Invisible Illness Info Repository

Ehlers-Danlos Syndrome (EDS) => Migraines, headaches => Topic started by: Ryan Ryder on August 23, 2015, 11:09:18 PM

Title: Chiari / Atlantoaxial / Craniocervical Junction Abnormalities Instability & Defo
Post by: Ryan Ryder on August 23, 2015, 11:09:18 PM
Title: Trauma at the Craniocervical Junction
Post by: Ryan Ryder on August 23, 2015, 11:12:59 PM
Trauma at the Craniocervical Junction

Slideshow Presentation

Anver Jameel, MD
Thomas Jefferson University


Title: Re: Craniocervical Junction Abnormalities
Post by: Ryan Ryder on August 23, 2015, 11:13:33 PM
Craniocervical Junction Abnormalities

by Michael Rubin, MDCM

Merck and the Merck Manuals

May 2014 (Last full review/revision by Michael Rubin, MDCM)

Cervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease the space for the lower brain stem and cervical cord.

These abnormalities can result in neck pain; syringomyelia; cerebellar, lower cranial nerve, and spinal cord deficits; and vertebrobasilar ischemia. Diagnosis is by MRI or CT.

Treatment often involves reduction, followed by stabilization via surgery or an external device.

Neural tissue is flexible and susceptible to compression. Craniocervical junction abnormalities can cause or contribute to cervical spinal cord or brain stem compression; some abnormalities and their clinical consequences include the following:

Fusion of the atlas (C1) and occipital bone: Spinal cord compression if the anteroposterior diameter of the foramen magnum behind the odontoid process is < 19 mm

Basilar invagination (upward bulging of the occipital condyles): A short neck and compression that can affect the cerebellum, brain stem, lower cranial nerves, and spinal cord

Atlantoaxial subluxation or dislocation (displacement of the atlas anteriorly in relation to the axis): Acute or chronic spinal cord compression

Klippel-Feil malformation (fusion of cervical vertebrae): Deformity and limited motion of the neck but usually no neurologic consequences

Platybasia (flattening of the skull base so that the angle formed by the intersection of the clival and anterior fossa planes is > 135°), seen on lateral skull imaging: No symptoms or cerebellar or spinal cord deficits or normal-pressure hydrocephalus

Structural abnormalities include the following:

Os odontoideum (anomalous bone that replaces all or part of the odontoid process)

Atlas assimilation (congenital fusion of the atlas and occipital bone)

Congenital Klippel-Feil malformation (eg, with Turner or Noonan syndrome), often associated with atlanto-occipital anomalies

Atlas hypoplasia

Chiari malformations (descent of the cerebellar tonsils or vermis into the cervical spinal canal, sometimes associated with platybasia—see Hydrocephalus)

Title: What is Craniocervical Instability?
Post by: Ryan Ryder on August 23, 2015, 11:26:50 PM
What is Craniocervical Instability?

The Pain Relief Foundation


Craniocervical Instability (CCI), also known as the Syndrome of Occipitoatlantialaxial Hypermobility, is a structural instability of the craniocervical junction which may lead to a pathological deformation of the brainstem, upper spinal cord, and cerebellum. It primarily occurs in patients with Ehlers-Danlos Syndrome and other hereditary disorders of connective tissue.

Title: Re: Craniocervical Junction Abnormalities
Post by: Ryan Ryder on November 22, 2015, 11:40:22 PM

Ehlers-Danlos, CSF and MS

"Ehlers-Danlos Syndrome is one of many different types of inherited and acquired disorders, as well as degenerative conditions (aging and wear and tear) of the craniocervical junction (skull and upper cervical spine junction)..."

Title: Chiari 1 malformation
Post by: Ryan Ryder on November 22, 2015, 11:52:46 PM
Chiari 1 malformation

By Gail Ions, M.C.S.P. with an introduction by T.H. Milhorat, M.D.

Ehlers Danlos Support UK

Introduction Hereditary disorders of connective tissue (HDCT) have long attracted interest and speculation.

In recent years, evidence has been accumulating that a sub-population of patients suffering from these conditions will develop symptoms and signs of Chiari 1 Malformation (CM1) attributable to hypermobility of the occipitoatlantoaxial joints at the base of the skull.

