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At least 217 records · Page 12Linked to original sources
PubMed2001

[When should you consider a muscular disease?].

Muscle diseases can take different clinical aspects and some unusual symptoms are often underestimated. Weakness of proximal limb muscles is the most frequent but distal weakness can be observed. Sometimes oculomotor, bulbar, facial, respiratory, paraspinal muscles or myocardium involvement may be the expression of a muscle disease. Similarly it is important to recognize a muscle disease in occurrence of neonatal hypotonia, muscle hypertrophy, muscle stiffness, transitory paralytic episodes, fatigability, myalgia, rhabdomyolysis or isolated increased creatine-kinases level. Biological and electrophysiological examinations have essentially a role in pointing a muscle disease. Muscle biopsy keeps the key role in the diagnosis. Muscle imaging allows mapping of the lesions and new techniques of NMR spectroscopy constitute an in vivo approach of muscle metabolism.

Biopsy↗
PubMedDate not supplied

[Characteristics in the treatment of scoliosis in muscular diseases].

INTRODUCTION: Patients suffering from the most frequent muscle disorders Duchenne muscular dystrophy (DMD) and spinal muscular atrophies (SMA), who ceased walking respectively are confined from the outset to the wheel-chair, are developing commonly a progressive scoliosis (collapsing spine) due to an increasing muscle weakness. Basing on the pelvic obliquity these scolioses are leading first of all to problems in sitting as well as difficulties in trunk and head control. Along with the increasing weakness of respiratory muscles these phenomena entail a restrictive respiratory insufficiency. CONSERVATIVE TREATMENT: An effective conservative treatment is not available for these scolioses. The use of a corset, however, can only be taken into consideration as a compromise, either for very young patients or those who refused an operation respectively who have reached an inoperable stage. The exclusive use of so-called "anatomic sitting supports" in the wheel-chair in order to treat or prevent a progressive scoliosis in DMD or SMA is absolutely to be rejected. They should only be applied for very young patients with SMA type II as a transitional solution until a corset or better an surgical stabilisation of the spine will be effected, or as a palliative measure in late stages. SURGICAL TREATMENT: Only the early as possible performed surgical stabilisation of the spine using adequate instrumentation (Luque, CD or modifications), enabling an early mobilization without corset or cast, is the most effective treatment of these scoliosis. Patients with DMD or SMA type III should be stabilized after loss of walking ability and definitive confinement to wheel-chair, if the curve is more than 20 degrees-30 degrees Cobb and progressive and forced vital capacity (FVC) is > 35%. The instrumentation should be applied between D3 or D4 and sacrum. The bony fusion mass should include the lumbar and lumbosacral region. The unfused instrumentation with the telescope-rod after Naumann is a good solution for patients with SMA type II and progressive curves already in the early childhood from ca. 5 years of age. First of all surgical spinal stabilisation improves the sitting comfort. Over and above this the improved cosmetic appearance should not be underestimated for the psychological condition of these patients. Additionally it is proved, that surgical stabilisation of the spine prolongs the life expectancy of patients with DMD. Furthermore stabilization of lung function can be achieved for both DMD and SMA patients in comparison to the natural history of these diseases. Altogether a decisive improvement of quality of life can be reached for all these patients.

Adolescent↗
PubMed1989

[Routine diagnostic tests in muscular diseases].

The main investigations routinely used for the diagnosis of muscle disease include biological and electrophysiological analyses, as well as muscle biopsy. The results of all these tests are discussed in order to their diagnostic and prognostic value in suspected or known muscle disease. However, the diagnosis can only be confirmed when all the data has been obtained: clinical, biological, electrophysiological, biopsy as well as familial or genetic data. When the data are discordant, it should be possible to proceed to nuclear magnetic resonance spectroscopy, and mitochondrial enzyme profiles.

Biopsy↗
PubMed1979

[Pathogenetic therapy of nervous system and muscular diseases using large doses of prednisolone on alternate days].

A total of 230 patients with myasthenia, polymositis, disseminated sclerosis and myeloradiculopolyneuronitis were administered high doses of prednisolone (up to 100 mg) every other day. The treatment efficacy and low incidence of side effects and complications are emphasized. High doses of prednisolone given every other day do not inhibit secretions of endogenic ACTH and cortisol. The immunodepressant effect of the drug remains unchanged.

Adolescent↗
PubMed1984

A genetic variant of Emery-Dreifuss disease. Muscular dystrophy with humeropelvic distribution, early joint contracture, and permanent atrial paralysis.

A 38-year-old woman, a product of consanguineous parents, had been observed to have limited neck flexion and elbow joints contracture since early childhood. In addition, she experienced humeropelvic muscular weakness and atrophy, so that she was unable to walk by age 27. At 34 years of age, she required a permanent pacemaker to treat complete atrioventricular block with ventricular bradycardia. A myocardial biopsy confirmed cardiomyopathy. The clinical features of the present case are similar to those of the Emery-Dreifuss syndrome; however, this case may be inherited through an autosomal recessive trait.

