Orphanet: Primary syringomyelia

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Primary syringomyelia

Disease definition

A rare central nervous system malformation characterized by a fluid-filled longitudinally oriented cavity (syrinx) within the spinal cord, which may or may not communicate with the central canal, does not have an ependymal lining, and is either idiopathic or seen as a familial malformation. Clinical manifestations in symptomatic patients include neuropathic pain, as well as sensory and motor disturbances. Typical presentations may be cape-like loss of pain and temperature sensation along the torso and arms, or disproportionately greater motor impairment in upper compared to lower extremities.


Classification level: Disorder
  • Synonym(s):
    • Congenital syringomyelia
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: Q06.4
  • ICD-11: 8D66.0
  • OMIM: -
  • UMLS: C0039144
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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