New scientific article on the Neuro-Cranio-Vertebral Syndrome associated with Ehlers-Danlos Syndrome

Published by ICSEB at 30 May, 2025

Ehlers-Danlos Syndrome (EDS) includes a complex group of heterogeneous, hereditary disorders, characterised by fragility and increased elasticity of the soft connective tissues, which concerns us particularly with respect to the type with vertebral pathology and cranio-cervical instability.

What relationship exists between Ehlers-Danlos Syndrome (EDS) and Neuro-Cranio-Vertebral Syndrome (NCVS)?

Can eliminating the traction of the Filum terminale through the Filum System® treatment help patients with Ehlers-Danlos associated with NCVS?

The new article “Neuro-Cranio-Vertebral Syndrome Associated with Ehlers–Danlos Syndrome: Diagnosis and Treatment”, published by Miguel B. Royo-Salvador, Marco V. Fiallos-Rivera and Horia C. Salca, attempts to answer these questions, proposing to do so through the theory of Neuro-Cranio-Vertebral Syndrome in relation to collagen dysfunction in the Filum Terminale due to EDS, and the resulting treatment.

In the Biomed publication, the elasticity of the vertebral column is considered to cause greater elongation than that of the Filum terminale. Consequently, the filum terminale reaches the limit of its elasticity, transforming into caudal traction of the Central Nervous System resulting in a Neuro-Cranio-Vertebral Syndrome.

The authors describe for the first time the results observed in a group of patients operated on at ICSEB with the dual diagnosis of EDS and NCVS, compared to a group of patients with only NCVS.

According to the authors, patients in the sample with EDS and NCVS presented more cranial and vertebral symptoms than the other group. Significant differences were also observed in the neurological signs present in EDS-NCVS compared to those with only NCVS. Furthermore, patients who underwent sectioning of the filum terminale (SFT) with the minimally invasive Filum System® technique showed significant improvement in signs and symptoms (Fig.1).

Fig.1 Summary of the postoperative course in 4 patients with NCVS and EDS, operated on with SFT. Symptom progression (A) and sign progression (B). Green: disappearance/improvement; yellow: no change; red: worsening.

Royo et al. highlight how the concept of cranio-cervical instability due to EDS, therefore, does not explain a large number of neurological signs and symptoms, which instead appear to be more consistent with the theory of traction transmitted by the Filum Terminale to the entire Central Nervous System in NCVS.

“Surgical treatment would only involve sectioning the filum terminale, while cervical fusion would never be justified in these patients,” report our researchers in conclusion of the study.

We thank the Chiari & Scoliosis & Syringomyelia Foundation for continuing to support the research of our R&D&I Department, and the latter for conducting another interesting study!

You can find the full article at: https://institutchiaribcn.com/en/neuro-cranio-vertebral-syndrome-associated-ehlers-danlos-syndrome-diagnosis-treatment/

You can also find more information about EDS at: https://institutchiaribcn.com/en/ehlers-danlos-syndrome/