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Post infectious cervical instability, variants of the Grisel Syndrome, a link to post-infectious CCI?

pattismith

Senior Member
Messages
3,946
I wonder if post infectious CCI (@JenB @jeff_w ) could fall into the Grisel syndrome (and variants).

Thank you @Daffodil who pointed these case reports.

Here some papers on that topic:



Atlantoaxial Instability of Inflammatory Origin in Adults: Case Reports, Literature Review, and Rationale for Early Surgical Intervention
Article· Literature ReviewinNeurosurgery 76(2) · September 2014 


Abstract
Background and importance: Acquired atlantoaxial instability of inflammatory origin (Grisel syndrome) is a rare condition. It usually occurs in children with benign upper airway problems and responds well to immobilization, rarely requiring C1-2 arthrodesis. Our recent experience with 2 adult cases suggests this may not be true in an older subpopulation. Clinical presentation: A 71-year-old man developed C1-2 instability in the setting of culture-negative endocarditis. Initial immobilization was attempted for 8 weeks but new imaging revealed progressive destruction of the odontoid and worsening instability. Symptoms resolved after C1-4 arthrodesis. A 35-year-old woman developed C1-2 instability after a molar extraction and otitis media. Despite 12 weeks of immobilization and antibiotics, symptoms persisted and the atlantodental interval increased. She was successfully treated with a C1-2 arthrodesis. Conclusion: A literature review revealed 13 reports (14 cases) of inflammatory atlantoaxial instability in patients aged 18 and older since 1830. Including the 2 cases reported here, 11 cases underwent initial nonoperative treatment with durable satisfactory results in only 2 of them (18.2%). Aspiration of the C1-2 phlegmon was diagnostic in only 4 of these 16 cases. Destruction of the odontoid was seen in a minority of cases (5/16, 31.3%). In this first review of the topic since the introduction of screw-based C1-2 fixation, it is suggested that nonoperative treatment is futile for inflammatory atlantoaxial instability in adults and strong consideration should be given to C1-2 arthrodesis. This procedure can reliably produce good outcomes with minimal morbidity.

Inflammatory C2-3 subluxation: A Grisel's syndrome variant
ArticleinArchives of Disease in Childhood 88(7):628-9 · August 2003


Abstract
The concurrence of non-traumatic atlanto-axial subluxation with inflammation of the adjacent neck tissues is known as Grisel's syndrome. We report a 5 year old boy with recurring episodes of head tilt and painful and restricted neck movements that developed after repeated bouts of sinusitis. Radiographs showed a subluxation of the C2-3 joint. Medical treatment, with cervical collar, physiotherapy, and non-steroid anti-inflammatory agents, led to complete cure of the disease. We suggest that Grisel's syndrome can occur in a location different from the classic atlanto-axial joint. To the best of our knowledge, this is the first report of a symptomatic case of Grisel's syndrome occurring at the C2-3 segment.


Midcervical Postinfectious Ligamentous Instability: A Variant of Grisel’s Syndrome
ArticleinPediatric Neurosurgery 29(3):133-7 · October 1998


Abstract
We report our recent experience with two cases of C3-C4 ligamentous instability that developed after the onset of an upper airway infection. To our knowledge, this is the first report in the literature of a variant of Grisel's syndrome occurring at the midcervical levels. The diagnosis and management of this entity are presented. Two infants with respiratory distress were evaluated at the Children's Hospital of Buffalo. During evaluation, cervical spine x-rays showed midcervical translational instability. Neither patient had a neurological deficit nor a history of trauma or bone disease. One of the patients was immmobilized with an external orthosis. Each patient remained neurologically intact, and the cervical spine returned to normal alignment after several months. We discuss a possible mechanism for this unusual association and recommend external immobilization.
 

Sidny

Senior Member
Messages
176
Medical treatment, with cervical collar, physiotherapy, and non-steroid anti-inflammatory agents, led to complete cure of the disease

This sounds awesome but considering the outcome was for a 5 year old boy who is rapidly growing I don’t think it will resolve as easily for adults. Too bad because I don’t think I’d fare too well in surgery, I have doubts I could even survive it.

