Cervicocranial syndrome: Difference between revisions

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{{Infobox medical condition (new)
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'''Cervicocranial [[syndrome]]''' or ('''Craniocervical Junction Syndrome: CCJ syndrome''') is a [[Neurological disorder|neurological]] [[Disease|illness]]. It is a combination of [[Symptom|symptoms]] that are caused by an abnormality in the neck. The [[Bone|bones]] of the [[neck]] that are affected are [[cervical vertebrae]] (C1 - C7). This syndrome can be identified by confirming cervical bone shifts, collapsed cervical bones or misalignment of the cervical bone leading to improper functioning of [[cervical spinal nerves]].[https://greenbergregen.com/cervicocranial-syndrome/] Cervicocranial syndrome is either [[Birth defect|congenital]] or acquired (as a result of [[injury]] or [[disease]]). Some examples of diseases that could result in cervicocranial syndrome are [[Chiari disease|Chiari disease,]] [[Klippel–Feil syndrome|Klippel-Feil malformation]] <ref>{{Cite journal|last=Avellaneda Fernández|first=Alfredo|last2=Isla Guerrero|first2=Alberto|last3=Izquierdo Martínez|first3=Maravillas|last4=Amado Vázquez|first4=María Eugenia|last5=Barrón Fernández|first5=Javier|last6=Chesa i Octavio|first6=Ester|last7=De la Cruz Labrado|first7=Javier|last8=Escribano Silva|first8=Mercedes|last9=Fernández de Gamboa Fernández de Araoz|first9=Marta|last10=García-Ramos|first10=Rocío|last11=García Ribes|first11=Miguel|date=2009-12-17|title=Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment)|url=https://doi.org/10.1186/1471-2474-10-S1-S1|journal=BMC Musculoskeletal Disorders|volume=10|issue=1|pages=S1|doi=10.1186/1471-2474-10-S1-S1|issn=1471-2474|pmc=PMC2796052|pmid=20018097}}</ref> [[osteoarthritis]], and trauma.<ref name=":0">{{Cite journal|last=Rosa|first=Scott|last2=Baird|first2=John W.|last3=Harshfield|first3=David|last4=Chehrenama|first4=Mahan|date=2018-08-01|title=Craniocervical Junction Syndrome: Anatomy of the Craniocervical and Atlantoaxial Junctions and the Effect of Misalignment on Cerebrospinal Fluid Flow|url=https://www.intechopen.com/books/hydrocephalus-water-on-the-brain/craniocervical-junction-syndrome-anatomy-of-the-craniocervical-and-atlantoaxial-junctions-and-the-ef|journal=Hydrocephalus - Water on the Brain|language=en|doi=10.5772/intechopen.72890}}</ref><ref>{{Cite journal|last=Smoker|first=Wendy R. K.|last2=Khanna|first2=Geetika|date=2008-10|title=Imaging the craniocervical junction|url=https://pubmed.ncbi.nlm.nih.gov/18461336/|journal=Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery|volume=24|issue=10|pages=1123–1145|doi=10.1007/s00381-008-0601-0|issn=0256-7040|pmid=18461336}}</ref> Treatment options include [[Cervical collar|neck braces]], [[Analgesic|pain medication]] and [[surgery]]. The quality of life for individuals suffering from CCJ syndrome can improve through surgery.<ref>{{Cite journal|last=Henderson|first=Fraser C.|last2=Francomano|first2=C. A.|last3=Koby|first3=M.|last4=Tuchman|first4=K.|last5=Adcock|first5=J.|last6=Patel|first6=S.|date=2019|title=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|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6821667/|journal=Neurosurgical Review|volume=42|issue=4|pages=915–936|doi=10.1007/s10143-018-01070-4|issn=0344-5607|pmc=6821667|pmid=30627832}}</ref> [[File:Cervical spine Anterior.png|thumb|Cervical Vertebrae (C1 - C7)|206x206px]]
== Signs and Symptoms ==
Cervicocranial syndrome has a wide range of symptoms. These symptoms<ref>{{Cite journal|last=Terrahe|first=K.|date=1985-06|title=[The cervico-cranial syndrome in the practice of the otorhinolaryngologist]|url=https://pubmed.ncbi.nlm.nih.gov/4033308/|journal=Laryngologie, Rhinologie, Otologie|volume=64|issue=6|pages=292–299|issn=0340-1588|pmid=4033308}}</ref> often include:


* [[Vertigo]]
'''Cervicocranial syndrome''' (or '''Craniocervical Junction Syndrome''') is a combination of symptoms that are caused by an abnormality in the [[neck]]. Symptoms often include [[vertigo]], chronic [[headache]], [[tinnitus]], facial pain, [[ear pain]], [[dysphagia]] and pain at the [[carotid artery]].<ref name=terrahe1985>{{cite journal |vauthors=Terrahe K |title=[The cervico-cranial syndrome in the practice of the otorhinolaryngologist] |language=German |journal=Laryngol Rhinol Otol (Stuttg) |volume=64 |issue=6 |pages=292–9 |year=1985 |pmid=4033308 |doi= 10.1055/s-2007-1008141|url=}}</ref> It is usually caused by [[spondylosis]], a degeneration of the spinal column.<ref name=terrahe1985/>
* Chronic [[Headache]] aka [[Headache|Cephalea]]
* [[Tinnitus]]<ref>{{Cite journal|last=Terrahe|first=K.|date=1985-06|title=[The cervico-cranial syndrome in the practice of the otorhinolaryngologist]|url=https://pubmed.ncbi.nlm.nih.gov/4033308/|journal=Laryngologie, Rhinologie, Otologie|volume=64|issue=6|pages=292–299|issn=0340-1588|pmid=4033308}}</ref>
* [[Facial pain|Facial Pain]]<ref>{{Cite web|title=Cervicocranial syndrome (Concept Id: C2355645)
- MedGen - NCBI|url=https://www.ncbi.nlm.nih.gov/medgen/?term=cervicocranial%20syndrome#Definition|access-date=2020-11-11|website=www.ncbi.nlm.nih.gov|language=en}}</ref>
* [[ear pain|Ear Pain]]
* [[Dysphagia]]
* [[Carotidynia]]
* [[Neck pain|Neck Pain]] (ex: during movement such extension and flexion)<ref>{{Cite web|title=Cervicocranial syndrome (Concept Id: C2355645)
- MedGen - NCBI|url=https://www.ncbi.nlm.nih.gov/medgen/?term=cervicocranial%20syndrome#Definition|access-date=2020-11-11|website=www.ncbi.nlm.nih.gov|language=en}}</ref>
* [[Syncope (medicine)|Syncope]]<ref>{{Cite journal|last=Cornelius|first=Jan Frederick|last2=Pop|first2=Raoul|last3=Fricia|first3=Marco|last4=George|first4=Bernard|last5=Chibbaro|first5=Salvatore|date=2019|title=Compression Syndromes of the Vertebral Artery at the Craniocervical Junction|url=https://pubmed.ncbi.nlm.nih.gov/30610316/|journal=Acta Neurochirurgica. Supplement|volume=125|pages=151–158|doi=10.1007/978-3-319-62515-7_22|issn=0065-1419|pmid=30610316}}</ref>
*[[Sinusitis|Sinus]] congestion
*Neck [[Crepitus]] Sound
*[[Visual impairment|Loss of vision]]
*[[Nystagmus|Involuntary eye-movement]]
*Severe Fatigue
*[[Chest pain|Chest Pain]]
*[[Clouding of consciousness|Brain Fog]]

== Cause ==
The cause of cervicocranial syndrome is either due to a defect ([[Mutation|genetic mutation]] or development of diseases later in life) or an [[injury]] pertaining to the [[neck]]: cervical area, that damages the [[Spinal nerve|spinal nerves]] traveling through the cervical region<ref>{{Cite web|title=Cervicocranial syndrome (Concept Id: C2355645)
- MedGen - NCBI|url=https://www.ncbi.nlm.nih.gov/medgen/386638|access-date=2020-11-11|website=www.ncbi.nlm.nih.gov|language=en}}</ref> <ref>{{Cite web|title=Cervicocranial syndrome (Concept Id: C2355645)
- MedGen - NCBI|url=https://www.ncbi.nlm.nih.gov/medgen/?term=cervicocranial%20syndrome#Definition|access-date=2020-11-11|website=www.ncbi.nlm.nih.gov|language=en}}</ref> resulting in [[vertebral subluxation]]. <ref>{{Cite journal|last=Vesela|first=Martina|last2=Stetkarova|first2=Ivana|last3=Lisy|first3=Jiri|date=2005-11|title=Prevalence of C1/C2 involvement in Czech rheumatoid arthritis patients, correlation of pain intensity, and distance of ventral subluxation|url=https://pubmed.ncbi.nlm.nih.gov/15666164/|journal=Rheumatology International|volume=26|issue=1|pages=12–15|doi=10.1007/s00296-004-0506-5|issn=0172-8172|pmid=15666164}}</ref> Examples of cases that can result in cervicocranial syndrome are: [[Traffic collision|car accidents]], [[trauma]], [[Osteoarthritis|osteoarthritis,]] [[Tumor microenvironment|tumor,]] [[Degenerative disc disease|degenerative]] [[pathology]]<ref>{{Cite journal|last=Cornelius|first=Jan Frederick|last2=Pop|first2=Raoul|last3=Fricia|first3=Marco|last4=George|first4=Bernard|last5=Chibbaro|first5=Salvatore|date=2019|title=Compression Syndromes of the Vertebral Artery at the Craniocervical Junction|url=https://pubmed.ncbi.nlm.nih.gov/30610316/|journal=Acta Neurochirurgica. Supplement|volume=125|pages=151–158|doi=10.1007/978-3-319-62515-7_22|issn=0065-1419|pmid=30610316}}</ref> and other numerous causes of [[Vertebral column|vertebral]] instability. There is no single cause that can mainly causes cervicocranial syndrome.

=== Genetic ===
[[File:Klippel–Feil syndrome.png|thumb|Individual with [[Klippel–Feil syndrome|Klippel-Feil syndrome]] showing fused cervical bones in the neck. ]]
The [[Gene|genes]] [[GDF6]], [[GDF3]], [[MEOX1]] used as examples, encode for making [[Protein|proteins]] that help with development. For example GDF6 gene plays an important role in [[Bone|bone development]] and [[joint]] formation.<ref>{{Cite journal|date=2013-01-10|title=Mutations in MEOX1, Encoding Mesenchyme Homeobox 1, Cause Klippel-Feil Anomaly|url=https://www.sciencedirect.com/science/article/pii/S0002929712006295|journal=The American Journal of Human Genetics|language=en|volume=92|issue=1|pages=157–161|doi=10.1016/j.ajhg.2012.11.016|issn=0002-9297}}</ref> The [[mutation]] in these genes can result in [[Klippel–Feil syndrome|Klippel-Feil syndrome]]. As a result of having [[Birth defect|congenital]] [[Klippel–Feil syndrome|Klippel-Feil syndrome]], the [[Spine|spinal]] [[anatomy]] of the individual will present abnormal fusion of any two of the seven cervical bones in the neck. <ref>{{Cite journal|last=Kaplan|first=Kevin M.|last2=Spivak|first2=Jeffrey M.|last3=Bendo|first3=John A.|date=2005-09-01|title=Embryology of the spine and associated congenital abnormalities|url=http://www.sciencedirect.com/science/article/pii/S1529943004009155|journal=The Spine Journal|language=en|volume=5|issue=5|pages=564–576|doi=10.1016/j.spinee.2004.10.044|issn=1529-9430}}</ref>This is considered to be an [[anomaly]] of cervical bones.<ref>{{Cite news|title=Congenital Osseous Anomalies of the Upper and Lower... : JBJS|language=en-US|work=LWW|url=https://journals.lww.com/jbjsjournal/Fulltext/2002/02000/Congenital_Osseous_Anomalies_of_the_Upper_and.17.aspx|access-date=2020-12-17}}</ref> It affects the functioning of cervical spinal nerves (C1 - C8) because of compression on the spinal cord. [[Spinal stenosis]] also adds damage the [[spinal cord]] resulting in symptoms that are caused by cervicocranial syndrome.<ref>{{Cite news|title=The Influence of Spinal Canal Narrowing and Timing of... : Spine|language=en-US|work=LWW|url=https://journals.lww.com/spinejournal/Fulltext/1999/08150/The_Influence_of_Spinal_Canal_Narrowing_and_Timing.2.aspx|access-date=2020-12-17}}</ref>