Significant degrees of joint deficiency can lead in turn to cranial settling with descent of the cerebellar tonsils into the upper cervical spinal canal, mimicking the more common examples of CM1 caused by underdevelopment of the posterior cranial fossa.

Incomplete diagnostic criteria for HDCT, especially as they relate to the large joints, and ongoing controversies over the radiographic definition of CM1 as well as the optimal methods for demonstrating cranial settling, have limited advances in our understanding of this association.

Mrs. Gail Ions, M.C.S.P. has summarized these issues nicely in a well-researched and informative chapter on the association of Ehlers-Danlos syndrome and CM1 that should be of interest to all patients with HDCT.

Title: Chiari Malformation & EDS
Post by: Ryan Ryder on November 22, 2015, 11:53:46 PM
Chiari Malformation & EDS

Posted By Alan Hakim

April 15, 2014

This article is written for both patients with Ehlers-Danlos Syndrome (EDS), healthcare professionals, and the general public. It describes the nature of type 1 Chiari malformation, the symptoms that can be associated with it, what we currently understand about the association with EDS, how it is identified, and the treatment options.

What is a Chiari malformation?

In some people the lower part of the brain can sit much lower than normal so that it is below the entrance to the skull. This is called a Chiari malformation (CM). It may cause no problem at all, however in this position it can put pressure on the lowest part of the brain and the top of the spinal cord and cause symptoms.

There are 3 types of CM.  Type 1 CM is the most common and the least serious form of the condition. In type 1 CM the lowest part of the back of the brain called the cerebellar tonsils is found to lie at the top of the spinal canal rather than the normal position inside the base of the skull. There may also be tethering of the spinal cord. Here the cord has become abnormally attached within the spine and is less free to move with movement of the spine. There occasionally may also be a syrinx – a fluid filled cavity in the spinal cord or brain stem.

Type 2 and 3 CM are less common. They are more serious. They are associated with a condition called spina bifida, a birth defect of spine development, and a condition called hydrocephalus that describes a build-up of fluid in the brain.

This article focuses on type 1 CM. To read more about CM in general take a look at the NHS page by clicking HERE, or the National Institutes of Health page HERE.

Title: Discrepancies of MRI findings between recumbent and upright positions in atlanto
Post by: Ryan Ryder on January 21, 2016, 03:13:05 AM
Discrepancies of MRI findings between recumbent and upright positions in atlantoaxial lesion. Report of two cases

Fumio Suzuki,corresponding author Tadateru Fukami, Atsusi Tsuji, Kenji Takagi, and Masayuki Matsuda

European Spine Journal
Eur Spine J. 2008 Sep; 17(Suppl 2): 221–224. Published online 2008 Aug 20.


Two cases of atlantoaxial (A-A) instability that showed different MRI findings between supine and upright positions are presented. The upright MRI represented the findings corresponding to their symptoms. In A-A lesions, conventional MR images taken in the supine position do not always explain the pathophysiological consequences. The MR images taken in the upright position disclose the actual spinal pathophysiology with gravitational effects.

Keywords: Dynamic study, Upright MRI, Atlantoaxial dislocation


....It is shown here that in patients with A-A dislocation the MR images taken in supine position do not always make clear the pathophysiology. In anterior A-A dislocation spinal cord is usually compressed in neck flexion but not in extension [4]

MRI can provide significant details regarding bony and/or soft tissue compressions in cervical spine, and the dynamic study might provide additional information in some cases.....

Especially in craniocervical junction pathology, care must be taken to interpret the findings of MRI in recumbent position that cannot always represent the real pathology.

For accurate understanding of A-A instability and planning the operative procedure, s-MRI should be evaluated by the actual dynamics, using the modality such as plain X-rays taken in the upright position, and by through neurological examination [1, 4].

The u-MRI represents the spinal cord compression in dynamic neck movement much more precisely than s-MRI that could be a powerful tool to reveal the real dynamic pathophysiology of A-A instability.


In A-A lesions, s-MRI does not always explain the pathophysiological consequences without the evaluation of dynamic study in upright position, such as plain X-rays. The dynamic u-MRI gives additonal informations of the actual pathophysiology with gravitational effects of the head.

