Cervicocranial Syndrome

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 osteoarthritis, and trauma. Treatment options include neck braces, pain medication and surgery. The quality of life for individuals suffering from CCJ syndrome can improve through surgery.

Signs and Symptoms

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

• Vertigo
• Chronic Headache aka Cephalea
• Tinnitus
• Facial Pain
• Ear Pain
• Dysphagia
• Carotidynia
• Neck Pain (ex: during movement such extension and flexion)
• Syncope
• Sinus congestion
• Neck Crepitus Sound
• Loss of vision
• Involuntary eye-movement
• Severe Fatigue
• Chest Pain
• Brain Fog

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 resulting in vertebral subluxation. Examples of cases that can result in cervicocranial syndrome are: car accidents, trauma, osteoarthritis, tumor, degenerative pathology and other numerous causes of vertebral instability. There is no single cause that can mainly cause cervicocranial syndrome.

Genetic

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. 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. This is considered to be an anomaly of cervical bones. 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.

Trauma

Traumatic injuries are caused when external forces damage the cervical spine, giving rise to various symptoms. 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.

Pathophysiology

The body is innervated by spinal nerves that branch off from the spinal cord. 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. 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. 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. 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.

Trauma

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

Diagnosis

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.

Prevention/Treatment

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

• Pressure release via realignment of the vertebrae
• Pain medication: acetominophen, aspirin, or ibuprofen
• Manipulation of neck by Chiropractor : For example, vertigo symptoms can be relieved
• 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)

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.

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. Correction and decompression cervical spinal surgeries significantly increase quality of life and reduce symptoms. Post-surgery, 93 to 100 percent patients report reduced cervicocranical syndrome symptoms such as neck pain.

Epidemiology

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

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," 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" conducted in 2018, concluded that there is a lack of evidence that support therapeutic exercise to reduce neck pain via manipulation.