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PubMed2006

[Pathophysiology of neuromuscular impairments in the critically ill patient].

OBJECTIVE: to analyze the causes and manifestations of muscle weakness that critically ill patients develop during their staying at the ICU, and literature review. DEVELOPMENT: in the early 1980s, a mixed axonal polyneuropathy was described in septic critically ill patients, which clinically manifested by muscle weakness of variable severity, leading to quadriplegia and/or ventilator dependency in its most severe presentation. Almost at the same time, an acute intensive care myopathy was described in asthmatic patients admitted to the ICU for asthma exacerbation. Later on, this myopathy was also observed in transplanted, septic, or burnt patients. Several authors consider acute intensive care myopathy as the main cause of muscle weakness in the ICU. CONCLUSIONS: in this article, we describe the clinical presentation, etiopathogenesis, diagnosis, and treatment of polyneuropathy of the critically ill patient and of acute intensive care myopathy. Both clinical pictures may be differentiated, with neurophysiological studies and eventually muscle biopsy being of great help. Although some authors rather include these conditions under the name of polyneuromyopathy, we propose the general denomination of Acute Neuromuscular Syndrome of the Critically Ill Patient, a more descriptive term no presupposing a single mechanism or etiology.

Critical Illness↗

PubMed1986

A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms.

A "post-polio" syndrome characterized by new neuromuscular symptoms, including muscle weakness, may develop years after recovery from acute paralytic poliomyelitis. We studied 27 patients (mean age, 50.6 years) in whom new muscle weakness developed a mean of 28.8 years after recovery from acute polio. We reevaluated these patients during a mean follow-up period of 8.2 years (range, 4.5 to 20) after they were originally studied at the National Institutes of Health. The total mean follow-up period after the onset of new weakness was 12.2 years (range, 6 to 29). The patients were assessed with quantitative muscle testing, muscle biopsy, electromyography, and virologic and immunologic examination of the cerebrospinal fluid. Muscle strength had declined in all patients. The rate of decline averaged 1 percent per year. The decrease was irregular, with subjective plateau periods that ranged from 1 to 10 years. None of the patients had amyotrophic lateral sclerosis. Oligoclonal bands (IgG) were found in the cerebrospinal fluid of 7 of 13 patients studied, but no specific elevation of antibodies to poliovirus was observed in the cerebrospinal fluid. The newly affected muscles that were evaluated longitudinally with follow-up muscle biopsies and electromyography showed signs of chronic and new denervation. Groups of atrophic muscle fibers (group atrophy) and "neurogenic jitter" were not present. New post-polio muscle weakness is not a life-threatening form of motor-neuron deterioration. It appears that this weakness is not due to a loss of whole motor neurons, as in amyotrophic lateral sclerosis, but that it is due to a dysfunction of the surviving motor neurons that causes a slow disintegration of the terminals of individual nerve axons.

Adult↗

PubMed2006

Clinicopathological phenotype of ALS with a novel G72C SOD1 gene mutation mimicking a myopathy.

A 71-year-old woman with a family history of amyotrophic lateral sclerosis (ALS) was investigated for symmetrical, proximal limb and abdominal muscle weakness. Initial examination showed mild proximal muscle weakness in the arms and legs, slightly elevated serum creatine kinase (CK) level, and normal electromyographic (EMG) findings. A myopathy was the presumed diagnosis. Over the next year, weakness became severe and tendon reflexes became unelicitable; no upper motor signs were present. EMG then showed acute and chronic denervation and a muscle biopsy showed target fibers and grouped atrophy. DNA analysis revealed a G72C CuZn-superoxide dismutase (SOD1) mutation. Fasciculations were absent throughout the disease. The patient died 53 months after symptom onset and autopsy revealed loss of lower motor neurons (LMN) and SOD1-positive inclusions. This case expands the phenotypic spectrum of ALS associated with SOD1 mutations to include presenting features that mimic a myopathy.

Aged↗

PubMed2002

[Three patients with divergent presentations of idiopathic inflammatory myopathy].

The group of idiopathic inflammatory myopathies encompasses polymyositis, dermatomyositis and inclusion body myositis. These diseases share the following features: progressive muscle weakness, an increase in serum creatine kinase activity and the presence of mononuclear cell infiltrates in the muscle biopsy. Polymyositis, dermatomyositis and inclusion body myositis are differentiated on the basis of the distribution of muscle weakness, and specific histopathological features. Many specialties may see these patients as the clinical presentation can vary widely and may be atypical, requiring further diagnostic procedures. A 40-year-old man with a heliotrope rash and periorbital oedema, but no muscle involvement, was diagnosed with dermatomyositis sine myositis. He was successfully treated with corticosteroids but died later of cardiac failure. A 72-year-old man with a pulmonary malignancy subsequently developed the clinical features of dermatomyositis. Steroid therapy diminished the complaints but he died of pulmonary embolism. A 54-year-old woman with the clinical features of inclusion body myositis did not have rimmed vacuoles in her muscle biopsy specimen and was initially erroneously diagnosed with polymyositis, for which she was treated with corticosteroids, but without beneficial effect.

