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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]
Craniocervical instability is more common in people with a connective tissue disease, including Ehlers-Danlos syndromes, [1] osteogenesis imperfecta, and rheumatoid arthritis. [2] It is frequently co-morbid with atlanto-axial joint instability, Chiari malformation , [ 3 ] or tethered spinal cord syndrome .
Patients who exhibit extreme joint hypermobility and connective tissue weakness as a result of Ehlers–Danlos syndrome or Marfan syndrome are susceptible to instabilities of the craniocervical junction; thus they are at risk for acquiring a Chiari malformation. Headache, neck pain, unsteady gait usually during childhood [4] 1.5
The cerebellopontine angle (CPA) (Latin: angulus cerebellopontinus) is located between the cerebellum and the pons. [1] The cerebellopontine angle is the site of the cerebellopontine angle cistern.
Underlying problems (e.g. craniocervical junction abnormalities, postoperative scarring, spinal tumors) are corrected when possible. Surgical decompression of the foramen magnum and upper cervical cord is the only useful treatment, but surgery usually cannot reverse severe neurologic deterioration.
Several indirect measurements on CT can be used to assess ligamentous integrity at the craniocervical junction. The Wackenheim line, a straight line extending along the posterior margin of the clivus through the dens, should not intersect the dens on plain film, with violation of this relationship raising concern for basilar invagination.
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It occurs at the cervicothoracic, thoracolumbar, or lumbosacral junction. For instance, the transverse process of the last cervical vertebra may resemble a rib. A transitional vertebra at the lumbosacral junction can cause arthritis , disk changes, or thecal sac compression.