Craniocervical junction instability (CCI) is a pathology more commonly seen in connective tissue disorder. It is associated with Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disorder leading to hypemobile joints, trauma and other disorders. Neck pain and disability from associated symptoms are prominent features. Dynamic instability is often missed on a recumbent MRI (magnetic resonance imaging). There is little evidence and recommendations in the literature assessing the use of positional upright MRI in Ehlers-Danlos syndrome. We aim to report radiographical features, allied conditions and modified Karnofsky scores in patients suspected of having CCI.
70 patients’ data with suspected CCI was collected from September 2019-August 2021. Inclusion criteria comprised of patients suspected of having CCI. Exclusion criteria included inadequate dynamic imaging, uncertain EDS status. 12 patients out of 70 were excluded. The patients were divided between EDS with its allied disorders and non EDS patients. Cranio – cervical segmental movement parameters in the neutral, extension and flexion positions were measured from upright MRI and CT (computed tomography) scans. These parameters included the basion axial angle (BAA), grabb oakes line (GOL), basion axial interval (BAI), basion dental interval (BDI), and C1/2 rotation. Modified Karnofsky score, presence or absence of Chiari malformations were collected prior to their appointment.
39 out of 58 patients had abnormal findings including 21 GOL, 28 BAA, 17 BAI, 18 rotation abnormal, 17 both BAA and GOL. 30 had EDS, 28 did not. 34 had been diagnosed with myalgic encephalopathy and or chronic fatigue syndrome (MECFS). Student t-test was used to compare both groups, with significant difference for the following parameters (P<0.05) BAA neutral, BAA flexion, GOL neutral GOL flexion, GOL extension (p<0.01), C1/2 rotation left(p<0.001) and right. Upright MRI and rotatory CT are useful tools in the evaluation of patients with CCI. Doctors should maintain a high index of suspicion for CCI. Further studies are needed to quantify these abnormalities and compare to normal population.