Hereditary spinal arachnoid cysts, distichiasis, and lymphedema

Ann Neurol. 1980 Apr;7(4):340-3. doi: 10.1002/ana.410070410.


Familial congenital spinal arachnoid cysts causing progressive paraplegia are reported in two adolescent siblings as part of a hereditary syndrome. The other features of this unusual dominantly inherited disorder include double rows of eyelashes, partial ectropion of the lower eyelids, and acquired late-onset lymphedema of the lower extremities. The siblings' mother, who had roentgenographic evidence of a similar intraspinal lesion, was free of neurological symptoms but had the other characteristic features. Surgical treatment of the arachnoid cysts improves the progressive paraplegia.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Arachnoid*
  • Child
  • Cysts / complications
  • Cysts / genetics*
  • Ectropion / genetics
  • Eyelashes / abnormalities
  • Eyelids / abnormalities*
  • Female
  • Humans
  • Lymphedema / genetics*
  • Male
  • Paraplegia / etiology*
  • Spinal Cord Compression / etiology
  • Syndrome