Including the 2 cases reported here, 11 cases underwent initial nonoperative treatment with durable satisfactory results in only 2 of them (18.2%)

Damn, wish non surgical interventions were more effective.
 

Daffodil

Senior Member
Messages
5,875
This sounds awesome but considering the outcome was for a 5 year old boy who is rapidly growing I don’t think it will resolve as easily for adults. Too bad because I don’t think I’d fare too well in surgery, I have doubts I could even survive it.



Damn, wish non surgical interventions were more effective.
I cant afford surgery so am going to try stem cells at regenexx. you never know...
 

pattismith

Senior Member
Messages
3,946
@pattismith Great work there. So there is precedent set for infections causing CCI! This could go useful in trying to convince the doctors for further testing as I reached a sticking point with a doctor saying infections couldn't cause connective tissue problems in the neck.

I should have done a synthesis of the studies, in fact Grisel syndrome is a post infectious cervico-cervical C1-C2 instability. Variants of the Grisel syndrome are mostly post infectious cervico-cervical C2-C3 or C3-C4 instability. CCI is almost never involved (maybe one case if I remember well).

So I wonder if post infectious CCI cases went just unnoticed, undiagnosed, under all the radars, because of diagnosis difficulties.


This sounds awesome but considering the outcome was for a 5 year old boy who is rapidly growing I don’t think it will resolve as easily for adults. Too bad because I don’t think I’d fare too well in surgery, I have doubts I could even survive it.



Damn, wish non surgical interventions were more effective.

The review of adults cases says surgery gives better results in adults, but it concerns mainly C1-C2 post infectious instability.
@Hip , I was thinking of you when I read about this case, I wonder if this endocarditis could be the result of an enterovirus.

"A 71-year-old man developed C1-2 instability in the setting of culture-negative endocarditis. Initial immobilization was attempted for 8 weeks but new imaging revealed progressive destruction of the odontoid and worsening instability. Symptoms resolved after C1-4 arthrodesis. "
 

Hip

Senior Member
Messages
17,874
I wonder if post infectious CCI (@JenB @jeff_w ) could fall into the Grisel syndrome (and variants).

Very interesting finding.

This article on Grisel syndrome states:
Aetiology

The syndrome may result from any inflammatory process of the head and neck. The most common aetiologies are as follows:

upper respiratory tract infections
tonsillectomy/adenotonsillectomy
otitis media
other ENT infections/surgery

The causative organisms are usually:

Staphylococcus aureus
Group B. Streptococcus
oral flora

I wonder if the chronic sore throat commonly found in ME/CFS could be considered one of these upper respiratory tract infections or inflammatory processes that could lead to Grisel syndrome?

Moreover, could the inflammation in the chronic sore throat of ME/CFS be the cause of the CCI/AAI that, on this forum, we are finding in ME/CFS?

Plus some ME/CFS patients report an inflammatory feeling at the back of the head, around the nape of the neck, so that's another head/neck location where there may be ongoing inflammation.



The torticollis caused by Grisel syndrome has a distinctive tilt and rotation of the neck appearance — see these images. It seems that by definition, Grisel syndrome only involves atlantoaxial subluxation, but not craniocervical effects.
Torticollis
canva-Right-torticollis.png



But I think these Grisel syndrome papers you quoted are a very interesting finding, as they make it clear that lax ligaments in the atlantoaxial joint can be caused by infection and inflammation.
 
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pattismith

Senior Member
Messages
3,946
The torticollis caused by Grisel syndrome has a distinctive tilt and rotation of the neck appearance — see these images. It seems that by definition, Grisel syndrome only involves atlantoaxial subluxation, but not craniocervical effects.
But I think these Grisel syndrome papers you quoted are a very interesting finding, as they make it clear that lax ligaments in the atlantoaxial joint can be caused by infection and inflammation.

I also quoted papers about Grisel syndrome variants (involving C2-C3 instability and C3-C4 instability).

