Inclusion body myositis with abundant ring fibers

Summary A 37-year-old male presenting with a 7-year history of leg weakness was found to have moderate weakness confined to the quadriceps and myopathic changes on electromyography. Serum creatine phosphokinase was 2130 units/liter. Biopsy of the quadriceps muscle revealed an inflammatory myopathy with cytoplasmic and nuclear filamentous inclusions characteristic of inclusion body myositis. An unusual finding was the large proportion of ring fibers along with severe atrophy and fibrosis. The pathogenesis of the ring fibers in this setting is discussed.     

Inverse relationship between plasminogen activator inhibitor-I activity and adiponectin in overweight and obese women. Interrelationship with visceral adipose tissue, insulin resistance, HDL-chol and inflammation

Adipose tissue is an active endocrine organ secreting different adipokines such as plasminogen activator inhibitor-1 (PAI-1) and adiponectin, among many others. In this study, we investigated the association between PAI-1 activity and serum adiponectin levels in a group of 444 overweight and obese women and assessed the interrelationship with visceral adipose tissue (VAT; CT-scan L4-L5), insulin resistance (HOMA-IR), HDL cholesterol (HDL-chol) and inflammation (hs-CRP). PAI-1 was inversely related to adiponectin (r = -0.25, p < 0.001; adjusted for age and BMI). After adjustment for age, VAT, HOMA-IR and hs-CRP, the relationship remained significant (r = -0.15; p = 0.001), but disappeared after additional adjustment for HDL-chol (r = -0.09; p = 0.067). Subjects were divided in two groups according to the median levels of adiponectin or PAI-1 levels. PAI-1 activity (19.1 +/- 11.4 vs. 15.8 +/- 8.6 AU/ml; p = 0.003) and adiponectin levels (9.8 +/- 4.6 vs. 8.4 +/- 4.0 microg/ml; p < 0.001) were significantly higher in the low adiponectin/PAI-1 groups. The difference in PAI-1 remained significant after adjustment for age and BMI (p = 0.001), became borderline significant after adjustment for age and VAT (p = 0.052), and disappeared after adjustment for age and HOMA-IR (p = 0.116) or age and HDL-chol (p = 0.443). The difference in adiponectin levels remained significant after adjustment for age, VAT, HOMA-IR and hs-CRP (p = 0.006), but disappeared after additional adjustment for HDL-chol (p = 0.089). Further analyses suggest a contribution of HOMA-IR and/or HDL-chol in the relationship between PAI-1 and adiponectin. HDL-chol was found to be the only factor independently determining both factors. In conclusion, in overweight and obese women, PAI-1 activity was inversely related to serum adiponectin, independent of visceral adipose tissue.     

Altered Prefrontal and Hippocampal Function During Verbal Encoding and Recognition in People With Prodromal Symptoms of Psychosis

Despite robust evidence of hippocampal abnormalities in schizophrenia, it is unclear whether hippocampal dysfunction predates the onset of psychosis. We used functional magnetic resonance imaging to investigate hippocampal function in subjects with an at-risk mental state (ARMS). Eighteen subjects meeting criteria for an ARMS and 22 healthy controls, matched for age, gender, and premorbid IQ, were scanned while performing a version of the Deese-Roediger-McDermott false memory task. During an encoding phase, subjects read lists of words aloud. Following a delay, they were presented with 24 target words, 24 semantically related lure words, and 24 novel words and required to indicate if each had been presented before. Behaviorally, the ARMS group made more false alarm responses for novel words than controls (P = .04) and had a lower discrimination accuracy for target words (P = .02). During encoding, ARMS subjects showed less activation than healthy controls in the left middle frontal gyrus, the bilateral medial frontal gyri, and the left parahippocampal gyrus. Correct recognition relative to false alarms was associated with differential engagement of the hippocampus bilaterally in healthy controls, but this difference was absent in the ARMS group. The ARMS was associated with altered function in the medial temporal cortex, as well as in the prefrontal regions, during both verbal encoding and recognition. These neurofunctional differences were associated with diminished recognition performance and may reflect the greatly increased risk of psychosis associated with the ARMS.     

Immunohistochemical analysis of a cerebral arteriovenous malformation obliterated by radiosurgery and presenting with re-bleeding. Case report

The purpose of this study was to analyze immunohistochemical characteristics of the cell population in a radiosurgically obliterated cerebral arteriovenous malformation (AVM) after recurrent hemorrhage. Immunohistochemical reactions were carried out on paraffin-embedded histological sections for von Willebrand factor (FVIII), CD34 endothelial antigen (CD34), vimentin and alpha-smooth-muscle actin (SMA) cytoskeletal proteins. Histopathological analysis revealed that the majority of AVM channels were replaced by hypocellular scar tissue. However some of them still disclosed thrombus organization by granulation tissue seven years after radiosurgery. FVIII and CD34 reactions revealed vessel neoformation in thromboses. Proliferating spindle-shaped cells with SMA and vimentin expression were identified in the granulation tissue. These histopathological findings suggest the role of re-canalization in recurrence of hemorrhage following radiosurgical obliteration of the AVM. Contribution of myofibroblastic elements in the vessels' occlusion after radiosurgery is also demonstrated.     

Ischiocrural Strength May Be a Better Prognostic Marker Than Quadriceps Strength in COPD

It is well known that chronic obstructive pulmonary disease (COPD) patients present with muscle dysfunction that may not correlate with the degree of severity of airflow obstruction. Historically, the strength deficit of the knee extensor musculature (quadriceps) has been described as an independent factor of mortality in COPD. We present the results of a retrospective study with longitudinal follow-up of 60 patients with severe COPD followed for 7 years. During follow-up 22 patients died, mainly of respiratory cause. We have observed that the strength of knee flexor muscles (ischiocrural) and dominant handgrip were independent predictors of mortality in severe COPD patients. However, knee extensor strength (quadriceps) was not an independent predictor of mortality. This is the first study which highlights the importance of knee flexor musculature as a prognostic factor in COPD.     

Extracardiac ice formation during CoolLoop cryoablation of atrial fibrillation

A 75‐year‐old male patient was referred for longstanding atrial fibrillation ablation. We performed this procedure combining an epicardial and endocardial approach. Under general anesthesia and via a left‐sided thoracoscopic approach, we isolated the pulmonary veins (PVs) and the roofline and inferior line were created using a radiofrequency tool. To isolate the endocardial PVs, a transseptal puncture was performed via the groin, and a cryoablation CoolLoop catheter (AFreeze GmbH, Innsbruck, Austria) was advanced into the left atrium. Ice crystals started to appear on the epicardial surface of the left inferior PV antrum after 121 seconds later, those crystals had formed an ice plaque. For the first time in humans, we were able to visualize the transmural effects of cryothermal energy ablation via a CoolLoop catheter on the epicardial surface of the ostium of the PV.