscle atrophy, weakness of hands, arms (esp. intraosseous Muscles)
      2. Fasciculations
      3. Deep Tendon Reflexes absent in upper extremities
    2. Lower extremity
      1. Increased Muscle tone
      2. Hyperactive Deep Tendon Reflexes
  3. Corticospinal tract involvement
    1. Muscle spasticity
  4. Spinal sympathetic fiber involvement
    1. Trophic changes

VIII. Associated conditions: Spinal cord disorders

  1. Scoliosis
  2. Vertebral fusions
  3. Platybasia

IX. Diagnosis

  1. Contrast-Enhanced MRI Spine
    1. Syrinx will appear as fluid-filled, gliosis lined cavity
    2. Defines extent of Syrinx (between C2 and T1)
    3. Identifies associated congenital abnormalities
  2. Electromyography (EMG)
    1. Denervation
  3. Lumbar Puncture
    1. CSF usually normal
  4. Nerve Conduction
    1. Normal

X. Management

  1. Neurosurgery Consultation (urgent if functional decline)
  2. Treat underlying cause (e.g. Chiari Malformation)

XI. Patient Resources

  1. American Syringomyelia Alliance Project
    1. http://www.asap.org
    2. Phone: 1-800-272-7282
    3. Address: P.O. Box 1586, Longview, Texas, 75606

XII. References

  1. Girolami in Cotran (1999) Robbins Pathology, P. 1361
  2. Barkovich in Goldman (2000) Cecil Medicine, p. 2077
  3. Achar (2020) Am Fam Physician 102(1):19-28 [PubMed]

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Related Studies

Ontology: Syringes (C0039142)

Definition (UMD) Instruments designed to inject, infuse, or withdraw fluids, usually through a fixed or attached needle. Most syringes consist of a hollow, transparent cylinder (barrel) ended in a tip and a plunger (i.e., plunger syringes); other syringes deliver the fluids from a prefilled, changeable cartridge that is inserted into the barrel (i.e., cartridge syringes). Syringes consisting of either a barrel and a plastic or rubber bulb attached to one end (bulb/barrel syringes) or a rubber or plastic bulb ended in a tapped prolongation (bulb syringes) are also used for some applications. Syringes are usually manufactured from transparent plastic materials (e.g., polyethylene, polypropylene) or glass, but some are metallic or include metal parts. Syringes are frequently used for subcutaneous (i.e., hypodermic), intramuscular, or intravenous (either directly or through catheters) injection; irrigation of cavities or wounds; and aspiration/withdrawal of blood or other body fluids. Some syringes are specially designed for clinical laboratory applications, including general laboratory use and chromatography tests.
Definition (HL7V3.0) <p>A barrel with a plunger.</p>
Definition (NCI_NCI-GLOSS) A small hollow tube used for injecting or withdrawing liquids. It may be attached to a needle in order to withdraw fluid from the body or inject drugs into the body.
Definition (NCI) A device for the administration of drug products that consists of a rigid barrel fitted with septum with a plunger at one end and a seal or needle at the other end. The needle assembly may be part of the device or separate.
Definition (MSH) Instruments used for injecting or withdrawing fluids. (Stedman, 25th ed)
Concepts Medical Device (T074)
MSH D013594
SnomedCT 61968008, 348578004, 367564007
English Syringes, SYRINGE, Syringe, device, syringe, syringe (treatment), syringes, Syringe - instrument, Syringe - instrument (physical object), Syringe (physical object), Syringe, Syringe, device (physical object), Syringe, NOS
Swedish Sprutor
Czech insulinová pera, injekční stříkačky
Finnish Ruiskut
French Seringues
Polish Strzykawki
Japanese シリンジ, 注射器
Spanish jeringa - instrumento, jeringa - instrumento (objeto físico), jeringa (objeto físico), jeringa, Jeringas
Norwegian Sprøyter
German Spritzen
Italian Siringhe
Dutch Injectiespuit, Spuit, injectie-, Syrinx
Portuguese Seringas

Ontology: Syringomyelia (C0039144)

Definition (MEDLINEPLUS)

Syringomyelia is a rare disorder that causes a cyst to form in your spinal cord. This cyst, called a syrinx, gets bigger and longer over time, destroying part of the spinal cord. Damage to the spinal cord from the syrinx can cause symptoms such as

  • Pain and weakness in the back, shoulders, arms or legs
  • Headaches
  • Inability to feel hot or cold

Symptoms vary according to the size and location of the syrinx. They often begin in early adulthood.

Syringomyelia usually results from a skull abnormality called a Chiari I malformation. A tumor, meningitis or physical trauma can also cause it. Surgery is the main treatment. Some people also need to have the syrinx drained. Medicines can help ease pain. In some cases, there are no symptoms, so you may not need treatment.

Definition (NCI) A rare disorder characterized by the formation of a cyst in the spinal cord. It results in weakness, pain, and stiffness in the shoulders, arms, legs, or back. It may be associated with Chiari malformation. Other causes include spinal cord injury, inflammation, or tumor.
Definition (MSH) Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269)
Concepts Disease or Syndrome (T047)
MSH D013595
SnomedCT 155020007, 267695005, 111496009
English Syringomyelias, SYRINGOMYELIA, Myelosyringosis, syringomyelia, syringomyelia (diagnosis), Myelosyringoses, Syringomyelus, Syringomyelia [Disease/Finding], Spinal cord cavitation, Syringomyelia-anesthesia syndrome, Syringomyelia-anaesthesia syndrome, Syringomyelia (disorder), Syringomyelia
Swedish Syringomyeli
Japanese セキズイクウドウショウ, 脊髄空洞症
Czech syringomyelie, Syringomyelie, myelosyringoza
Finnish Syringomyelia
Italian SM, Siringomielia
Polish Jamistość rdzenia
Hungarian Syringomyelia
Norwegian Syringomyeli
French Gliomatose médullaire, Syringomyélie
Spanish mielosiringosis, siringomielia (trastorno), siringomielia, síndrome de anestesia - siringomielia, Siringomielia
Portuguese Siringomiela, Siringomielia
Dutch syringomyelie, Syringomyelie
German Syringobulbie, Syringomyelie

Ontology: Syringobulbia (C0270771)

Concepts Congenital Abnormality (T019) , Disease or Syndrome (T047)
SnomedCT 26594006
English Syringobulbia, syringobulbia (diagnosis), syringobulbia, Syringobulbia (disorder)
Dutch syringobulbie
French Syringobulbie
German Syringobulbie
Italian Siringobulbia
Portuguese Siringobulbia
Spanish Siringobulbia, siringobulbia (trastorno), siringobulbia
Japanese 延髄空洞症, エンズイクウドウショウ
Czech Syringobulbie
Hungarian Syringobulbia