Cervicocranial syndrome

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Cervicocranial syndrome
Other namesCraniocervical junction syndrome
Specialty Neurology

Cervicocranial syndrome or (craniocervical junction syndrome, CCJ syndrome) is a combination of symptoms that are caused by an abnormality in the cervical vertebrae leading to improper function of cervical spinal nerves. Cervicocranial syndrome is either congenital [1] or acquired. [2] Cervicocranial syndrome may be caused by Chiari disease, Klippel-Feil malformation, [3] osteoarthritis, and physical trauma. [4] Treatment options include neck braces, pain medication and surgery. The quality of life for individuals suffering from Cervicocranial syndrome can improve through surgery. [5]

Contents

Cervical Vertebrae (C1 - C7) Cervical spine Anterior.png
Cervical Vertebrae (C1 - C7)

Signs and symptoms

Cervicocranial syndrome has a wide range of symptoms. These symptoms [6] often include:

Cause

Cervicocranial syndrome can be caused either due to a defect (genetic mutation [9] or development of diseases later in life) or an injury pertaining to the cervical area of the neck that damages the spinal nerves traveling through the cervical region [10] [7] resulting in ventral subluxation. [11] Examples of cases that can result in cervicocranial syndrome are: car accidents, trauma, osteoarthritis, tumor, degenerative pathology [8] and other numerous causes of vertebral instability. There is no single cause that can mainly cause cervicocranial syndrome.

Genetic

Individual with Klippel-Feil syndrome showing fused cervical bones in the neck. Klippel-Feil syndrome.png
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. [12] 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. [13] This is considered to be an anomaly of cervical bones. [14] It affects the functioning of cervical spinal nerves (C1 - C8) because of compression on the spinal cord. Spinal stenosis also adds damage to the spinal cord resulting in symptoms that are caused by cervicocranial syndrome. [15]

Trauma

Traumatic injuries are caused when external forces damage the cervical spine, giving rise to various symptoms. [16] In a motor vehicle accident, the vehicle jerks the neck forward and backward resulting in cervical spine damage. This is called whiplash. [17] The neurological and biological symptoms resulting from neck trauma emerge as a culmination of clinically isolated or combined symptoms caused by cervicocranial syndrome. [16]

Pathophysiology

The body is innervated by spinal nerves that branch off from the spinal cord. [18] 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. [18] 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 C1 (atlas), C2 (axis) and the lower part of the skull: occipital bone. [19] 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.

Atlanto-occipital assimilation

When the occipital bone and the atlas (C1) are fused together in a condition called atlanto-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] [22] [23]

Trauma

Traumatic injuries are caused when external forces damage the cervical spine, giving rise to various symptoms. [16] 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 [24] Neck braces can help temporarily. Surgery is required if needed. Non-surgical treatment, to realign spinal misalignment, is corrected by a chiropractor.

Diagnosis

1. Cervical spine x-ray (lateral view) Cervical XRayFlexionExtension.jpg
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. 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) X-ray of the cervical spine of a 20 year old male - odontoid.jpg
2. Cervical spine x-ray (odontoid view)

Prevention and treatment

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

When cervicocranial 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. [27]

Prognosis

The prognosis of individuals living with cervicocranial syndrome varies due to multiple causes such as co-morbidities and varied trauma. Instability of the cervical spine can cause endangerment of patients and their neurological integrity. [28] Correction and decompression cervical spinal surgeries significantly increase quality of life and reduce symptoms. Post-surgery, 93 to 100 percent of patients report reduced cervicocranial syndrome symptoms such as neck pain. [29] [30]

Epidemiology

Cervicocranial syndrome significantly affects the aging world population and is associated with significant morbidity. [30] It affects men and women equally when occurring due to atlanto-occipital assimilation. [31] Increased incidence among low-socioeconomic groups and among groups that do not have access to healthcare show subsequently higher rates of morbidity and mortality. [30]

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.

Related Research Articles

<span class="mw-page-title-main">Tetraplegia</span> Paralysis of all four limbs and torso

Tetraplegia, also known as quadriplegia, is defined as the dysfunction or loss of motor and/or sensory function in the cervical area of the spinal cord. A loss of motor function can present as either weakness or paralysis leading to partial or total loss of function in the arms, legs, trunk, and pelvis. The paralysis may be flaccid or spastic. A loss of sensory function can present as an impairment or complete inability to sense light touch, pressure, heat, pinprick/pain, and proprioception. In these types of spinal cord injury, it is common to have a loss of both sensation and motor control.

<span class="mw-page-title-main">Cervical spine disorder</span> Medical condition

Cervical spine disorders are illnesses that affect the cervical spine, which is made up of the upper first seven vertebrae, encasing and shielding the spinal cord. This fragment of the spine starts from the region above the shoulder blades and ends by supporting and connecting the skull.