Adult↗
PubMed1977

Mitochondrial abnormalities in some human muscular diseases and in experimental ischemic myopathy.

Morphologic abnormalities have been observed in two cases of human polymyositis and in three cases of ocular myopathies. Similar findings can be observed in experimental ischemic myopathy. "Ragged red" fibres, with anomalous distribution of oxidative enzymes, mitochondrial alterations, with presence of intracristal paracrystalline inclusions and degenerative myofibrillar changes are the similar features. The similarity between some of these alterations, expecially mitochondrial changes, is remarkable, but it is difficult to correlate them to the primary etiology of described human myopathies.

Adult↗
PubMed2002

[Camptocormia: an infrequent muscular disease].

The camptocormia is an entity characterized by a kyphosis lumbar reductible in supine decubitus, associated to dysfunctions in computed tomography scans and histologics in the paravertebrals muscles of the lumbar segment. We contribute a new case corresponding to a 70 year-old woman with chronic lumbar pain and kyphosis dorsolumbar reductible, in who the on-line tomography showed compatible images with fatty degeneration and atrophy of the lumbar musculature, discoveries that were confirmed in the histologic study.

Aged↗
PubMed2000

[Macrophagic myofasciitis. Study and Research Group on Acquired and Dysimmunity-related muscular diseases (GERMMAD)].

UNLABELLED: MACROPHAGIC MYOFASCIITIS: A most unusual inflammatory myopathy, first described by Germmad had been reported with increasing frequency since 1993 in the leading French myopathology centers. We present our experience with this new disease: macrophagic myofasciitis. CLINICAL FEATURES: By November 1999, 70 cases of macrophagic myofasciitis had been recorded since our first description. The first 22 patients (sex ratio M/F = 1:3) referred with the presumptive diagnosis of polymyositis (n = 11), polymyalgia rheumatica (n = 5), mitochondrial cytopathy (n = 4), and congenital myopathy or muscle dystrophy (n = 1 each). Symptoms included myalgia (91%), anthralgia (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%). LABORATORY FINDINGS: Abnormal laboratory findings included elevated CK levels (50%), markedly increased erythrocyte sedimentation rate (37%), and myopathic EMG (35%). Muscle biopsy showed a unique myopathological pattern characterized by: i) centripetal infiltration of epimysium, perimysium and perifascicular endomysium by sheets of large cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-, with a PAS-positive content; ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; iii) presence of occasional CD8+ T-cells; iv) inconspicuous muscle fiber damage. The picture was easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. The infectious diseases know to be associated with reactive histiocytes, including Whippleís disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients improved under corticosteroid therapy and/or immunomodulatory therapeutic CONCLUSION: A new inflammatory muscle disorder, characterized by a distinctive pathological pattern of macrophagic myofasciitis is emerging in France.

Adult↗
PubMed1989

[Muscular diseases in relation to drug consumption].

Toxic myopathies are an uncommon manifestation of chemotherapeutic agents. Most myopathies are characterized by a weakness affecting proximal limb-muscles more than distal ones. Steroids, chloroquine and ipecac syrup are the most common drugs inducing myopathies. Toxic myositis is rare and reported with D-penicillamine abuse. Myotoxicity of local agents injection such as anesthetics, steroids or antibiotics is also known. Congenital myopathies such as malignant hyperthermia, hypokalemic periodic paralysis or paroxysmal myoglobinuria may also be induced by drugs but are very rare.

Antipsychotic Agents↗
PubMed1994

[Use of Diprivan in muscular diseases and malignant hyperthermia].

An unknown myopathy can be revealed by the administration of an anaesthetic agent. The symptoms are those of malignant hyperpyrexia (MH). The MH phenotype can be detected by means of contracture tests in vitro. All anaesthetics, excepting the triggering agents, can be given. The safety of propofol as an induction and maintenance agent in this category of patients has been demonstrated. When the presence of a myopathy is known before an anaesthetic, the administration of succinylcholine associated or not with a halogenated inhalational agent carries a risk of severe complication. Among the anaesthetic agents having little effect on the skeletal muscle, propofol seems interesting, also because of its pharmacokinetic properties. Myopathy is difficult to diagnose, either because the patient undergoes surgery before being symptomatic or because he is only a carrier of MH. In case of an abnormal reaction following the administration of recognized triggering agents or the occurrence of MH, the procedure should be discontinued. In case of absolute necessity, the procedure may be continued but with non-triggering agents only.

Anesthesia, Intravenous↗
PubMed1979

[Effect of hemodialysis on the creatine kinase activity in inflammatory muscular diseases].