Adult↗

PubMed2006

Cytokines in idiopathic inflammatory myopathies.

Recent findings suggest cytokines as important key molecules in the pathogenic mechanisms of idiopathic inflammatory myopathies, myositis. In this review, we focus on cytokines with a potential role in disease mechanisms in myositis and present some general information on individual cytokines and an updated summary from the literature concerning cytokines in these disorders. The idiopathic inflammatory myopathies is a heterogeneous group of disorders clinically characterized by symmetric proximal muscle weakness and by certain defined histolopathological findings, including inflammatory infiltrates in muscle tissue. Other prominent findings in the target tissue of these patients are defined molecular changes of blood vessels and muscle fibers, including reformation to high endothelial venule (HEV)-like blood vessels and intensive MHC class I expression in muscle fibers. The predominant clinical symptoms of muscle weakness and decreased muscle endurance are shared by all subsets of inflammatory myopathies and indicate that some pathogenic mechanisms related to muscle function may be shared by the different disease groups. Studies on cytokine gene, RNA and protein expression in muscle tissue from patients with various forms of the disease also indicate similar profiles, despite different phenotypes of the inflammatory cells present in muscle tissue from the different subsets of myositis. There is a pronounced expression of various cytokines in muscle tissue, among which the proinflammatory cytokines TNF-alpha and IL-1 are most widely explored in the inflammatory myopathies, which has made them into potential therapeutic targets. The use of targeted cytokine therapy has been successful in several other chronic inflammatory diseases and although the exact role of cytokines in chronic idiopathic inflammatory myopathies remains to be delineated their potential role as targets for new therapies in this disorder will be discussed in this review.

Animals↗

PubMed1995

Findings in post-poliomyelitis syndrome. Weakness of muscles of the calf as a source of late pain and fatigue of muscles of the thigh after poliomyelitis.

The purpose of this study was to identify overuse of muscles and other alterations in the mechanics of gait in twenty-one patients who had muscular dysfunction as a late consequence of poliomyelitis. All of the patients had good or normal strength (grade 4 or 5) of the vastus lateralis and zero to fair strength (grade 0 to 3) of the calf, as determined by manual testing. Dynamic electromyography was used, while the patients were walking, to quantify the intensity and duration of contraction of the inferior part of the gluteus maximus, the long head of the biceps femoris, the vastus lateralis, and the soleus muscles. Patterns of contact of the foot with the floor, temporal-spatial parameters, and motion of the knee and ankle were recorded. The principal mechanisms of substitution for a weak calf muscle fell into three groups: overuse of the quadriceps (twelve patients) or a hip extensor (the inferior part of the gluteus maximus in eight patients and the long head of the biceps femoris in four), or both; equinus contracture (twelve patients); and avoidance of loading-response flexion of the knee (five patients). Most patients used more than one method of substitution. These observations support the theory that post-poliomyelitis syndrome results from long-term substitutions for muscular weakness that place increased demands on joints, ligaments, and muscles and that treatment--based on the early identification of overuse of muscles and ligamentous strain--should aim at modification of lifestyle and include use of a brace.

Ankle Joint↗

PubMed1987

[Leigh's subacute necrotizing encephalomyelopathy due to decreased activity of the pyruvate dehydrogenase complex].

A new patient with Leigh's syndrome (subacute necrotizing encephalomyelopathy due to pyruvate dehydrogenase complex deficiency) is presented. A Turkish boy of consanguinously married healthy parents developed progressive muscle weakness since infancy. At the age of 3 years he was unable to sit, stand or walk. Clinical examination showed general muscle weakness, hypotonia, muscle hypotrophy, bilateral ptosis, partial bilateral external ophthalmoplegia, nystagmus, intention tremor and hypoactive tendon reflexes. The EEG showed diffuse slowing, the cerebral CT scan disclosed mild hydrocephalus e vacuo. Motor nerve conduction velocity was slightly decreased, the EMG revealed signs of neuropathy. In the biopsied muscle only a mild hypotrophy of type 2 fibres was found, no abnormal mitochondria could be detected. The sural nerve was slightly abnormal: loss of large myelinated axons, loss of unmyelinated nerves. CSF protein was elevated to 80 mg/dl, protein electrophoresis revealed the pattern of markedly impaired blood-CSF barrier. Serum lactate and pyruvate were permanently elevated. In the urine the excretion of alanine was raised. The clinical state deteriorated during intercurrent infections; somnolence, vomiting and Cheyne-Stoke's respiration occurred. At the age of 3 1/2 years the child died of pneumonia. In the liver tissue a decreased activity of the pyruvate dehydrogenase complex was found. Neuropathological examination of the brain demonstrated wide-spread changes of Leigh's spongiform encephalopathy. Several enzyme deficiencies have hitherto been associated with Leigh's syndrome: This patients confirms earlier findings that a subgroup of Leigh's syndrome is caused by pyruvate dehydrogenase complex deficiency.

