CCI and Tethered Cord

Hypermobile patients of all types (EDS, HSD, Marfan, OI, Sticklers) are all quite prone to cranio-cervical instability (CCI), settling (CCS), Chiari malformation, tethered cord syndrome (TCS), and CSF leaks and leaking. Some people are struggling to avoid internal decapitation in some cases. Many have signs of CNS apnea as a result too.

(This makes sense if you think about the skull settling at night lying down, especially if you have a retroflexed odontoid that might be stabbing you in the back of the brain stem. I actually had to remember to breathe even in the day time at my worst in 2012. It slowly resolved as I took more vitamin C.)

Few doctors recognize and understand this yet. And yes, you could actually have ALL OF THE ABOVE IN THE SAME PATIENT in some rare but unfortunately cases. Do not let your doctors dismiss this or you! Updated March 8, 2023

Links on Cranio-cervical instability, Chiari, Tethered cord and CSF Leaks and leaking

• What is Craniocervical Instability? via the Pain Foundation
• Overview: Craniocervical Instability and Related Disorders – Chiari Bridges
• Tethered Cord Syndrome
• How Cranio-cervical misalignment affects fluid flow – Dr. Rosa DC 2016
• Presentation and physical therapy management of upper cervical instability in patients with symptomatic generalized joint hypermobility: International expert consensus recommendations January 2023 Russek, Block, Byrne et al
• Ehlers-Danlos, POTS, and Occult Cerbrospinal Fluid Leak: A Case Report 2017 Aggarwal Stanford
• Chiari 0 (occult Chiari) via Bobby Jones CSF (MD) blog
• Chiari 0 malformation with syringomyelia syringobulbia and syrinx cavity in pons 2016 Zhou, Wang, Li et al
• Tethered Cord Syndrome in Ehlers-Danlos – Diagnostic Criteria, Surgical Indication and Filum Pathology – Klinge, P. MD 2015 Conference slides
• Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study 2004 Schievink, Karp Gordon, Tourje
• How common is normal CSF pressure in spontaneious intracranial hypotension? (CSF leaks) 2015
• General Signs and Symptoms of a Spinal CSF Leak – Dr. Wouter Schievink 2014
• Hypermobility and Headache – Dr. Vernon Rowe, 2018
• Periventricular heterotopia: New Insights into Ehlers-Danlos Syndrome 2005
• Ehlers-Danlos, POTS and a Headache Cured – via American Academy of Pain Medicine
• Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders: 5-year follow-up after craniocervical reduction, fusion, and stabilization 2019 Henderson, Francomano, Tuchman et al
• Signs and Symptoms of Atlas Subluxation Complex 2019 Grayson Blom, DC
• The Anatomy of the Atlas Subluxation by UpCSpine 2019
• Undiagnosed Atlas Subluxation in Patient with Pain and Poor Myofascial Function 2019 Hartz, Sauer, Howe et al MD
• [VIDEO] Dr. Mandell’s Atlas technique – the Miracle Bone That Can Help Spinal Pain, Muscles, Body Organs – YouTube 2017
• A Case Study of Severe CCJ Instability and a PICL Procedure 2019 Chris Centeno, MD
• Learning Patience: Eighteen Months of Spinal CSF Leak Recovery (great insight into life post blood patching for one patient)
• Head, Neck, Brain, Spine – learn how to read MRIs and CTs of the head and neck
• A cautionary thread on chiropractic care for anyone, but especially the hypermobile community via @TheDudeDO 2021 (JG note: yes, SOME of us can benefit from SOME careful chiropractic care, some even a lot, BUT… in the wrong hands, we can be seriously harmed or even killed so… choose carefully, preferably one who truly understands hypermobility. And take great care around the head and neck! No high-velocity neck thrusts! Folks have been decapitated this way, no joke.)