=== Trauma ===
Traumatic injuries are caused when external forces damage the cervical spine, giving rise to various symptoms.<ref name=":1">{{Cite journal|last=Tanaka|first=Nobuhiro|last2=Atesok|first2=Kivanc|last3=Nakanishi|first3=Kazuyoshi|last4=Kamei|first4=Naosuke|last5=Nakamae|first5=Toshio|last6=Kotaka|first6=Shinji|last7=Adachi|first7=Nobuo|date=2018-02-28|title=Pathology and Treatment of Traumatic Cervical Spine Syndrome: Whiplash Injury|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5851023/|journal=Advances in Orthopedics|volume=2018|doi=10.1155/2018/4765050|issn=2090-3464|pmc=5851023|pmid=29682354}}</ref> In a [[motor vehicle]] [[accident]], the vehicle jerks the neck forward and backward resulting in cervical spine damage. This is called [[Whiplash (medicine)|whiplash]]. The neurological and biological symptoms resulting from neck trauma emerge as a culmination of clinically isolated or combined symptoms caused by cervicocranial syndrome.<ref name=":1" />

== Pathophysiology ==
The body is innervated by spinal nerves that branch off from the spinal cord. <ref name=":3">{{Citation|last=Waxenbaum|first=Joshua A.|title=Anatomy, Head and Neck, Cervical Nerves|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK538136/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=30844163|access-date=2020-12-17|last2=Reddy|first2=Vamsi|last3=Bordoni|first3=Bruno}}</ref> This innervation enables the brain to receive sensory inputs and send motor outputs. There are 8 cervical spinal nerves of the peripheral nervous system. Cervical spinal nerves C1, C2 and C3 help control the movements of the head and neck. Cervical spinal nerve C4 helps control upward shoulder movements. Cervical spinal nerve C3, C4 and C5 help power the diaphragm and aid in breathing. Cervical spinal nerve C6 helps in wrist extension and some functioning of biceps. Cervical spinal nerve C7 controls triceps and wrist extension. Cervical spinal nerve C8 helps control the hand.<ref name=":3" /> The cervicocranial syndrome occurs when symptoms arise due to cervical vertebrae damage (misalignment, collapse, shift or disease, such as tumor) resulting in the improper functioning of the cervical spinal nerves.

=== Examples of Cervicocranial Syndrome Pathophysiology ===

==== [[Chordoma]] ====
The craniocervical junction region comprises of C1 (Atlas), C2 (Axis) and the lower part of the [[skull]]: [[occipital bone|occipital bone.]]<ref name=":0" /> In case of [[Neoplasm|tumor]]: [[chordoma]], in the craniocervical junction region, this leads to pressure on the cervical [[Spinal nerve|spinal nerves]], which results in their improper functioning of the cervical spinal nerves. Hence, leading to symptoms of cervicocranial syndrome.<ref>{{Cite journal|last=Kratimenos|first=George P.|last2=Crockard|first2=H. Alan|date=1993-01-01|title=The far lateral approach for ventrally placed foramen magnum and upper cervical spine tumours|url=https://doi.org/10.3109/02688699309103469|journal=British Journal of Neurosurgery|volume=7|issue=2|pages=129–140|doi=10.3109/02688699309103469|issn=0268-8697}}</ref> To decompress the pressure on the nerves, the tumor is removed and the foramen through which the spinal nerve roots travel through is enlarged to allow the nerves to pass through so that symptoms of cervicocranial syndrome can be reduced and the nerves are sending signals.

==== Atlato-Occipital Assimilation ====
When the [[occipital bone]] and the [[Atlas (anatomy)|atlas (C1)]] are fused together in a condition called [https://www.merckmanuals.com/home/brain,-spinal-cord,-and-nerve-disorders/craniocervical-junction-disorders/craniocervical-junction-disorders atlato-occipital assimilation] it causes improper functioning of the cervical spinal nerves due to the vascular compression. Surgical procedure can decompress the nerves and reduce symptoms.<ref>{{Cite journal|last=Menezes|first=Arnold H.|date=1997-09-01|title=Craniovertebral junction anomalies: Diagnosis and management|url=http://www.sciencedirect.com/science/article/pii/S1071909197800381|journal=Seminars in Pediatric Neurology|series=Neurosurgical Issues for the Pediatric Patient|language=en|volume=4|issue=3|pages=209–223|doi=10.1016/S1071-9091(97)80038-1|issn=1071-9091}}</ref>

==== Trauma ====
[[Injury|Traumatic injuries]] are caused when external forces damage the cervical spine, giving rise to various symptoms.<ref name=":1" /> In a car accident, the vehicle jerks the neck forward and backward resulting in cervical spine damage resulting in a [[Whiplash (medicine)|whiplash]]. As a result, the cervical spine become misaligned and produces direct spinal cord irritation creating tighter muscles on one side of the body<ref>{{Cite web|title=Society of Chiropractic Orthospinology - An Upper Cervical Procedure - What Is Orthospinology?|url=https://orthospinology.org/patients/what-is-orthospinology#:~:text=Normal%20health%20is%20the%20natural,this%20type%20of%20cord%20compromise.|access-date=2020-12-17|website=orthospinology.org}}</ref> Neck braces can help temporarily. Surgery is required if needed. Non-surgical treatment, to realign spinal misalignment, is corrected by a chiropractor.