Title: Combined occipital-cervical and atlantoaxial disassociation without neurologic i
Post by: Ryan Ryder on January 21, 2016, 03:13:52 AM
Combined occipital-cervical and atlantoaxial disassociation without neurologic injury: case report and review of the literature.

Bisson E1, Schiffern A, Daubs MD, Brodke DS, Patel AA.

Spine (Phila Pa 1976)

Apr 15, 2010


Case report and literature review

To discuss the diagnosis, limitations, and treatment of combined occipital-cervical and atlantoaxial disassociation with normal neurologic function.

Injuries to the craniocervical junction can lead to devastating neurologic deficits. Occipital-cervical disassociation is a well-documented injury pattern that can lead to pain, spinal cord injury, and death. Early diagnosis and treatment can preserve neurologic function. Combined injuries to both the occipital-cervical and atlantoaxial segments are less common and, to date, have only been described with severe neurologic injury.

Retrospective review of a case. Literature review was performed through Medline and Pubmed searches

This is the first case to present a combined occipital-cervical and atlantoaxial disassociation with a neurologically intact patient. Initial physical examination was limited, but early imaging revealed evidence of instability. Early diagnosis and early (< 24) surgical stabilization was performed with no complications and neurologic preservation. One-year follow-up visit revealed normal neurologic examination with neck pain VAS = 2/10 and neck disability index = 6.

Combined injuries to the occipital-cervical and atlantoaxial can result in upper cervical instability. Despite previous reports, neurologic preservation remains a possibility in this injury pattern. Limitations in physical examination and radiographic imaging persist, but early diagnosis and surgical stabilization may improve neurologic outcomes.

Title: Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Ce
Post by: Ryan Ryder on January 21, 2016, 03:15:45 AM
Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Cervical Instability

Danielle Steilen,* Ross Hauser,* Barbara Woldin, and Sarah Sawyer

Open Orthop J.

Oct 1, 2014 (Published online)


The use of conventional modalities for chronic neck pain remains debatable, primarily because most treatments have had limited success. We conducted a review of the literature published up to December 2013 on the diagnostic and treatment modalities of disorders related to chronic neck pain and concluded that, despite providing temporary relief of symptoms, these treatments do not address the specific problems of healing and are not likely to offer long-term cures. The objectives of this narrative review are to provide an overview of chronic neck pain as it relates to cervical instability, to describe the anatomical features of the cervical spine and the impact of capsular ligament laxity, to discuss the disorders causing chronic neck pain and their current treatments, and lastly, to present prolotherapy as a viable treatment option that heals injured ligaments, restores stability to the spine, and resolves chronic neck pain.

The capsular ligaments are the main stabilizing structures of the facet joints in the cervical spine and have been implicated as a major source of chronic neck pain. Chronic neck pain often reflects a state of instability in the cervical spine and is a symptom common to a number of conditions described herein, including disc herniation, cervical spondylosis, whiplash injury and whiplash associated disorder, postconcussion syndrome, vertebrobasilar insufficiency, and Barré-Liéou syndrome.

When the capsular ligaments are injured, they become elongated and exhibit laxity, which causes excessive movement of the cervical vertebrae. In the upper cervical spine (C0-C2), this can cause a number of other symptoms including, but not limited to, nerve irritation and vertebrobasilar insufficiency with associated vertigo, tinnitus, dizziness, facial pain, arm pain, and migraine headaches. In the lower cervical spine (C3-C7), this can cause muscle spasms, crepitation, and/or paresthesia in addition to chronic neck pain. In either case, the presence of excessive motion between two adjacent cervical vertebrae and these associated symptoms is described as cervical instability.

Therefore, we propose that in many cases of chronic neck pain, the cause may be underlying joint instability due to capsular ligament laxity. Currently, curative treatment options for this type of cervical instability are inconclusive and inadequate. Based on clinical studies and experience with patients who have visited our chronic pain clinic with complaints of chronic neck pain, we contend that prolotherapy offers a potentially curative treatment option for chronic neck pain related to capsular ligament laxity and underlying cervical instability.

