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Neurology98 papers

Syringomyelia

Last edited: 4/14/2026

Overview

Syringomyelia is a neurological disorder characterized by the formation of fluid-filled cavities (syrinxes) within the spinal cord, often leading to neurological deficits and pain. It commonly occurs in the cervical region and can be associated with conditions such as Chiari malformation, spinal cord tumors, trauma, and, rarely, cervical disc disease 13.

Diagnosis

  • MRI: Essential for diagnosis, identifying syrinxes and associated abnormalities like cervical disc herniation or Chiari malformation 13.
  • Clinical Presentation: Includes sensory loss, muscle weakness, pain, and autonomic dysfunction 3.
  • Radiological Grading: Syrinx size and localization can be assessed using MRI; syrinx shapes classified as central, enlarged, or deviated 3.
  • HLA Typing: Considered in cases with suspected genetic predisposition; increased HLA-A9 frequency noted in patients with syringomyelia 7.
  • Management

  • Surgical Intervention: Primary treatment for symptomatic syringomyelia, particularly when associated with Chiari malformation or significant cord compression 3.
  • Postoperative Imaging: Monitoring changes in syrinx size and localization post-surgery to predict pain relief outcomes 3.
  • Pain Management: Multimodal approaches including NSAIDs, muscle relaxants, and possibly opioids for acute pain; long-term management tailored to individual symptoms 3.
  • Special Populations

  • Pediatrics: Specific data limited; management typically follows adult guidelines with close monitoring 4.
  • Comorbidities: Consideration of associated conditions like Chiari malformation or spinal cord tumors in treatment planning 34.
  • Key Recommendations

  • MRI is crucial for diagnosing syringomyelia and assessing associated abnormalities (Evidence: Strong 13).
  • Surgical decompression is recommended for symptomatic patients, especially those with Chiari malformation (Evidence: Moderate 3).
  • Postoperative MRI evaluation of syrinx changes can predict pain relief outcomes (Evidence: Moderate 3).
  • Genetic factors, such as HLA-A9 status, may influence syringomyelia development and warrant consideration in certain cases (Evidence: Weak 7).
  • Close monitoring is advised in pediatric cases due to limited specific data (Evidence: Expert opinion).
  • References

    1 Celal Iplikçioğlu A, Latifaci I, Karabağ H. Cervical syringomyelia associated with cervical disc disease. Ideggyogyaszati szemle 2024. link 2 Bosmia AN, Tubbs RI, Clapp DC, Batzdorf U, Loukas M, Tubbs RS. Johann Conrad Brunner (1653-1727) and the first description of syringomyelia. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2014. link 3 Nakamura M, Chiba K, Nishizawa T, Maruiwa H, Matsumoto M, Toyama Y. Retrospective study of surgery-related outcomes in patients with syringomyelia associated with Chiari I malformation: clinical significance of changes in the size and localization of syrinx on pain relief. Journal of neurosurgery 2004. link 4 Basu S, Nair N. Syringomyelia in caudal dysplasia sequence. The Journal of the Association of Physicians of India 2003. link 5 Mehta J, Khanna S. Syringomyelia as a cause of limb hypertrophy. Neurology India 2002. link 6 Busis NA, Hochberg FH. Familial syringomyelia. Journal of neurology, neurosurgery, and psychiatry 1985. link 7 Newman PK, Wentzel J, Foster JB. HLA and syringomyelia. Journal of neuroimmunology 1982. link90015-7) 8 Hampton F, Williams B, Loizou LA. Syncope as a presenting feature of hindbrain herniation with syringomyelia. Journal of neurology, neurosurgery, and psychiatry 1982. link 9 Bentley SJ, Campbell MJ, Kaufmann P. Familial syringomyelia. Journal of neurology, neurosurgery, and psychiatry 1975. link

    Original source

    1. [1]
      Cervical syringomyelia associated with cervical disc disease.Celal Iplikçioğlu A, Latifaci I, Karabağ H Ideggyogyaszati szemle (2024)
    2. [2]
      Johann Conrad Brunner (1653-1727) and the first description of syringomyelia.Bosmia AN, Tubbs RI, Clapp DC, Batzdorf U, Loukas M, Tubbs RS Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (2014)
    3. [3]
    4. [4]
      Syringomyelia in caudal dysplasia sequence.Basu S, Nair N The Journal of the Association of Physicians of India (2003)
    5. [5]
      Syringomyelia as a cause of limb hypertrophy.Mehta J, Khanna S Neurology India (2002)
    6. [6]
      Familial syringomyelia.Busis NA, Hochberg FH Journal of neurology, neurosurgery, and psychiatry (1985)
    7. [7]
      HLA and syringomyelia.Newman PK, Wentzel J, Foster JB Journal of neuroimmunology (1982)
    8. [8]
      Syncope as a presenting feature of hindbrain herniation with syringomyelia.Hampton F, Williams B, Loizou LA Journal of neurology, neurosurgery, and psychiatry (1982)
    9. [9]
      Familial syringomyelia.Bentley SJ, Campbell MJ, Kaufmann P Journal of neurology, neurosurgery, and psychiatry (1975)

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