== Diagnosis ==
[[File:Cervical XRayFlexionExtension.jpg|alt=Flexion and Extension|thumb|222x222px|1. Cervical Spine x-ray (lateral view)]]
Once there is an onset of the symptoms in the patient, the patients are screened through cervical-spinal [[Imaging technique|imaging techniques]]: [[X-ray]], [[CT scan|CT]], [[Magnetic resonance imaging|MRI]]. [https://www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/neurological-diagnostic-tests-and-procedures-fact] The scanning technique points out any cervical vertebrae defects and misalignments. (Image 1. and 2.) When cervicocranial syndrome is caused as a result of a [[Genetic disorder|genetic disease]], then [[Genealogy|family history]] and [[genetic testing]] aid in making an accurate diagnosis of cervicocranial syndrome.
[[File:X-ray of the cervical spine of a 20 year old male - odontoid.jpg|thumb|2. Cervical Spine x-ray (Odontoid view)]]

== Prevention/Treatment ==
The treatment options vary since there are numerous causes of cervicocranial syndrome. General treatments include:

* Pressure release via realignment of the vertebrae
* [[Analgesic|Pain medication]]: [[acetominophen]], [[aspirin]], or [[ibuprofen]]
* Manipulation of neck by [[Chiropractic|Chiropractor]] : For example, vertigo symptoms can be relieved<ref>{{Cite journal|last=Mahlstedt|first=K.|last2=Westhofen|first2=M.|last3=König|first3=K.|date=1992-05|title=[Therapy of functional disorders of the craniovertebral joints in vestibular diseases]|url=https://pubmed.ncbi.nlm.nih.gov/1616544/|journal=Laryngo- Rhino- Otologie|volume=71|issue=5|pages=246–250|doi=10.1055/s-2007-997289|issn=0935-8943|pmid=1616544}}</ref>
* [[Cervical collar|Neck braces]] to avoid movement of neck and provide stability
* [[Physical therapy]]
* [[Injection]]: Combination ([[anesthetic]] and [[cortisone]]) drug to help alleviate the pain
*[[Surgery]] to restore function and form of the spine
*Cervical spinal cord stimulation (cSCS) <ref>{{Cite journal|last=Chan|first=Andrew K.|last2=Winkler|first2=Ethan A.|last3=Jacques|first3=Line|date=2016-07-01|title=Rate of perioperative neurological complications after surgery for cervical spinal cord stimulation|url=https://thejns.org/spine/view/journals/j-neurosurg-spine/25/1/article-p31.xml|journal=Journal of Neurosurgery: Spine|language=en-US|volume=25|issue=1|pages=31–38|doi=10.3171/2015.10.SPINE15670}}</ref>
When cervicocranical syndrome is caused by a [[mutation]] in [[Gene|genes]] and it runs in the family due to other co-morbidities, [[genetic counseling]] helps patients cover risks, prevention and expectations of caring and passing genes to a newborn.<ref>{{Cite journal|last=Rossi|first=Ernest L.|last2=Cozzolino|first2=Mauro|last3=Mortimer|first3=Jane|last4=Atkinson|first4=David|last5=Rossi|first5=Kathryn Lane|date=2011-10|title=A Brief Protocol for the Creative Psychosocial Genomic Healing Experience: The 4-Stage Creative Process in Therapeutic Hypnosis and Brief Psychotherapy|url=http://dx.doi.org/10.1080/00029157.2011.605967|journal=American Journal of Clinical Hypnosis|volume=54|issue=2|pages=133–152|doi=10.1080/00029157.2011.605967|issn=0002-9157}}</ref>

== Prognosis ==
The prognosis of an individual living with cervicocranial syndrome varies because of the multiple causes such as co-morbidities and varied trauma. Instability of the cervical spine can cause endangerment of patients and their neurological integrity.<ref>{{Cite journal|last=Wenning|first=Katharina E.|last2=Hoffmann|first2=Martin F.|date=2020-01-09|title=Does isolated atlantoaxial fusion result in better clinical outcome compared to occipitocervical fusion?|url=https://doi.org/10.1186/s13018-019-1525-y|journal=Journal of Orthopaedic Surgery and Research|volume=15|issue=1|pages=8|doi=10.1186/s13018-019-1525-y|issn=1749-799X}}</ref> Correction and decompression cervical spinal surgeries significantly increase quality of life and reduce symptoms. Post-surgery, 93 to 100 percent patients post-surgery report reduced cervicocranical syndrome symptoms such as neck pain.<ref>{{Cite web|date=2017-09-15|title=ACDF Surgery: Success Rate and What to Expect After Surgery|url=https://www.healthline.com/health/acdf-surgery|access-date=2020-12-17|website=Healthline|language=en}}</ref><ref>{{Cite journal|last=Yin|first=Yi-Heng|last2=Qiao|first2=Guang-Yu|last3=Yu|first3=Xin-Guang|date=2016-11-01|title=Surgical Treatment of Occipitocervical Dislocation with Atlas Assimilation and Klippel-Feil Syndrome Using Occipitalized C1 Lateral Mass and C2 Fixation and Reduction Technique|url=http://www.sciencedirect.com/science/article/pii/S1878875016305861|journal=World Neurosurgery|language=en|volume=95|pages=46–52|doi=10.1016/j.wneu.2016.07.058|issn=1878-8750}}</ref><ref name=":2">{{Cite journal|last=Waheed|first=Muhammad Abdul-Aziz|last2=Hasan|first2=Sazid|last3=Tan|first3=Lee A.|last4=Bosco|first4=Aju|last5=Reinas|first5=Rui|last6=Wengel|first6=Paula Valerie ter|last7=Hey|first7=Hwee Weng Dennis|last8=Aleem|first8=Ilyas S.|date=2020/03|title=Cervical spine pathology and treatment: a global overview|url=http://jss.amegroups.com/article/view/4882|journal=Journal of Spine Surgery|language=en|volume=6|issue=1|pages=340–350|doi=10.21037/jss.2020.01.12|issn=2414-4630}}</ref>

