Home About us Contact
|
Home About us Contact | ||
|
|
||
Proximal Weakness (proximal + weakness)
Selected AbstractsInflammatory myositis in systemic sclerosis: a South Australian perspectiveINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 3 2005T. Y.-T. Abstract Background:, Muscle atrophy and weakness occurs commonly in patients with systemic sclerosis, especially late in the course of the disease. However, profound proximal muscle weakness secondary to myositis is an infrequent finding. Aim:, To determine the frequency and disease characteristics of patients with myositis in our cohort of systemic sclerosis patients. Methods:, A retrospective case note review of the clinical course of all patients enrolled on the South Australian scleroderma register, a population-based register of 374 living and 234 deceased patients with systemic sclerosis, last updated to the end of December 2002. Results:, Twenty patients with myositis were identified, the majority with diffuse cutaneous systemic sclerosis and overlap syndromes. The calculated frequency of this complication was 3.3% in our population-based cohort. All patients suffered profound proximal muscle weakness complicated by functional impairment. Other clinical features included weakness of cervical musculature (15%), dyspnoea (10%) and dysphagia (10%). Creatine kinase level was elevated in 80% of the patients, with the mean peak creatine kinase level of 1129 U/L. When further investigations were undertaken, 80% of patients demonstrated myopathic changes on electromyography and 92% of patients were found to have histological findings characteristic of an inflammatory process. Positive antinuclear antibodies were identified in all patients, including two with anti-PM-Scl autoantibodies. Conclusion:, Myositis is an infrequent clinical feature in patients with systemic sclerosis. Profound proximal weakness in association with elevated creatine kinase levels and myopathic changes on electromyography should alert the clinician to this complication. The presence of anti-PM-Scl autoantibodies in association with overlap syndromes may have a more favourable prognostic significance. [source] POSTIRRADIATION LUMBOSACRAL RADICULOPLEXOPATHY: IMPROVEMENT AFTER IMMUNE THERAPYJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2000A. Bersano A delayed progressive impairment of peripheral nervous system including brachial and lumbosacral radiculoplexopathy is a well-known complication of local radiotherapy. No treatment for this infrequent complication is currently available. Recently, improvement after treatment with high dose immunoglobulin (IVIg) has been reported in some patients, suggesting either an immune-mediated inflammatory nerve damage induced by irradiation or a dysimmune neuropathy (CIDP-like) misdiagnosed as a postirradiation disease. We report on two patients who developed motor lumbosacral radiculoplexopathy several years after local radiotherapy. The first patient (ZA) is a 49 y.o. man developing a progressive proximal>distal weakness and hypotrophy of lower limbs, 20 years after radiotherapy of lumbosacral region for seminoma. Electrophysiological studies showed markedly reduced motor conduction velocities (CV) and prolonged F-wave latencies in lower limb nerves. The second patient (BF), is a 52 y.o. woman who developed progressive left brachial plexopathy and distal>proximal weakness and hypotrophy of lower limbs 12 years after a first course of toracoascellar and lumbar irradiation for Hodgkin lymphoma followed by a second course of cervicoclavicular irradiation for tumor recurrence 7 years later. Electrophysiological studies showed markedly reduced CMAP amplitudes and proportionally reduced CV in motor nerves. No sensory impairment was detected in both patients. CSF protein was elevated in both patients while cells were normal. On the assumption of a possible dysimmune origin of the disease, patient ZA underwent high dose intravenous steroid treatment, while patient BF, who had previously deteriorated after steroids, was treated with IVIg. After treatment, patient ZA became able to walk with less waddling, to rise from the floor and climb stairs without support, and to run. Improvement was less consistent in patient BF, whose right leg strength improved even if she still needed bilateral support to walk. The improvement observed in both patients supports the hypothesis that, at least in some patients, an immune-mediated mechanism may underlie postirradiation radiculoplexopathy. [source] Chronic sensorimotor polyradiculopathy with antibodies to P2: An electrophysiological and immunoproteomic analysisMUSCLE AND NERVE, Issue 1 2008Ricard Rojas-Garcia MD Abstract In this study we report a patient with chronic progressive sensory ataxia, proximal weakness, immunoglobulin M (IgM) monoclonal gammopathy, and elevated protein levels in the cerebrospinal fluid, who showed a good response to prednisone. Electrophysiological study disclosed abnormalities predominantly of late responses (F waves and H reflexes), with no evidence of demyelination in the peripheral nerves, suggesting motor and preganglionic sensory nerve roots as the site of the lesion. An immune-mediated pathogenesis was considered and, to identify possible target antigens, we performed bidimensional electrophoresis and a Western blot study. Based on the suspected lesion site, we used human anterior and posterior root extracts. We identified IgM reactivity against peripheral nerve myelin protein P2. Enzyme-linked immunosorbent assay confirmed IgM reactivity toward one synthetic peptide from P2. To our knowledge, reactivity against P2 has not been reported previously in a paraproteinemic neuropathy. Furthermore, we demonstrated that bidimensional electrophoresis and Western blot of the tissue involved, as determined by clinical and electrophysiological studies, may be useful to establish clinical,immunological correlations in paraproteinemic neuropathies. Muscle Nerve, 2008 [source] Impact of gravity loading on post-stroke reaching and its relationship to weaknessMUSCLE AND NERVE, Issue 2 2007Randall F. Beer PhD Abstract The ability to extend the elbow following stroke depends on the magnitude and direction of torques acting at the shoulder. The