<span class="mw-page-title-main">Spondylosis</span> Degeneration of the vertebral column

Spondylosis is the degeneration of the vertebral column from any cause. In the more narrow sense, it refers to spinal osteoarthritis, the age-related degeneration of the spinal column, which is the most common cause of spondylosis. The degenerative process in osteoarthritis chiefly affects the vertebral bodies, the neural foramina and the facet joints. If severe, it may cause pressure on the spinal cord or nerve roots with subsequent sensory or motor disturbances, such as pain, paresthesia, imbalance, and muscle weakness in the limbs.

<span class="mw-page-title-main">Spinal cord injury</span> Injury to the main nerve bundle in the back of humans

A spinal cord injury (SCI) is damage to the spinal cord that causes temporary or permanent changes in its function. It is a destructive neurological and pathological state that causes major motor, sensory and autonomic dysfunctions.

<span class="mw-page-title-main">Cervical vertebrae</span> Vertebrae of the neck

In tetrapods, cervical vertebrae are the vertebrae of the neck, immediately below the skull. Truncal vertebrae lie caudal of cervical vertebrae. In sauropsid species, the cervical vertebrae bear cervical ribs. In lizards and saurischian dinosaurs, the cervical ribs are large; in birds, they are small and completely fused to the vertebrae. The vertebral transverse processes of mammals are homologous to the cervical ribs of other amniotes. Most mammals have seven cervical vertebrae, with the only three known exceptions being the manatee with six, the two-toed sloth with five or six, and the three-toed sloth with nine.

<span class="mw-page-title-main">Degenerative disc disease</span> Loss of function in the spines intervertebral discs

Degenerative disc disease (DDD) is a medical condition typically brought on by the aging process in which there are anatomic changes and possibly a loss of function of one or more intervertebral discs of the spine. DDD can take place with or without symptoms, but is typically identified once symptoms arise. The root cause is thought to be loss of soluble proteins within the fluid contained in the disc with resultant reduction of the oncotic pressure, which in turn causes loss of fluid volume. Normal downward forces cause the affected disc to lose height, and the distance between vertebrae is reduced. The anulus fibrosus, the tough outer layers of a disc, also weakens. This loss of height causes laxity of the longitudinal ligaments, which may allow anterior, posterior, or lateral shifting of the vertebral bodies, causing facet joint malalignment and arthritis; scoliosis; cervical hyperlordosis; thoracic hyperkyphosis; lumbar hyperlordosis; narrowing of the space available for the spinal tract within the vertebra ; or narrowing of the space through which a spinal nerve exits with resultant inflammation and impingement of a spinal nerve, causing a radiculopathy.

In medicine, a stinger, also called a burner or nerve pinch injury, is a neurological injury suffered by athletes, mostly in high-contact sports such as ice hockey, rugby, American football, and wrestling. The spine injury is characterized by a shooting or stinging pain that travels down one arm, followed by numbness and weakness in the parts of the arms, including the biceps, deltoid, and spinati muscles. Many athletes in contact sports have suffered stingers, but they are often unreported to medical professionals.

<span class="mw-page-title-main">Occipital neuralgia</span> Medical condition

Occipital neuralgia (ON) is a painful condition affecting the posterior head in the distributions of the greater occipital nerve (GON), lesser occipital nerve (LON), third occipital nerve (TON), or a combination of the three. It is paroxysmal, lasting from seconds to minutes, and often consists of lancinating pain that directly results from the pathology of one of these nerves. It is paramount that physicians understand the differential diagnosis for this condition and specific diagnostic criteria. There are multiple treatment modalities, several of which have well-established efficacy in treating this condition.

<span class="mw-page-title-main">Klippel–Feil syndrome</span> Congenital condition characterised by fusion of two or more vertebrae in the neck

Klippel–Feil syndrome (KFS), also known as cervical vertebral fusion syndrome, is a rare congenital condition characterized by the abnormal fusion of any two of the seven bones in the neck. It can result in a limited ability to move the neck and shortness of the neck, resulting in the appearance of a low hairline. Most people only have one or two of those symptoms so it may not be noticeable without medical imaging.

<span class="mw-page-title-main">Rectus capitis posterior minor muscle</span> Tendon

The rectus capitis posterior minor is a muscle in the upper back part of the neck. It is one of the suboccipital muscles. Its inferior attachment is at the posterior arch of atlas; its superior attachment is onto the occipital bone at and below the inferior nuchal line. The muscle is innervated by the suboccipital nerve. The muscle acts as a weak extensor of the head.

<span class="mw-page-title-main">Occipital condyles</span> Undersurface protuberances of the occipital bone in vertebrates

The occipital condyles are undersurface protuberances of the occipital bone in vertebrates, which function in articulation with the superior facets of the atlas vertebra.

A syrinx is a rare, fluid-filled neuroglial cavity within the spinal cord (syringomyelia), in the brain stem (syringobulbia), or in the nerves of the elbow, usually in a young age.