In 10 patients with polymyositis at different stages of the intensity of clinical changes the effect of serum dialysis on CPK activity was studied. The controlgroup comprised 5 patients with Duchenne type myopathy and 11 healthy subjects. In 80% of patients with polymyositis a fall in CPK activity was observed after dialysis, similarly as in all patients with Duchenne type dystrophy and in healthy subjects. In the ramaining patients with polymyositis a significant rise in CPK activity occurred after dialysis. These patients were in the acute stage of the disease and had not yet been treated.

Acute Disease↗
PubMed1978

[The myocardiopathies of hereditary neuro-muscular diseases].

This joint work has studied the cardiomyopathies occurring in hereditary neuro-muscular disorders (270 cases). The Duchenne type of disorder (74 cases) was responsible for asystole (4 cases), for cardiomegaly, and especially for abnormalities of the ECG (59 cases)--Q waves and large R waves in V1 and V6. The cardiomyopathy was of the hypokinetic type, with histological evidence of degeneration of the myocardial fibres. Dystrophia myotonica of Steinart (23 cases) caused conductive disorders (17 cases) which were either atrioventricular or intra-ventricular or both. Studies of the His pathway confirmed that these abnormalities were more diffuse in 5 cases. The main histological feature was interstitial fibrosis. There was a high risk of sudden death; ECG follow-up should be close. Friedreich's disease (20 cases) in its complete form led to later development of obstructive cardiomyopathy, with a systolic ejection murmur, cardiomegaly, and abnormalities of the ECG--left ventricular hypertrophy in the vertical axis, right ventricular and septal hypertrophy, repolarisation disorders similar to those found in coronary artery disease. Histology showed hypertrophy with degeneration of the myocardial fibres and interstitial fibrosis. This complete form was rare (7 cases out of 20); on the other hand, ECG abnormalites were very common (16 cases out of 20). The authors have tried to study the relationships between primary cardiomyopathies (50 cases) and peripheral neuromuscular disorders. 17 of the 39 peripheral muscle biopsies were abnormal, but a well-defined muscular dystrophy could not be found in them.

Cardiomyopathies↗
PubMed1995

[The value of in-vivo 31-phosphorus spectroscopy in the diagnosis of generalized muscular diseases. The clinical results and the differential diagnostic aspects].

PURPOSE: In a prospective study, characteristics and the diagnostic potential of in vivo 31-phosphorus spectroscopy in cases of generalised muscle diseases were analysed. METHOD: 41 patients with myogenic and neurogenic muscle diseases and 11 healthy volunteers were examined using MRI and in vivo 31-phosphorus spectroscopy by means of a 50 mm double-tuned surface coil. RESULTS: The spectra showed significant changes of the metabolite ratios depending on the degree of the disease. Inflammatory muscle diseases were characterised by increased PME and PDE peaks, which indicates that there is a higher conversion of the cell membrane. The spectra of muscular dystrophy showed a slight increase of PDE and Pi. A strongly reduced PCr and an increased Pi peak were demonstrated in cases of muscle atrophy depending on their degree of markedness. The pH values were minimally increased in comparison to the volunteers. In other muscle diseases, such as glycogenosis or myotonia, no significant changes were detected. CONCLUSION: Standardised in vivo 31-phosphorus spectroscopy of generalised muscle diseases provides noninvasive prognostic information on the type and behaviour of the disease and is complementary to clinical and histological findings.

Adolescent↗
PubMed1978

[Isoenzymes of creatine kinase: distribution in the skeletal muscle and in sera of patients with muscular diseases or damages (author's transl)].

In skeletal muscle isoenzymes of CK were determined by immunprecipitation and chromatography. The activity of CK-MB was between 17 and 47 U/g muscle, corresponding to a quota between 2,1 and 4,2% of the total activity. In sera of patients with muscular dystrophy, polymyositis, hypothyroidism, after arterial embolism, epilepsy, hyperventilation, operations and polytrauma with and without injury to the thorax isoenzymes were measure by immune precipitation- and immune inhibition-test. The percentage of CK-MB in all sera was less than 6% of the total CK-activity (range: 0 to 6%). Only patients in the first day after neurosurgical operations showed a quota till to 6.5% CK-MB. In serum of patients after polytrauma without injury to the thorax the percentage of CK-MB ranged from 0-5.7% while after polytrauma with injury to the thorax and a reasonable suspicion of a damage to the myocardium this quota was between 5.1 and 23.6% of the total activity. CK-BB activity was not detectable in any cases. Therefore a disease or damage of the skeletal muscle is more probable, if the percentage of CK-MB in less than 6%, because in sera of patients with myocardial infarction in the first 48 h after beginning of the symptoms this quota of CK-MB in the most cases in more than 6%.

Creatine Kinase↗