Keywords: Atlanto-axial joint, Barré- Liéou syndrome, C1-C2 facet joint, capsular ligament laxity, cervical instability, cervical radiculopathy, chronic neck pain, facet joints, post-concussion syndrome, prolotherapy, spondylosis, vertebrobasilar insufficiency, whiplash.

Title: Atlanto-Axial Dislocation And Fracture: Causes, Symptoms, Diagnosis, Treatment,
Post by: Ryan Ryder on January 21, 2016, 03:16:27 AM
Atlanto-Axial Dislocation And Fracture: Causes, Symptoms, Diagnosis, Treatment, Prognosis

Pramod Kerkar, MD, FFARCSI

Pain Assist Inc. (ePainAssist)


The atlanto-axial joint is one of the complex and important joint in the body. The Atlantoaxial joint stabilizes the neck during activities and at rest. Ligaments and tendons supports Atlanto-axial joint. Non-displaced fracture or dislocation of Atlanto-axial joint results in severe pain, numbness and weakness in upper extremities. Displaced fracture or dislocation of the Atlanto-axial joint may cause life-threatening complications.1 In this article, we will discuss the anatomical details of Atlanto-axial Joint, causes, injuries and treatment of injuries resulting in fracture or dislocation of Atlanto-axial joint.

Title: Atlantoaxial Rotatory Subluxation
Post by: Ryan Ryder on January 21, 2016, 03:17:20 AM
Atlantoaxial Rotatory Subluxation


Musculoskeltal Health Newsletter

Also called atlantoaxial rotatory fixartion, atlantoaxial rotatory subluxation is a condition where there is a fixed rotation of C1 over C2. This condition occurs when normal motion between the atlas and axis becomes limited or fixed either  spontaneously or following minor trauma [which usually is the case]. It can also  or follow an upper respiratory tract infection. The cause of this subluxation is not completely understood. It is related to increased laxity of ligaments and capsular structures caused by inflammation or trauma.

Atlantoaxial rotatory subluxation is a common cause of childhood torticollis. Both the subluxation and torticollis usually are temporary. but rarely they persist and become  atlantoaxial rotatory fixation.

Title: Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari mal
Post by: Ryan Ryder on January 21, 2016, 03:18:11 AM
Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue.


Journal of Neurosurgery Spine

December 2007


Chiari malformation Type I (CM-I) is generally regarded as a disorder of the paraxial mesoderm. The authors report an association between CM-I and hereditary disorders of connective tissue (HDCT) that can present with lower brainstem symptoms attributable to occipitoatlantoaxial hypermobility and cranial settling.

The prevalence of HDCT was determined in a prospectively accrued cohort of 2813 patients with CM-I. All patients underwent a detailed medical and neuroradiological workup that included an assessment of articular mobility. Osseous structures composing the craniocervical junction were investigated morphometrically using reconstructed 3D computed tomography and plain x-ray images in 114 patients with HDCT/CM-I, and the results were compared with those obtained in patients with CM-I (55 cases) and healthy control individuals (55 cases).

The diagnostic criteria for Ehlers-Danlos syndrome and related HDCT were met in 357 (12.7%) of the 2813 cases. Hereditability was generally compatible with a pattern of autosomal dominant transmission with variable expressivity. The diagnostic features of HDCT/CM-I were distinguished from those of CM-I by clinical stigmata of connective tissue disease, a greater female preponderance (8:1 compared with 3:1, p < 0.001), and a greater incidence of lower brainstem symptoms (0.41 compared with 0.11, p < 0.001), retroodontoid pannus formation (0.71 compared with 0.11, p < 0.001), and hypoplasia of the oropharynx (0.44 compared with 0.02, p < 0.001).

Measurements of the basion-dens interval, basion-atlas interval, atlas-dens interval, dens-atlas interval, clivus-atlas angle, clivus-axis angle, and atlas-axis angle were the same in the supine and upright positions in healthy control individuals and patients with CM-I. In patients with HDCT/CM-I, there was a reduction of the basion-dens interval (3.6 mm, p < 0.001), an enlargement of the basion-atlas interval (3.0 mm, p < 0.001), and a reduction of the clivus-axis angle (10.8 degrees, p < 0.001), clivus-atlas angle (5.8 degrees, p < 0.001), and atlas-axis angle (5.3 degrees, p < 0.001) on assumption of the upright position. These changes were reducible by cervical traction or returning to the supine position.

