Background and aims: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. There are few prospective studies validating risk factors for development of PVT. We analysed the incidence, factors associated with PVT development and its influence on cirrhosis decompensations and orthotopic liver transplant (OLT)-free survival. Methods: In this prospective observational study between January 2014 and March 2019, 445 consecutive patients with chronic liver disease were screened and finally 241 with cirrhosis included. Factors associated with PVT development and its influence on cirrhosis decompensations and OLT-free survival by time dependent covariate coding were analysed. Results: Majority of patients belonged to Child-Pugh class A 184 (76.3%) and the average MELD score was 10 ± 5. Previous cirrhosis decompensations occurred in 125 (52.1%), 63 (26.1%) were on NSBB and 59 (27.2%) had undergone banding for bleeding prophylaxis. Median follow-up was 29 (1-58) months. Cumulative incidence of PVT was 3.7% and 7.6% at 1 and 3 years. Previous decompensation of cirrhosis and low platelet counts but not NSBB independently predicted the development of PVT. During follow-up, 82/236 (34.7%) patients developed cirrhosis decompensations.OLT-free survival was 100% and 82.8% at 3 years, with and without PVT respectively. MELD score, but not PVT, independently predicted cirrhosis decompensations (HR 1.14; 95%CI:1.09-1.19) and OLT-free survival (HR 1.16;95%CI:1.11-1.21).

No abstract

We have completed a survey of European neurological practice concerning cerebral vasculitis. Twenty-nine respondents from 15 countries provided information concerning the diagnosis and management. The results confirmed the anticipated low frequency of the disease, but also illustrated the power of any putative collaborative effort. Interestingly, there was a wide variation in clinical practice, in particular concerning the perceived importance of cerebral angiography as a diagnostic test and the very common use of steroids as first-line treatment, rather than more potent immunosuppressive agents. This variation is probably to be explained at least, in part, by the absence of any firm evidence base to inform clinical practice. A European collaborative effort -in which there has emerged considerable interest -offers a realistic opportunity to generate sound clinical evidence and thence scientifically robust practical guidelines.

We present a retrospective study of 6 patients with spinal cord infarction in the territory of the Adamkiewicz artery. In all patients, the clinical picture was stereotyped: sudden onset of paraplegia and bilateral radicular pain, dissociated sensory loss below the level of infarction and sphincter dysfunction. Emergency neuroradiological investigation ruled out a compressive lesion in all cases. In one patient, spinal angiography was performed and identified an occlusion of the Adamkiewicz artery. Treatment was supportive and all patients had a substantial recovery over a period of weeks.

Gorham-Stout syndrome is a rare disease characterized by progressive osteolysis leading to disappearance of the bone. Vascular proliferations have been implicated in the pathogenesis of this syndrome. The case of a 7-year-old girl with a prominent invasive lymphatic malformation on the lumbosacral area and massive osteolysis of the pelvic girdle is reported.