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Related Experiment Videos

Adult rhombencephalosynapsis. Case report.

L L Guyot1, C D Kazmierczak, D B Michael

  • 1Department of Neurological Surgery, Detroit Receiving Hospital and Wayne State University School of Medicine, Michigan 48201, USA.

Journal of Neurosurgery
|August 10, 2000
PubMed
Summary

Rhombencephalosynapsis (RS) is a rare cerebellar disorder. Surgical decompression successfully relieved a symptomatic adult patient

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Neurosurgery

Background:

  • Rhombencephalosynapsis (RS) is a rare cerebellar malformation characterized by fused hemispheres and absent vermis.
  • RS can be associated with hydrocephalus and other neurological abnormalities.

Observation:

  • A symptomatic adult with RS presented with severe pain and subtle gait ataxia.
  • MR imaging revealed fused cerebellar hemispheres, absent vermis, and mild hydrocephalus with altered CSF flow.
  • Preoperative ICP was normal, but postoperative ICP increased following suboccipital decompression and duraplasty.

Findings:

  • The patient experienced sustained symptomatic relief for 3 years post-surgery.
  • Postoperative cine-MR imaging showed improved CSF dynamics.
  • Surgical intervention for RS in adults can lead to significant pain relief.

Implications:

  • This case suggests that surgical decompression may be a viable treatment for symptomatic adult RS patients.
  • Altered posterior fossa pressure, rather than generalized hydrocephalus, may be the primary cause of symptoms in some adult RS cases.
  • Further research is warranted to explore surgical outcomes in adult RS patients.

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