== Epidemiology ==
Cervicocranial syndrome significantly affects the aging world population and is associated with significant morbidity.<ref name=":2" /> It affects men and women equally when occurring due to atlanto-occipital assimilation. <ref>{{Cite web|last=Knipe|first=Henry|title=Atlanto-occipital assimilation {{!}} Radiology Reference Article {{!}} Radiopaedia.org|url=https://radiopaedia.org/articles/atlanto-occipital-assimilation?lang=us|access-date=2020-12-17|website=Radiopaedia|language=en-US}}</ref> 12% - 50% cervical spine injuries occur in a setting of a cervical spine fracture<ref>{{Cite journal|last=Yadollahi|first=Mahnaz|last2=Paydar|first2=Shahram|last3=Ghaem|first3=Haleh|last4=Ghorbani|first4=Mohammad|last5=Mousavi|first5=Seyed Mohsen|last6=Taheri Akerdi|first6=Ali|last7=Jalili|first7=Eimen|last8=Niakan|first8=Mohammad Hadi|last9=Khalili|first9=Hossein Ali|last10=Haghnegahdar|first10=Ali|last11=Bolandparvaz|first11=Shahram|date=2016-03-16|title=Epidemiology of Cervical Spine Fractures|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5124335/|journal=Trauma Monthly|volume=21|issue=3|doi=10.5812/traumamon.33608|issn=2251-7464|pmc=5124335|pmid=27921020}}</ref> that result in cervicocranial syndrome symptoms. Increased incidences among low-socioeconomic groups and among groups that so not access to healthcare show subsequently higher rates of morbidity and mortality.<ref name=":2" />

== Research Directions ==
Cervicocranial syndrome can be caused with or as a result of numerous neurological problems so not one single disease can be pinpointed. Further research can explore the common neurological problems causing cervicocranial syndrome and look at various treatments including therapeutic ones.

For example a study, ''"The influence of cranio-cervical rehabilitation in patients with myofascial temporomandibular pain disorders,''" <ref>{{Cite journal|last=Halmova|first=K.|last2=Holly|first2=D.|last3=Stanko|first3=P.|date=2017|title=The influence of cranio-cervical rehabilitation in patients with myofascial temporomandibular pain disorders|url=https://pubmed.ncbi.nlm.nih.gov/29216730/|journal=Bratislavske Lekarske Listy|volume=118|issue=11|pages=710–713|doi=10.4149/BLL_2017_134|issn=0006-9248|pmid=29216730}}</ref> explored the therapeutic options of physical therapy and concluded that 88% from a total of 98 patients (79 female and 19 male), felt reduced pain. On the contrary another study, ''"The efficacy of manual therapy and therapeutic exercise in patients with chronic neck pain: A narrative review"'' <ref>https://www.kheljournal.com/archives/2018/vol5issue1/PartA/5-1-6-780.pdf</ref> conducted in 2018, concluded that there is a lack of evidence that support therapeutic exercise to reduce neck pain via manipulation.

A study, "''Epidemiology of Cervical Spine Fractures,"'' <ref>{{Cite journal|last=Yadollahi|first=Mahnaz|last2=Paydar|first2=Shahram|last3=Ghaem|first3=Haleh|last4=Ghorbani|first4=Mohammad|last5=Mousavi|first5=Seyed Mohsen|last6=Taheri Akerdi|first6=Ali|last7=Jalili|first7=Eimen|last8=Niakan|first8=Mohammad Hadi|last9=Khalili|first9=Hossein Ali|last10=Haghnegahdar|first10=Ali|last11=Bolandparvaz|first11=Shahram|date=2016-03-16|title=Epidemiology of Cervical Spine Fractures|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5124335/|journal=Trauma Monthly|volume=21|issue=3|doi=10.5812/traumamon.33608|issn=2251-7464|pmc=5124335|pmid=27921020}}</ref>evaluated the epidemiology of CSfx in 469 trauma patients. The data was collected over a period of 3.5 years. The age range was 16 - 60yrs and maximum age was 86yrs. The results showed that young adults were more frequently affected. The males were likely to be affected as result of vehicle accidents and the mortality rate was 6.18%. Further research needs to access risk factors and techniques to reduce mortality among young patients.

Future research direction, understanding cervicocranial syndrome in young patients and in older population - are they different or the same? Understanding which cervical spine morbidities do not cause cervicocranial syndrome.


==References==
==References==
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Revision as of 05:16, 17 December 2020

Cervicocranial syndrome
Other namesCraniocervical Junction Syndrome
SpecialtyNeurology

Cervicocranial syndrome or (Craniocervical Junction Syndrome: CCJ syndrome) is a neurological illness. It is a combination of symptoms that are caused by an abnormality in the neck. The bones of the neck that are affected are cervical vertebrae (C1 - C7). This syndrome can be identified by confirming cervical bone shifts, collapsed cervical bones or misalignment of the cervical bone leading to improper functioning of cervical spinal nerves.[1] Cervicocranial syndrome is either congenital or acquired (as a result of injury or disease). Some examples of diseases that could result in cervicocranial syndrome are Chiari disease, Klippel-Feil malformation [1] osteoarthritis, and trauma.[2][3] Treatment options include neck braces, pain medication and surgery. The quality of life for individuals suffering from CCJ syndrome can improve through surgery.[4]

Cervical Vertebrae (C1 - C7)

Signs and Symptoms

Cervicocranial syndrome has a wide range of symptoms. These symptoms[5] often include:

Cause

The cause of cervicocranial syndrome is either due to a defect (genetic mutation or development of diseases later in life) or an injury pertaining to the neck: cervical area, that damages the spinal nerves traveling through the cervical region[10] [11] resulting in vertebral subluxation. [12] Examples of cases that can result in cervicocranial syndrome are: car accidents, trauma, osteoarthritis, tumor, degenerative pathology[13] and other numerous causes of vertebral instability. There is no single cause that can mainly causes cervicocranial syndrome.

