BackgroundNormally, chronic cerebrospinal venous insufficiency (CCSVI) has been studied using echo-colour Doppler (ECD). Subjects are examined in the supine and sitting positions, in accordance with a static protocol without rotation of the head. A dynamic approach, to assess venous sizes with different degrees of head rotation, has only been performed to improve jugular venous catheterisation. These echographic studies have suggested that head rotation to the contralateral side increases the cross-sectional area (CSA) of the internal jugular veins (IJVs) in supine subjects. Our goal was to evaluate the behaviour of CSA of the IJVs during supine head rotation in multiple sclerosis (MS) patients with CCSVI, compared to healthy controls (HCs).MethodsThe IJVs of 313 MS patients with CCSVI (male 43.8%, male/female 137/176; mean age 45 years old, range 19–77 years) and 298 HCs, matched by gender (male 43.6%, male/female 130/168) and age (mean age 46 years old, range 20–79 years), were compared using ECD. Their CSAs were evaluated with the subjects seated in a tiltable chair, first in the supine position at the level of the cricoid cartilage, with the head in a neutral position, and then after contralateral rotation to 90° from midline.ResultsSignificant differences between the jugular CSAs before and after head rotation were observed only in the MS patients for the IJVs with wall collapse (F[6,1215] = 6414.57, p < 0.001), showing on longitudinal scans a typical “hourglass” aspect that we defined as “miopragic”. No significant difference was found in the distribution of these miopragic veins with regard to MS duration. There was a strong association between the CCSVI scores and the complexity of jugular morphological types (Χ2 [9, N = 313] = 75.183, p < 0.001). Wall miopragia was mainly observed in MS patients with SP (59.3%) and PP (70.0%) clinical forms, compared to RR (48.3%) forms (p = 0.015).ConclusionA dynamic ECD approach allowed us to detect IJVs with a significant increase in their CSAs during head rotation, but only in MS subjects. This feature, most likely the expression of congenital wall miopragia, could be secondary to dysregulation of collagen synthesis, but further histochemical studies will be needed to confirm this hypothesis.

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Background: The pathophysiological mechanism defined "chronic cerebrospinal venous insufficiency" (CCSVI) diagnosed using Zamboni criteria has raised a heated debate about possible correlations with several neurological disorders, but also on subjectivity of these ultrasonographic criteria used for its diagnosis. Although in 2011 new criteria have been introduced to reduce the high inter-observer variability only two studies were conducted according to the new investigation protocol. Therefore, we wanted to verify the impact of the revised protocol and its ability to meet the demand for reduction of the high heterogeneity in inter-observer agreement. Patients and Methods: Between June 2010 and June 2014, 1020 subjects (693 MS patients and 327 HCs) were prospectively screened for CCSVI by two investigators, blinded regarding the observed subject. After exclusion of discordant cases between the two examiners, 630 patients with clinically defined MS and 10 patients with CIS (clinically isolated syndrome) were matched by gender (male 38.7%, male/female 248/392) and age (mean age 44.0 years, range 18.5-77.0 years) with 315 HCs (male 43.2%, male/female 136/179-mean age 46.5 years, range 19.8-79.9 years). Results: The prevalence of CCSVI in MS subjects before the introduction of the new ECD criteria (94.3%) was observed to be significantly reduced (83.4%) after their introduction (p<0.001). In MS patients, the strength of inter-observer agreement changed from moderate (k=0.532) to good (k=0.761) before/after the revision. B-mode analysis detected only 65.7% of valvular defects. Its diagnostic accuracy was 88.6% (95% CI: 84.0%-93.2%), with a sensitivity of 83.5% (95% CI: 75.2%-89.9%), a specificity of 96.0% (95% CI: 88.8%-99.2%), a positive predictive value of 96.8% (95% CI: 91.0%-99.3%) and a negative predictive value of 80.0% (95% CI: 70.3%-87.7%). Conclusions:The new ECD criteria introduced by the revised protocol ensure, at this time, a substantial reduction of the inter-observer variability. Under this perspective, M-mode analysis is essential for its ability to identify the valvular abnormalities frequently not detectable by Bmode analysis.