<span class="mw-page-title-main">Disc herniation</span> Injury to the intervertebral disc

A spinal disc herniation or simply a disc herniation is an injury to the intervertebral disc between two spinal vertebrae, usually caused by excessive strain or trauma to the spine. It may result in back pain, pain or sensation in different parts of the body, and physical disability. The most conclusive diagnostic tool for disc herniation is MRI, and treatments may range from painkillers to surgery. Protection from disc herniation is best provided by core strength and an awareness of body mechanics including good posture.

Tethered cord syndrome (TCS) refers to a group of neurological disorders that relate to malformations of the spinal cord. Various forms include tight filum terminale, lipomeningomyelocele, split cord malformations (diastematomyelia), occult, dermal sinus tracts, and dermoids. All forms involve the pulling of the spinal cord at the base of the spinal canal, literally a tethered cord. The spinal cord normally hangs loose in the canal, free to move up and down with growth, and with bending and stretching. A tethered cord, however, is held taut at the end or at some point in the spinal canal. In children, a tethered cord can force the spinal cord to stretch as they grow. In adults the spinal cord stretches in the course of normal activity, usually leading to progressive spinal cord damage if untreated. TCS is often associated with the closure of a spina bifida. It can be congenital, such as in tight filum terminale, or the result of injury later in life.

Atlanto-occipital dislocation, orthopedic decapitation, or internal decapitation describes ligamentous separation of the spinal column from the skull base. It is possible for a human to survive such an injury; however, 70% of cases result in immediate death. It should not be confused with atlanto-axial dislocation, which describes ligamentous separation between the first and second cervical vertebra.

<span class="mw-page-title-main">Radiculopathy</span> Medical condition

Radiculopathy, also commonly referred to as pinched nerve, refers to a set of conditions in which one or more nerves are affected and do not work properly. Radiculopathy can result in pain, weakness, altered sensation (paresthesia) or difficulty controlling specific muscles. Pinched nerves arise when surrounding bone or tissue, such as cartilage, muscles or tendons, put pressure on the nerve and disrupt its function.

<span class="mw-page-title-main">Central cord syndrome</span> Human spinal cord disorder

Central cord syndrome (CCS) is the most common form of cervical spinal cord injury (SCI). It is characterized by loss of power and sensation in arms and hands. It usually results from trauma which causes damage to the neck, leading to major injury to the central corticospinal tract of the spinal cord. CCS most frequently occurs among older persons with cervical spondylosis, however, it also may occur in younger individuals.

<span class="mw-page-title-main">Spinal disease</span> Disease involving the vertebral column

Spinal disease refers to a condition impairing the backbone. These include various diseases of the back or spine ("dorso-"), such as kyphosis. Dorsalgia refers to back pain. Some other spinal diseases include spinal muscular atrophy, ankylosing spondylitis, scoliosis, lumbar spinal stenosis, spina bifida, spinal tumors, osteoporosis and cauda equina syndrome.

<span class="mw-page-title-main">Spinal stenosis</span> Disease of the bony spine that results in narrowing of the spinal canal

Spinal stenosis is an abnormal narrowing of the spinal canal or neural foramen that results in pressure on the spinal cord or nerve roots. Symptoms may include pain, numbness, or weakness in the arms or legs. Symptoms are typically gradual in onset and improve with leaning forward. Severe symptoms may include loss of bladder control, loss of bowel control, or sexual dysfunction.

Craniocervical instability (CCI) is a medical condition characterized by excessive movement of the vertebra at the atlanto-occipital joint and the atlanto-axial joint located between the skull and the top two vertebra, known as C1 and C2. The condition can cause neural injury and compression of nearby structures, including the brain stem, spinal cord, vagus nerve, and vertebral artery, resulting in a constellation of symptoms.

References

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  2. ↑ Rubin, Michael (2023). "Craniocervical Junction Abnormalities". Merck Manual.
  3. ↑ 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.
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  27. ↑ Rossi, Ernest L.; Cozzolino, Mauro; Mortimer, Jane; Atkinson, David; Rossi, Kathryn Lane (October 2011). "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. PMID   22125895. S2CID   34260924.
  28. ↑ 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. PMC   6953136 . PMID   31918713.
  29. ↑ 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. PMID   27465418.
  30. 1 2 3 Waheed, Muhammad Abdul-Aziz; Hasan, Sazid; Tan, Lee A.; Bosco, Aju; Reinas, Rui; Wengel, Paula Valerie ter; Hey, Hwee Weng Dennis; Aleem, Ilyas S. (March 2020). "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. PMC   7154356 . PMID   32309671.
  31. ↑ Knipe, Henry. "Atlanto-occipital assimilation | Radiology Reference Article | Radiopaedia.org". Radiopaedia. Retrieved 2020-12-17.
  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. ↑ The efficacy of manual therapy and therapeutic exercise in patients with chronic neck pain: A narrative review kheljournal.com
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