This is the reason why I suggested that post-infectious CCI might be an undiagnosed Grisel syndrome variant, that could be unrecognized because of the existing difficulties to make the CCI diagnostic.(C1-C2 instability is certainly the easiest neck instability to diagnose, because of the obvious postural defect that occurs)
----------------
Here another interesting case caused by M. Pneumoniae, (and Epstein B virus is also quoted as a possible cause in the syndrome).

Grisel's syndrome caused by Mycoplasma pneumoniae infection: a case report and review of the literature.
2019

Abstract
BACKGROUND:
Grisel's syndrome is a non-traumatic subluxation of the atlantoaxial joints, which is caused by an inflammatory process involving the upper neck. Torticollis, neck pain, and reduced neck mobility are the main clinical signs of presentation. Predisposing factors are trauma, hyperlaxity of the transverse and alar ligaments of the atlantoaxial joints, and surgical interventions carried out in this area.

Several viral and bacterial pathogens have been reported as causative events of Grisel's syndrome, including Epstein-Barr virus, Kawasaki disease, Streptococcus pyogenes, Staphylococcus aureus, and other infectious agents. Grisel's syndrome linked to Mycoplasma pneumoniae infection as the trigger has not previously been reported.
Mycoplasma pneumoniae is a small prokaryotic microbe and a frequent etiologic factor of respiratory tract infections and, less frequently, of extrapulmonary body organs. The recognition of the Grisel's syndrome is based on clinical and neuroradiological investigations, and early diagnosis and specific treatment are crucial to the successful outcome of the disease.
RESULTS:
We report the case of an 8-year-old girl with Grisel's syndrome caused by an upper respiratory tract infection due to Mycoplasma pneumoniae. Diagnostic suspicion and treatment of Grisel's syndrome were established quickly by anamnestic and clinical data and confirmed by radiological findings. The girl was immediately treated with specific antibiotic therapy and cervical immobilization, thus preventing the most dangerous complications of the disorder.
CONCLUSION:
Mycoplasma pneumoniae, among the other infectious agents, may be cause of scute torticollis and Gresel's syndrome.
 
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Hip

Senior Member
Messages
17,874
I also quoted papers about Grisel syndrome variants (involving C2-C3 instability and C3-C4 instability).

Yes, I noticed those: occasionally you get joint subluxation further down the spine in Grisel syndrome, but for some reason, this syndrome does not seem to affect the joint between the skull and spine (craniocervical joint).

Maybe that's simply because when the craniocervical joint is affected, you get different symptoms, and then the whole process is given a different name (such as cervical medullary syndrome, which is the set of symptoms craniocervical instability causes).
 

pattismith

Senior Member
Messages
3,946
Yes, I noticed those: occasionally you get joint subluxation further down the spine in Grisel syndrome, but for some reason, this syndrome does not seem to affect the joint between the skull and spine (craniocervical joint).

Maybe that's simply because when the craniocervical joint is affected, you get different symptoms, and then the whole process is given a different name (such as cervical medullary syndrome, which is the set of symptoms craniocervical instability causes).

I did some researches about "cervical medullary syndrome", and found only two published papers about it, by Henderson, one in 2017 and one in 2019. Henderson states that this syndrome is associated to CCI in hereditary hypermobile patients.
So this syndrome is a newly description, and is not associated with any infection in the description.
 

Hip

Senior Member
Messages
17,874
I did some researches about "cervical medullary syndrome", and found only two published papers about it, by Henderson, one in 2017 and one in 2019. Henderson states that this syndrome is associated to CCI in hereditary hypermobile patients.
So this syndrome is a newly description, and is not associated with any infection in the description.

Yes, from what I could make out, "cervical medullary syndrome" seems to be a phrase or syndrome coined by Henderson, but if you search "medullary syndrome" that does exist. I am not sure of the relation though.