The identification of HDCT in 357 patients with CM-I establishes an association between two presumably unrelated mesodermal disorders. Morphometric evidence in this cohort-cranial settling, posterior gliding of the occipital condyles, and reduction of the clivus-axis angle, clivus-atlas angle, and atlas-axis angle in the upright position-suggests that hypermobility of the occipitoatlantal and atlantoaxial joints contributes to retroodontoid pannus formation and symptoms referable to basilar impression.

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Title: Re: Chiari / Atlantoaxial / Craniocervical Junction Abnormalities Instability & Defo
Post by: Ryan Ryder on February 12, 2017, 11:57:44 PM
Cranio-cervical Instability in Patients with Hypermobility Connective Disorders

Fraser C. Henderson Sr. M.D.*


Pathological laxity of the cranio-
cervical junction Results in what patients describe as “bobble head”,
with neck and suboccipital pain, bulbar symptoms and myelopathy.

A recent Consensus Statement established that a group of symptoms-
the Cervical Medullary Syndrome - may be associated with cranio-
cervical instability (CCI) [2].

These symptoms are well established in
the literature: altered vision, hearing , speech, swallowing and balance,
the presence of vertigo , dizziness, altered sleep architecture, and signs
of dysautonomia -such as Postural Orthostatic Tachycardia Syndrome-
weakness and sensory loss.

The Consensus concluded that the presence
of the Cervical Medullary Syndrome should prompt consideration of a
disorder of the cranio-cervical junction.

Title: Re: Chiari / Atlantoaxial / Craniocervical Junction Abnormalities Instability & Defo
Post by: Ryan Ryder on February 12, 2017, 11:59:15 PM
Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis

Fraser C. Henderson, William A. Wilson,1 Stephen Mott,2 Alexander Mark,3 Kristi Schmidt,4 Joel K. Berry,5 Alexander Vaccaro,6 and Edward Benzel7


signs of cervical myelopathy (sensorimotor findings, hyper-reflexia); second, bulbar symptoms (lower cranial nerve dysfunction, respiratory disorder, changes in vision or tracking, auditory vestibular symptoms, dysautonomia) listed in Table 1;

there is support in the literature that memory is affected with alterations of the brainstem reticular activating system,[54,55] sleep abnormalities,[56] altered visual tracking or modulation of audition,[57,58] and chronic pain.[59]

Mechanical compression at the cervicomedullary junction occurs in Chiari 1 malformation,[2,6,10,16,61,62] achondroplasia[63–65] ; or as a result of basilar invagination, clival hypoplasia, anterior indentation of the pons, upward displacement of the brainstem or anterior displacement of the foramen magnum.[3,6,16,24,66,67]

Table 1
Brainstem Disability Index

The following 20 symptoms may be referable to pathology at the level of the brainstem.

Please indicate yes or no whether your child has any of the following symptoms on a recurring or chronic basis.

Double vision
Memory loss
Ringing in the ears
Speech difficulties
Difficulty swallowing
Sleep apnea
Snoring or frequent awakening
Choking on food
Hands turn blue in cold weather
Numbness in your arms and shoulders
Numbness in your back and legs
Gets tired very easily
Unsteady walking
More clumsy than you used to be Urinates more often (every 1-2 hours)
Irritable bowel disease or gastroesophageal reflux disease
Weaker than you would expect in your arms or hand
Weaker in your legs
5% for each positive response, 0%-100%

Title: Re: Chiari / Atlantoaxial / Craniocervical Junction Abnormalities Instability & Defo
Post by: Ryan Ryder on February 13, 2017, 12:18:40 AM
Neurosurgical Management of Hereditary Hypermobility Connective Tissue Disorders

Fraser C. Henderson

Title: Re: Chiari / Atlantoaxial / Craniocervical Junction Abnormalities Instability & Defo
Post by: Ryan Ryder on February 13, 2017, 12:30:06 AM
recognition-of-cranio-cervical-instability in the complex Chiari patient

Fraser C. Henderson