mechanisms underlying this link remain unclear. The purpose of this study was to evaluate whether the effects of shoulder loading on elbow function were related to weakness or its distribution in the paretic limb. Ten subjects with longstanding hemiparesis performed movements with the arm either passively supported against gravity by an air bearing, or by activation of shoulder muscles. Isometric maximum voluntary torques at the elbow and shoulder were measured using a load cell. The speed and range of elbow extension movements were negatively impacted by actively supporting the paretic limb against gravity. However, the effects of gravity loading were not related to proximal weakness or abnormalities in the elbow flexor,extensor strength balance. The findings support the existence of abnormal descending motor commands that constrain the ability of stroke survivors to generate elbow extension torque in combination with abduction torque at the shoulder. Muscle Nerve, 2007 [source] Clinical and genetic aspects of distal myopathiesMUSCLE AND NERVE, Issue 11 2001David S. Saperstein MD Abstract Although most muscle disorders produce proximal weakness, some myopathies may manifest predominantly or exclusively distal weakness. Although several congenital, inflammatory, or metabolic myopathies may produce mainly distal weakness, there are several distinct entities, typically referred to as distal myopathies. Most of these are inherited conditions. The distal myopathies are rare, but characteristic clinical and histological features aid in their identification. Advances in molecular genetics have led to the identification of the gene lesions responsible for several of these entities and have also expanded our understanding of the genetic relationships of distal myopathies to other inherited disorders of muscle. This review summarizes current knowledge of the clinical and molecular aspects of the distal myopathies. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 1440,1450, 2001 [source] A novel autoantibody recognizing 200-kd and 100-kd proteins is associated with an immune-mediated necrotizing myopathyARTHRITIS & RHEUMATISM, Issue 9 2010Lisa Christopher-Stine Objective Myofiber necrosis without prominent inflammation is a nonspecific finding in patients with dystrophies and toxic or immune-mediated myopathies. However, the etiology of a necrotizing myopathy is often obscure, and the question of which patients would benefit from immunosuppression remains unanswered. The aim of this study was to identify novel autoantibodies in patients with necrotizing myopathy. Methods Muscle biopsy specimens and serum samples were available for 225 patients with myopathy. Antibody specificities were determined by performing immunoprecipitations from 35S-methionine,labeled HeLa cell lysates. Selected biopsy specimens were stained for membrane attack complex, class I major histocompatibility complex (MHC), and endothelial cell marker CD31. Results Muscle biopsy specimens from 38 of 225 patients showed predominantly myofiber necrosis. Twelve of these patients had a known autoantibody association with or other etiology for their myopathy. Sixteen of the remaining 26 sera immunoprecipitated 200-kd and 100-kd proteins; this specificity was observed in only 1 of 187 patients without necrotizing myopathy. Patients with the anti-200/100 autoantibody specificity had proximal weakness (100%), high creatine kinase levels (mean maximum 10,333 IU/liter), and an irritable myopathy on electromyography (88%). Sixty-three percent of these patients had been exposed to statins prior to the onset of weakness. All patients responded to immunosuppressive therapy, and many experienced a relapse of weakness when the medication was tapered. Immunohistochemical studies showed membrane attack complex on small blood vessels in 6 of 8 patients and on the surface of non-necrotic myofibers in 4 of 8 patients. Five of 8 patients had abnormal capillary morphology, and 4 of 8 patients expressed class I MHC on the surface of non-necrotic myofibers. Conclusion An anti,200/100-kd specificity defines a subgroup of patients with necrotizing myopathy who previously were considered to be autoantibody negative. We propose that these patients have an immune-mediated myopathy that is frequently associated with prior statin use and should be treated with immunosuppressive therapy. [source] Approach to the patient with chronic polyneuropathyACTA NEUROLOGICA SCANDINAVICA, Issue 2007Å. Mygland Background ,, Chronic polyneuropathy is a common disorder with heterogenic clinical presentation and many different etiologies. Diagnostic investigation is challenging. Materials and methods ,, An algorithm for diagnostic investigation of chronic polyneuropathy is presented. It was designed to secure practical usefulness for general neurologists, identification of the most common causes with an adequate number of laboratory tests, and more focused investigation when necessary. The proposal is based on review of articles found by PubMed search using the terms ,,peripheral neuropathy, cause, and investigation'', relevant book chapters, and own clinical experience. Results ,, All patients should undergo a routine investigation for the most common causes of polyneuropathy by asking for diabetes, heredity, alcohol abuse, toxic medications and agents, symptoms of Sjögren's syndrome, renal failure, and the following laboratory tests; glucose, haemoglobin, leucocytes, thrombocytes, ESR, creatinin, ALAT, GT, vitamin B12, serum electrophoresis, TSH and thyroxin. If routine investigation is negative, a targeted approach based on clinical type and electrophysiological findings is recommended. The most common type with slowly progressive, symmetric sensory symptoms beginning in the feet can often be classified as cryptogenic without further investigation. Polyneuropathies with subacute onset, progressive weakness, asymmetric symptoms, proximal weakness, selective involvement of sensory fibers or demyelinating pathology, or other organ manifestations, have various etiologies that necessitate individual focused investigation. Interpretation ,, Diagnostic investigation of polyneuropathy can be simplified and systematized. [source] | ||||||||||||||||||||||