OBJECTIVE To describe the clinical and electrophysiologic features of patients with inclusion body myositis that was misinterpreted as motor neuron disease. PATIENTS AND METHODS We retrospectively retrieved the medical records of 70 patients with a pathologic diagnosis of inclusion body myositis. From this group, we selected those who had been first diagnosed as having motor neuron disease or...

Sporadic inclusion body myositis (IBM) is an acquired muscle disorder associated with ageing, for which there is no effective treatment. Ongoing developments include: genetic studies that may provide insights regarding the pathogenesis of IBM, improved histopathological markers, the description of a new IBM autoantibody, scrutiny of the diagnostic utility of clinical features and biomarkers, th...

Dr. Marcos R. Gomes de Freitas – Rua Gastão Ruch 16 / 1402 24220-100 Niterói RJ Brasil. E-mail: [email protected] Neurological disorders are frequent complications of human immunodeficiency virus (HIV) type 1 infection, and include central nervous system (CNS) infections, neoplasm, vascular complications, peripheral neuropathies, and myopathies. Early series emphasized CNS diseases, with ...

Inclusion body myositis is an increasingly recognised form of inflammatory myopathy with characteristic clinical and histopathological features which has seldom been reported in the United Kingdom. This paper presents the clinicopathological features of a series of patients diagnosed in Nottingham from 1986 to 1990. During this period, 1319 muscle biopsy samples were processed by this laborator...

Background Within the group of inflammatory myopathies, Inclusion-Body Myositis (IBM) is a distinct pathology with clinical and histological features very different from other entities. In literature, an association between primary Sjögren’s syndrome (pSS) IBM has been previously reported. Objectives The aim our study was to compare infiltrates associated or not pSS and, among patients pSS, mus...

We describe four cases of a new clinicopathological entity presenting with either a frontotemporal dementia or corticobasal degeneration syndrome with a mean age of onset of 45 years (range 41-50) characterized pathologically by deposition of neurofilament proteins. All four patients had a rapidly progressive course and have become mute and non-ambulatory, and three have died after mean illness...

Insoluble, inactive inclusion bodies are frequently formed upon recombinant protein production in transformed microorganisms. These inclusion bodies, which contain the recombinant protein in an highly enriched form, can be isolated by solid/liquid separation. After solubilization, native proteins can be generated from the inactive material by using in vitro folding techniques. New folding proce...

As part of the current research programme these laboratories are investigating the synovia of normal and abnormal human joints by light and electron microscopy. By synovia is meant the material lining joints and tendon sheaths on the interior of the fibrous capsule, including, therefore, synovial cells, loose connective tissue, blood vessels, and inflammatory cells which may be present. Several...

PURPOSE OF REVIEW Inclusion body myositis (IBM) is a poorly understood progressive muscle disease of middle and later life. Its dual pathologies of autoimmunity and unexplained myofiber degeneration and loss have been enigmatic since its earliest descriptions over 40 years ago. No reliable effective therapy currently exists for IBM. This review provides an update of current issues in the pathog...