Genetic

Individual with Klippel-Feil syndrome showing fused cervical bones in the neck.

The genes GDF6, GDF3, MEOX1 used as examples, encode for making proteins that help with development. For example GDF6 gene plays an important role in bone development and joint formation.[14] The mutation in these genes can result in Klippel-Feil syndrome. As a result of having congenital Klippel-Feil syndrome, the spinal anatomy of the individual will present abnormal fusion of any two of the seven cervical bones in the neck. [15]This is considered to be an anomaly of cervical bones.[16] It affects the functioning of cervical spinal nerves (C1 - C8) because of compression on the spinal cord. Spinal stenosis also adds damage the spinal cord resulting in symptoms that are caused by cervicocranial syndrome.[17]

Trauma

Traumatic injuries are caused when external forces damage the cervical spine, giving rise to various symptoms.[18] In a motor vehicle accident, the vehicle jerks the neck forward and backward resulting in cervical spine damage. This is called whiplash. The neurological and biological symptoms resulting from neck trauma emerge as a culmination of clinically isolated or combined symptoms caused by cervicocranial syndrome.[18]

Pathophysiology

The body is innervated by spinal nerves that branch off from the spinal cord. [19] This innervation enables the brain to receive sensory inputs and send motor outputs. There are 8 cervical spinal nerves of the peripheral nervous system. Cervical spinal nerves C1, C2 and C3 help control the movements of the head and neck. Cervical spinal nerve C4 helps control upward shoulder movements. Cervical spinal nerve C3, C4 and C5 help power the diaphragm and aid in breathing. Cervical spinal nerve C6 helps in wrist extension and some functioning of biceps. Cervical spinal nerve C7 controls triceps and wrist extension. Cervical spinal nerve C8 helps control the hand.[19] The cervicocranial syndrome occurs when symptoms arise due to cervical vertebrae damage (misalignment, collapse, shift or disease, such as tumor) resulting in the improper functioning of the cervical spinal nerves.

Examples of Cervicocranial Syndrome Pathophysiology

Chordoma

The craniocervical junction region comprises of C1 (Atlas), C2 (Axis) and the lower part of the skull: occipital bone.[2] In case of tumor: chordoma, in the craniocervical junction region, this leads to pressure on the cervical spinal nerves, which results in their improper functioning of the cervical spinal nerves. Hence, leading to symptoms of cervicocranial syndrome.[20] To decompress the pressure on the nerves, the tumor is removed and the foramen through which the spinal nerve roots travel through is enlarged to allow the nerves to pass through so that symptoms of cervicocranial syndrome can be reduced and the nerves are sending signals.

Atlato-Occipital Assimilation

When the occipital bone and the atlas (C1) are fused together in a condition called atlato-occipital assimilation it causes improper functioning of the cervical spinal nerves due to the vascular compression. Surgical procedure can decompress the nerves and reduce symptoms.[21]

Trauma

Traumatic injuries are caused when external forces damage the cervical spine, giving rise to various symptoms.[18] In a car accident, the vehicle jerks the neck forward and backward resulting in cervical spine damage resulting in a whiplash. As a result, the cervical spine become misaligned and produces direct spinal cord irritation creating tighter muscles on one side of the body[22] Neck braces can help temporarily. Surgery is required if needed. Non-surgical treatment, to realign spinal misalignment, is corrected by a chiropractor.

Diagnosis

Flexion and Extension
1. Cervical Spine x-ray (lateral view)

Once there is an onset of the symptoms in the patient, the patients are screened through cervical-spinal imaging techniques: X-ray, CT, MRI. [2] The scanning technique points out any cervical vertebrae defects and misalignments. (Image 1. and 2.) When cervicocranial syndrome is caused as a result of a genetic disease, then family history and genetic testing aid in making an accurate diagnosis of cervicocranial syndrome.

2. Cervical Spine x-ray (Odontoid view)

Prevention/Treatment

The treatment options vary since there are numerous causes of cervicocranial syndrome. General treatments include:

When cervicocranical syndrome is caused by a mutation in genes and it runs in the family due to other co-morbidities, genetic counseling helps patients cover risks, prevention and expectations of caring and passing genes to a newborn.[25]

Prognosis

The prognosis of an individual living with cervicocranial syndrome varies because of the multiple causes such as co-morbidities and varied trauma. Instability of the cervical spine can cause endangerment of patients and their neurological integrity.[26] Correction and decompression cervical spinal surgeries significantly increase quality of life and reduce symptoms. Post-surgery, 93 to 100 percent patients post-surgery report reduced cervicocranical syndrome symptoms such as neck pain.[27][28][29]

Epidemiology

Cervicocranial syndrome significantly affects the aging world population and is associated with significant morbidity.[29] It affects men and women equally when occurring due to atlanto-occipital assimilation. [30] 12% - 50% cervical spine injuries occur in a setting of a cervical spine fracture[31] that result in cervicocranial syndrome symptoms. Increased incidences among low-socioeconomic groups and among groups that so not access to healthcare show subsequently higher rates of morbidity and mortality.[29]

Research Directions

Cervicocranial syndrome can be caused with or as a result of numerous neurological problems so not one single disease can be pinpointed. Further research can explore the common neurological problems causing cervicocranial syndrome and look at various treatments including therapeutic ones.

For example a study, "The influence of cranio-cervical rehabilitation in patients with myofascial temporomandibular pain disorders," [32] explored the therapeutic options of physical therapy and concluded that 88% from a total of 98 patients (79 female and 19 male), felt reduced pain. On the contrary another study, "The efficacy of manual therapy and therapeutic exercise in patients with chronic neck pain: A narrative review" [33] conducted in 2018, concluded that there is a lack of evidence that support therapeutic exercise to reduce neck pain via manipulation.

