159 research outputs found
Optimism in inclusion body myositis: a double-blind randomised controlled phase III trial investigating the effect of sirolimus on disease progression in patients with IBM as measured by the IBM Functional Rating Scale
No full textNecrotising autoimmune myopathy
No full textNecrotising Autoimmune Myopathy is a subacute proximal myopathy with high creatine kinase levels and biopsy findings of necrotic and regenerating fibres with minimal inflammation. It is associated with anti-SRP and anti-HMGCR antibodies, malignancy and connective tissue disorders, and is responsive to immunotherapy. This review aims to increase clinician awareness of this rare but potentially treatable condition, by describing the clinical presentation, serological and biopsy findings, and providing an overview of the currently utilised immunotherapy regimens
Advances in inclusion body myositis: genetics, pathogenesis and clinical aspects
No full textIntroduction: Inclusion body myositis is the most common acquired muscle disease affecting older adults. It has an insidious onset with a very specific pattern of muscle involvement, but the aetiopathogenesis is still unknown. Pathologically the combination of inflammatory changes, degenerative changes as well as mitochondrial and nuclear changes are seen, and probably all contribute to the loss of muscle, however the primary abnormality remains a mystery. Treatment is currently supportive, but clinical trials are ongoing and are directed at new targets.
Areas covered: Clinical profile, genetic susceptibility, pathogenesis and treatment
Expert opinion: Understanding the aetiopathogeneis is vital to identify future treatment targets. In addition, understanding the natural history and the roles of biomarkers including the anti-CN1a antibody is vital for designing future clinical trials in IBM, to be properly designed and of sufficient duration to detect clinically significant changes
Necrotizing autoimmune myopathy
No full textPURPOSE OF REVIEW:
Necrotizing autoimmune myopathy (NAM) is a relatively newly recognized subgroup of idiopathic inflammatory myopathies, which despite diverse causes, have the common histopathological features of myocyte necrosis without significant inflammation. Patients present with a subacute severe symmetrical proximal myopathy, associated with a markedly elevated creatine kinase level. These are most likely immune-mediated, as they respond to immunotherapy. The review aims to define this heterogeneous entity and summarize the salient clinical, laboratory, and muscle biopsy findings, in order to facilitate the diagnosis and treatment of this condition.
RECENT FINDINGS:
Statin-associated NAM has been linked with an antibody against the 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) protein, which is up-regulated in regenerating fibres. This finding suggests that NAM is an antibody-mediated disease, and may explain the continuing progression of disease despite cessation of the statin. In addition it may provide a useful diagnostic test in the future, to help differentiate immune from nonimmune statin myopathies.
SUMMARY:
It is important to recognize and distinguish NAM from other causes of myocyte necrosis, because it has the potential of being amenable to treatment
Reply to Lowe and Barth
No full textIn this reply the author attempts to answer criticisms and to clarify some points made in "What Can Systems Theory Do for Archaeology?" (Salmon 1978). Particular emphasis is placed on the distinction between philosophical models of explanation and empirical models of phenomena. Some confusion has arisen because the term "systems model" has been applied to both types.</jats:p
Perceiving voices in contemporary art : an auditory exploration of image, sculpture and architecture
No full textThis thesis investigates some recent developments of sound in contemporary art through an exploration of image, sculpture and architecture. While the areas of image, sculpture and architecture are so crucial to art history that they are included in most important introductory texts to art history, most of these texts remain relatively silent as to the impact that sound and visual art has mutually had on the other. Three contemporary art case studies are presented: (1) Geneviève Cadieux's La voie lactée (1992) an image which incorporates the soundscape, (2) Myriam Laplante's sculptural installation Elixir (2004) that draws on acousmatic sound to give presence to objects, and (3) Janet Cardiff's video walk Conspiracy Theory (2002) a critical examination of the voice in architecture. Through an interpretive exploration of these works, the author seeks to understand how sound might be important to the "silent" image, object, or building. This thesis argues that sound and image can be used together in art to map out new territory, that psychologically stimulating effects occur when sound is projected on to objects, and that sound spaces can be constructed giving an impression of architecture space and material. By approaching sound in art through image, sculpture and architecture, this thesis may assist in establishing the lexicon of sound that is so familiar in other fields of study in art historical terms. This is absolutely crucial for ongoing studies of contemporary art where sound has become so prevalent
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