Abnormal spinal cord motion at the craniocervical junction in hypermobile Ehlers-Danlos patients

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  • 1 Departments of Neurosurgery,
  • | 2 Neuropathology, and
  • | 3 Radiology, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, Rhode Island; and
  • | 4 Department of Neurosurgery, Medical School Hannover, Germany
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OBJECTIVE

The craniocervical junction (CCJ) is anatomically complex and comprises multiple joints that allow for wide head and neck movements. The thecal sac must adjust to such movements. Accordingly, the thecal sac is not rigidly attached to the bony spinal canal but instead tethered by fibrous suspension ligaments, including myodural bridges (MDBs). The authors hypothesized that pathological spinal cord motion is due to the laxity of such suspension bands in patients with connective tissue disorders, e.g., hypermobile Ehlers-Danlos syndrome (EDS).

METHODS

The ultrastructure of MDBs that were intraoperatively harvested from patients with Chiari malformation was investigated with transmission electron microscopy, and 8 patients with EDS were compared with 8 patients without EDS. MRI was used to exclude patients with EDS and craniocervical instability (CCI). Real-time ultrasound was used to compare the spinal cord at C1–2 of 20 patients with EDS with those of 18 healthy control participants.

RESULTS

The ultrastructural damage of the collagen fibrils of the MDBs was distinct in patients with EDS, indicating a pathological mechanical laxity. In patients with EDS, ultrasound revealed increased cardiac pulsatory motion and irregular displacement of the spinal cord during head movements.

CONCLUSIONS

Laxity of spinal cord suspension ligaments and the associated spinal cord motion disorder are possible pathogenic factors for chronic neck pain and headache in patients with EDS but without radiologically proven CCI.

ABBREVIATIONS

CCI = craniocervical instability; CCJ = craniocervical junction; EDS = Ehlers-Danlos syndrome; MDB = myodural bridge; SAS = subarachnoid space; SC = spinal cord; TEM = transmission electron microscopy.
Illustrations from Walker et al. (pp 80–90). © Barrow Neurological Institute, Phoenix, Arizona.

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Contributor Notes

Correspondence Petra M. Klinge: Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, RI. petra_klinge@brown.edu.

INCLUDE WHEN CITING Published online May 21, 2021; DOI: 10.3171/2020.10.SPINE201765.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

P.M.K. and A.M. contributed equally to this work.

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