A study, "Epidemiology of Cervical Spine Fractures," [34]evaluated the epidemiology of CSfx in 469 trauma patients. The data was collected over a period of 3.5 years. The age range was 16 - 60yrs and maximum age was 86yrs. The results showed that young adults were more frequently affected. The males were likely to be affected as result of vehicle accidents and the mortality rate was 6.18%. Further research needs to access risk factors and techniques to reduce mortality among young patients.

Future research direction, understanding cervicocranial syndrome in young patients and in older population - are they different or the same? Understanding which cervical spine morbidities do not cause cervicocranial syndrome.

References

  1. ^ Avellaneda Fernández, Alfredo; Isla Guerrero, Alberto; Izquierdo Martínez, Maravillas; Amado Vázquez, María Eugenia; Barrón Fernández, Javier; Chesa i Octavio, Ester; De la Cruz Labrado, Javier; Escribano Silva, Mercedes; Fernández de Gamboa Fernández de Araoz, Marta; García-Ramos, Rocío; García Ribes, Miguel (2009-12-17). "Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment)". BMC Musculoskeletal Disorders. 10 (1): S1. doi:10.1186/1471-2474-10-S1-S1. ISSN 1471-2474. PMC 2796052. PMID 20018097.{{cite journal}}: CS1 maint: PMC format (link) CS1 maint: unflagged free DOI (link)
  2. ^ a b Rosa, Scott; Baird, John W.; Harshfield, David; Chehrenama, Mahan (2018-08-01). "Craniocervical Junction Syndrome: Anatomy of the Craniocervical and Atlantoaxial Junctions and the Effect of Misalignment on Cerebrospinal Fluid Flow". Hydrocephalus - Water on the Brain. doi:10.5772/intechopen.72890.
  3. ^ Smoker, Wendy R. K.; Khanna, Geetika (2008-10). "Imaging the craniocervical junction". Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery. 24 (10): 1123–1145. doi:10.1007/s00381-008-0601-0. ISSN 0256-7040. PMID 18461336. {{cite journal}}: Check date values in: |date= (help)
  4. ^ Henderson, Fraser C.; Francomano, C. A.; Koby, M.; Tuchman, K.; Adcock, J.; Patel, S. (2019). "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". Neurosurgical Review. 42 (4): 915–936. doi:10.1007/s10143-018-01070-4. ISSN 0344-5607. PMC 6821667. PMID 30627832.
  5. ^ Terrahe, K. (1985-06). "[The cervico-cranial syndrome in the practice of the otorhinolaryngologist]". Laryngologie, Rhinologie, Otologie. 64 (6): 292–299. ISSN 0340-1588. PMID 4033308. {{cite journal}}: Check date values in: |date= (help)
  6. ^ Terrahe, K. (1985-06). "[The cervico-cranial syndrome in the practice of the otorhinolaryngologist]". Laryngologie, Rhinologie, Otologie. 64 (6): 292–299. ISSN 0340-1588. PMID 4033308. {{cite journal}}: Check date values in: |date= (help)
  7. ^ "Cervicocranial syndrome (Concept Id: C2355645) - MedGen - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2020-11-11. {{cite web}}: line feed character in |title= at position 47 (help)
  8. ^ "Cervicocranial syndrome (Concept Id: C2355645) - MedGen - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2020-11-11. {{cite web}}: line feed character in |title= at position 47 (help)
  9. ^ Cornelius, Jan Frederick; Pop, Raoul; Fricia, Marco; George, Bernard; Chibbaro, Salvatore (2019). "Compression Syndromes of the Vertebral Artery at the Craniocervical Junction". Acta Neurochirurgica. Supplement. 125: 151–158. doi:10.1007/978-3-319-62515-7_22. ISSN 0065-1419. PMID 30610316.
  10. ^ "Cervicocranial syndrome (Concept Id: C2355645) - MedGen - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2020-11-11. {{cite web}}: line feed character in |title= at position 47 (help)
  11. ^ "Cervicocranial syndrome (Concept Id: C2355645) - MedGen - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2020-11-11. {{cite web}}: line feed character in |title= at position 47 (help)
  12. ^ Vesela, Martina; Stetkarova, Ivana; Lisy, Jiri (2005-11). "Prevalence of C1/C2 involvement in Czech rheumatoid arthritis patients, correlation of pain intensity, and distance of ventral subluxation". Rheumatology International. 26 (1): 12–15. doi:10.1007/s00296-004-0506-5. ISSN 0172-8172. PMID 15666164. {{cite journal}}: Check date values in: |date= (help)
  13. ^ Cornelius, Jan Frederick; Pop, Raoul; Fricia, Marco; George, Bernard; Chibbaro, Salvatore (2019). "Compression Syndromes of the Vertebral Artery at the Craniocervical Junction". Acta Neurochirurgica. Supplement. 125: 151–158. doi:10.1007/978-3-319-62515-7_22. ISSN 0065-1419. PMID 30610316.
  14. ^ "Mutations in MEOX1, Encoding Mesenchyme Homeobox 1, Cause Klippel-Feil Anomaly". The American Journal of Human Genetics. 92 (1): 157–161. 2013-01-10. doi:10.1016/j.ajhg.2012.11.016. ISSN 0002-9297.
  15. ^ Kaplan, Kevin M.; Spivak, Jeffrey M.; Bendo, John A. (2005-09-01). "Embryology of the spine and associated congenital abnormalities". The Spine Journal. 5 (5): 564–576. doi:10.1016/j.spinee.2004.10.044. ISSN 1529-9430.
