Neuropsychiatric manifestations of Sydenham's chorea: a systematic review.

AIM: Sydenham's chorea is a post-streptococcal, autoimmune, neuropsychiatric movement disorder. Sydenham's chorea is a major criterion for diagnosis of acute rheumatic fever with the implication of potential long-term sequelae including cardiac complications. It is well established that there is psychiatric comorbidity in Sydenham's chorea, but there are variations in the literature regarding the nature and prevalence of psychiatric diagnoses associated with Sydenham's chorea. The aim of this review was to systematically evaluate the evidence for psychiatric symptoms presenting with Sydenham's chorea. Knowledge of comorbid psychiatric symptomatology will support early diagnosis and treatment, leading to improved long-term outcomes for children with Sydenham's chorea. METHOD: The study used a systematic search strategy, using MEDLINE, MEDLINE in Process, EMBASE, and The Cochrane Library. Abstracts were screened to identify relevant papers which were then assessed further. Eligible papers were summarized. RESULTS: A total of 1429 abstracts of relevant studies were found, and 49 papers reporting neuropsychiatric symptoms in Sydenham's chorea were summarized. Obsessive-compulsive disorder was the most commonly studied, and hence reported, neuropsychiatric symptom in children with Sydenham's chorea. The studies analysed used a variety of tools to identify affected children and used different methods for analysing results. Attention-deficit-hyperactivity disorder, affective disorders, tic disorders, executive function disturbances, and psychotic features were also reported as comorbidities. INTERPRETATION: There is good evidence of neuropsychiatric comorbidities in Sydenham's chorea. In countries with a high prevalence of rheumatic fever, the early recognition of salient cognitive and psychiatric symptoms may aid in the management of Sydenham's chorea.

Temporal artery biopsy: are we doing it right?

PURPOSE: Temporal artery biopsy (TAB) is the gold standard for diagnosing temporal arteritis; however, sensitivity is relatively poor (30-40 per cent). The British Society of Rheumatology (BSR) guidelines state two major factors that can improve sensitivity: TAB specimen size > 10mm; and pre-biopsy steroid treatment < 7 days. Owing to the low sensitivity, TA treatment is often commenced/continued despite negative histology. The purpose of this paper is to establish the extent to which TAB results influence clinical management and determine specimen adequacy regarding BSR guidelines. DESIGN/METHODOLOGY/APPROACH: In total, 55 patients underwent TAB between 2009-2011. Patients' medical notes were analysed, specifically looking at biopsy specimen size, histology results and steroid therapy duration, pre- and post-biopsy. FINDINGS: From 55 TABs, three (6 per cent) were positive, 47 (85 per cent) were negative and five (9 per cent) were "inadequate". Of those patients with negative results, 18 (46 per cent) received > six months steroid treatment. From 50 "adequate" specimens, 31 (62 per cent) were < 10mm and 11 (28 per cent) received > seven days steroid treatment pre-biopsy. PRACTICAL IMPLICATIONS: Despite negative results, many patients went on to receive long-term steroids. Action must be taken to reduce false and true negative biopsies. False negatives may-be reduced by improving adherence to BSR guidance (increased specimen size and early biopsy after commencing steroids). To reduce total true-negative biopsies, the authors suggest implementing the American College of Rheumatology scoring system, designed to objectify the decision to perform TAB. ORIGINALITY/VALUE: This article addresses a common problem seen in most UK hospitals. There is little literature discussing a plausible solution to reducing negative biopsies.

Notch 1 and 3 receptor signaling modulates vascular smooth muscle cell growth, apoptosis, and migration via a CBF-1/RBP-Jk dependent pathway.

Vascular smooth muscle cell (SMC) fate decisions (cell growth, migration, and apoptosis) are fundamental features in the pathogenesis of vascular disease. We investigated the role of Notch 1 and 3 receptor signaling in controlling adult SMC fate in vitro by establishing that hairy enhancer of split (hes-1 and -5) and related hrt's (hrt-1, -2, and -3) are direct downstream target genes of Notch 1 and 3 receptors in SMC and identified an essential role for nuclear protein CBF-1/RBP-Jk in their regulation. Constitutive expression of active Notch 1 and 3 receptors (Notch IC) resulted in a significant up-regulation of CBF-1/RBP-Jk-dependent promoter activity and Notch target gene expression concomitant with significant increases in SMC growth while concurrently inhibiting SMC apoptosis and migration. Moreover, inhibition of endogenous Notch mediated CBF-1/RBP-Jk regulated gene expression with a non-DNA binding mutant of CBF-1, a Notch IC deleted of its delta RAM domain and the Epstein-Barr virus encoded RPMS-1, in conjunction with pharmacological inhibitors of Notch IC receptor trafficking (brefeldin A and monensin), resulted in a significant decrease in cell growth while concomitantly increasing SMC apoptosis and migration. These findings suggest that endogenous Notch receptors and downstream target genes control vascular cell fate in vitro. Notch signaling, therefore, represents a novel therapeutic target for disease states in which changes in vascular cell fate occur in vivo.

Pneumatosis Intestinalis of the Small Bowel; Radiological and Intra-operative findings.

Pneumatosis Intestinalis is defined as the infiltration of gas into the bowel wall. It is a radiological and intra-operative finding of varying aetiology which varies from benign to life threatening conditions. We describe here a case of a 67 year old woman who presented with diffuse abdominal pain and was found to have Pneumatosis Intestinalis.