Cervical medullary syndrome does appear to be associated with infection: in this 2012 video at 13:12, Dr Henderson says says there are three components to diagnosing craniocervical instability:

1 — Clinical: the presence of cervical medullary syndrome (symptoms: headache, neck pain, visual changes, photosensitivity, hearing loss, tinnitus, hyperacusis, dizziness, vertigo, imbalance, dysarthria, word finding difficulties, dysphagia, choking, dyspnea, disordered CO2 regulation, sleep apnea, disordered sleep architecture, loss of REM sleep, gastric reflux, nausea, vomiting, IBS, weakness, spasticity, tremors, jerking, clinic movements and dystonia, sensory loss, hypersensitivity, RSD, Raynaud's, memory and cognitive issues).

2 — Radiological (MRI scan): metrics of instability, pannus, syringomyelia, spina bifida.

3 — Etiological: a reasonable explanation for ligamentous instability (such as inflammation, rheumatoid arthritis, lupus, infection, etc).

So they think infection or inflammation can cause the lax ligaments that lead to CCI/AAI.
 

pattismith

Senior Member
Messages
3,946
Yes, from what I could make out, "cervical medullary syndrome" seems to be a phrase or syndrome coined by Henderson, but if you search "medullary syndrome" that does exist. I am not sure of the relation though.


Cervical medullary syndrome does appear to be associated with infection: in this 2012 video at 13:12, Dr Henderson says says there are three components to diagnosing craniocervical instability:

1 — Clinical: the presence of cervical medullary syndrome (symptoms: headache, neck pain, visual changes, photosensitivity, hearing loss, tinnitus, hyperacusis, dizziness, vertigo, imbalance, dysarthria, word finding difficulties, dysphagia, choking, dyspnea, disordered CO2 regulation, sleep apnea, disordered sleep architecture, loss of REM sleep, gastric reflux, nausea, vomiting, IBS, weakness, spasticity, tremors, jerking, clinic movements and dystonia, sensory loss, hypersensitivity, RSD, Raynaud's, memory and cognitive issues).

2 — Radiological (MRI scan): metrics of instability, pannus, syringomyelia, spina bifida.

3 — Etiological: a reasonable explanation for ligamentous instability (such as inflammation, rheumatoid arthritis, lupus, infection, etc).

So they think infection or inflammation can cause the lax ligaments that lead to CCI/AAI.

that's interesting, but still not widely recognized I think.
Did you find any post infectious CCI case published under the label of cervical medullary syndrome?
 

Hip

Senior Member
Messages
17,874
Did you find any post infectious CCI case published under the label of cervical medullary syndrome?

No I have not seen any specific cases mentioned or published, but the fact that infection is listed by Dr Henderson as a possible cause of the lax ligaments that lead to CCI/AAI suggests that he may have seen cases like this.

In this post I suggest that the MMP connective-tissue degrading enzymes which the immune system secretes in response to infection may explain why ligaments can be weakened by infection.
 

Daffodil

Senior Member
Messages
5,875
@Hip where does dr. H say the infection could be a cause of the lax ligaments? Is this in his paper?

i wish i had the full use of my brain right now...so much research to be done :-/

thank you
 

Daffodil

Senior Member
Messages
5,875
does anyone know if AAI can have causes other than lax ligaments? like aside from downs syndrome or some congenital structural issue.
 

Hip

Senior Member
Messages
17,874
@Hip where does dr. H say the infection could be a cause of the lax ligaments? Is this in his paper?

It's just in the slide found at timecode 13:12 in this 2012 video by Dr Henderson, where it lists various "reasonable explanations for ligamentous instability" — explanations which include: inflammation, rheumatoid arthritis, lupus, infection.

But that is the only place where I have seen the suggestion that infection can create the lax ligaments of CCI/AAI.
 

Daffodil

Senior Member
Messages
5,875
It's just in the slide found at timecode 13:12 in this 2012 video by Dr Henderson, where it lists various "reasonable explanations for ligamentous instability" — explanations which include: inflammation, rheumatoid arthritis, lupus, infection.

But that is the only place where I have seen the suggestion that infection can create the lax ligaments of CCI/AAI.
oh ok ok yes now i remember that line. thanks!