  16. ^ "Congenital Osseous Anomalies of the Upper and Lower... : JBJS". LWW. Retrieved 2020-12-17.
  17. ^ "The Influence of Spinal Canal Narrowing and Timing of... : Spine". LWW. Retrieved 2020-12-17.
  18. ^ a b c Tanaka, Nobuhiro; Atesok, Kivanc; Nakanishi, Kazuyoshi; Kamei, Naosuke; Nakamae, Toshio; Kotaka, Shinji; Adachi, Nobuo (2018-02-28). "Pathology and Treatment of Traumatic Cervical Spine Syndrome: Whiplash Injury". Advances in Orthopedics. 2018. doi:10.1155/2018/4765050. ISSN 2090-3464. PMC 5851023. PMID 29682354.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  19. ^ a b Waxenbaum, Joshua A.; Reddy, Vamsi; Bordoni, Bruno (2020), "Anatomy, Head and Neck, Cervical Nerves", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 30844163, retrieved 2020-12-17
  20. ^ Kratimenos, George P.; Crockard, H. Alan (1993-01-01). "The far lateral approach for ventrally placed foramen magnum and upper cervical spine tumours". British Journal of Neurosurgery. 7 (2): 129–140. doi:10.3109/02688699309103469. ISSN 0268-8697.
  21. ^ Menezes, Arnold H. (1997-09-01). "Craniovertebral junction anomalies: Diagnosis and management". Seminars in Pediatric Neurology. Neurosurgical Issues for the Pediatric Patient. 4 (3): 209–223. doi:10.1016/S1071-9091(97)80038-1. ISSN 1071-9091.
  22. ^ "Society of Chiropractic Orthospinology - An Upper Cervical Procedure - What Is Orthospinology?". orthospinology.org. Retrieved 2020-12-17.
  23. ^ Mahlstedt, K.; Westhofen, M.; König, K. (1992-05). "[Therapy of functional disorders of the craniovertebral joints in vestibular diseases]". Laryngo- Rhino- Otologie. 71 (5): 246–250. doi:10.1055/s-2007-997289. ISSN 0935-8943. PMID 1616544. {{cite journal}}: Check date values in: |date= (help)
  24. ^ Chan, Andrew K.; Winkler, Ethan A.; Jacques, Line (2016-07-01). "Rate of perioperative neurological complications after surgery for cervical spinal cord stimulation". Journal of Neurosurgery: Spine. 25 (1): 31–38. doi:10.3171/2015.10.SPINE15670.
  25. ^ Rossi, Ernest L.; Cozzolino, Mauro; Mortimer, Jane; Atkinson, David; Rossi, Kathryn Lane (2011-10). "A Brief Protocol for the Creative Psychosocial Genomic Healing Experience: The 4-Stage Creative Process in Therapeutic Hypnosis and Brief Psychotherapy". American Journal of Clinical Hypnosis. 54 (2): 133–152. doi:10.1080/00029157.2011.605967. ISSN 0002-9157. {{cite journal}}: Check date values in: |date= (help)
  26. ^ Wenning, Katharina E.; Hoffmann, Martin F. (2020-01-09). "Does isolated atlantoaxial fusion result in better clinical outcome compared to occipitocervical fusion?". Journal of Orthopaedic Surgery and Research. 15 (1): 8. doi:10.1186/s13018-019-1525-y. ISSN 1749-799X.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  27. ^ "ACDF Surgery: Success Rate and What to Expect After Surgery". Healthline. 2017-09-15. Retrieved 2020-12-17.
  28. ^ Yin, Yi-Heng; Qiao, Guang-Yu; Yu, Xin-Guang (2016-11-01). "Surgical Treatment of Occipitocervical Dislocation with Atlas Assimilation and Klippel-Feil Syndrome Using Occipitalized C1 Lateral Mass and C2 Fixation and Reduction Technique". World Neurosurgery. 95: 46–52. doi:10.1016/j.wneu.2016.07.058. ISSN 1878-8750.
  29. ^ a b c Waheed, Muhammad Abdul-Aziz; Hasan, Sazid; Tan, Lee A.; Bosco, Aju; Reinas, Rui; Wengel, Paula Valerie ter; Hey, Hwee Weng Dennis; Aleem, Ilyas S. (2020/03). "Cervical spine pathology and treatment: a global overview". Journal of Spine Surgery. 6 (1): 340–350. doi:10.21037/jss.2020.01.12. ISSN 2414-4630. {{cite journal}}: Check date values in: |date= (help)CS1 maint: unflagged free DOI (link)
  30. ^ Knipe, Henry. "Atlanto-occipital assimilation | Radiology Reference Article | Radiopaedia.org". Radiopaedia. Retrieved 2020-12-17.
  31. ^ Yadollahi, Mahnaz; Paydar, Shahram; Ghaem, Haleh; Ghorbani, Mohammad; Mousavi, Seyed Mohsen; Taheri Akerdi, Ali; Jalili, Eimen; Niakan, Mohammad Hadi; Khalili, Hossein Ali; Haghnegahdar, Ali; Bolandparvaz, Shahram (2016-03-16). "Epidemiology of Cervical Spine Fractures". Trauma Monthly. 21 (3). doi:10.5812/traumamon.33608. ISSN 2251-7464. PMC 5124335. PMID 27921020.
  32. ^ Halmova, K.; Holly, D.; Stanko, P. (2017). "The influence of cranio-cervical rehabilitation in patients with myofascial temporomandibular pain disorders". Bratislavske Lekarske Listy. 118 (11): 710–713. doi:10.4149/BLL_2017_134. ISSN 0006-9248. PMID 29216730.
  33. ^ https://www.kheljournal.com/archives/2018/vol5issue1/PartA/5-1-6-780.pdf
  34. ^ Yadollahi, Mahnaz; Paydar, Shahram; Ghaem, Haleh; Ghorbani, Mohammad; Mousavi, Seyed Mohsen; Taheri Akerdi, Ali; Jalili, Eimen; Niakan, Mohammad Hadi; Khalili, Hossein Ali; Haghnegahdar, Ali; Bolandparvaz, Shahram (2016-03-16). "Epidemiology of Cervical Spine Fractures". Trauma Monthly. 21 (3). doi:10.5812/traumamon.33608. ISSN 2251-7464. PMC 5124335. PMID 27921020.

External links

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