Biopsy↗

PubMed2003

A role for anabolic steroids in the rehabilitation of patients with COPD? A double-blind, placebo-controlled, randomized trial.

STUDY OBJECTIVES: Skeletal muscle weakness commonly occurs in patients with COPD. Long-term use of systemic glucocorticosteroids further contributes to muscle weakness. Anabolic steroids could be an additional mode of intervention to improve outcome of pulmonary rehabilitation by increasing physiologic functioning, possibly mediated by increasing erythropoietic function. PATIENTS AND METHODS: We randomly assigned 63 male patients with COPD to receive on days 1, 15, 29, and 43 a deep IM injection of 50 mg of nandrolone decanoate (ND) [Deca-Durabolin; N.V. Organon; Oss, The Netherlands] in 1 mL of arachis oil, or 1 mL of arachis oil alone (placebo) in a double-blind design. All patients participated in a standardized pulmonary rehabilitation program. Outcome measures were body composition by deuterium and bromide dilution, respiratory and peripheral muscle function, incremental exercise testing, and health status by the St. George's Respiratory Questionnaire. RESULTS: Treatment with ND relative to placebo resulted in higher increases in fat-free mass (FFM; mean, 1.7 kg [SD, 2.5] vs 0.3 kg [SD, 1.9]; p = 0.015) owing to a rise in intracellular mass (mean, 1.8 kg [SD, 3.1] vs - 0.5 kg [SD, 3.1]; p = 0.002). Muscle function, exercise capacity, and health status improved in both groups to the same extent. Only after ND were increases in erythropoietic parameters seen (erythropoietin: mean, 2.08 U/L [SD, 5.56], p = 0.067; hemoglobin: mean, 0.29 mmol/L [SD, 0.73], p = 0.055). In the total group, the changes in maximal inspiratory mouth pressure (PImax) and peak workload were positively correlated with the change in hemoglobin (r = 0.30, p = 0.032, and r = 0.34, p = 0.016, respectively), whereas the change in isokinetic leg work was correlated with the change in erythropoietin (r = 0.38, p = 0.013). In the patients receiving maintenance treatment with low-dose oral glucocorticosteroids (31 of 63 patients; mean, 7.5 mg/24 h [SD, 2.4]), greater improvements in PImax (mean, 6.0 cm H(2)O [SD, 8.82] vs - 2.18 cm H(2)O [SD, 11.08], p = 0.046), and peak workload (mean, 20.47 W [SD, 19.82] vs 4.80 W [SD, 7.74], p = 0.023) were seen after 8 weeks of treatment with ND vs placebo. CONCLUSIONS: In conclusion, a short-term course of ND had an overall positive effect relative to placebo on FFM without expanding extracellular water in patients with COPD. In the total group, the improvements in muscle function and exercise capacity were associated with improvements in erythropoietic parameters. The use of low-dose oral glucocorticosteroids as maintenance medication significantly impaired the response to pulmonary rehabilitation with respect to respiratory muscle function and exercise capacity, which could be restored by ND treatment.

Aged↗

PubMed2003

[Polymyositis with ventilatory incapacity and respiratory insufficiency].

The inflammatory diseases of muscle are a group of disorders characterized by proximal muscle weakness. Most cases fall into three major diagnostic categories, polymyositis (PM), dermatomyositis and inclusion body myositis. Respiratory complications are a common feature and are an important cause of morbidity and mortality. The three main types of pulmonary involvement are interstitial lung disease, aspiration pneumonia and ventilatory incapacity (VI) due to muscle weakness. There are few reported cases in which mechanical ventilation has been used in patients with PM and VI in absence of lung disease. We present a patient with PM and VI due to muscle weakness who underwent therapy maintenance with mechanical ventilator and was weaned 4 months later.

Female↗

PubMed2000

[A case of polymyositis presenting pregnancy with acute respiratory failure].

A 33-year-old pregnant woman developed respiratory difficulty with bilateral pleural effusion 31 weeks into gestation. On admission she had an elevated serum level of creatine kinase, but muscle weakness in the extremities was mild. After an immediate and successful Cesarean section, she developed respiratory failure and mechanical ventilation was required. The patient was diagnosed as having polymyositis from the limb muscle biopsy. She was treated with dexamethasone at a dose of 8 mg, methylprednisolone++, 1 g daily for three days, and then prednisolone 60 mg daily. One week later the serum level of creatine kinase was normalized and the patient was weaned from ventilator support. The dose of prednisolone was tapered 10 mg every week and she was discharged two months after delivery without prednisolone. A chest CT scan revealed no interstitial pneumonitis or aspiration pneumonia, so her respiratory failure seemed to be ascribable to polymyositis-related respiratory muscle weakness and pleural effusion. However, severe involvement of respiratory muscles without generalized marked muscle weakness is extremely rare, and pleural complications have usually been described in association with pulmonary parenchymal diseases. During the following two years polymyositis has not recurred in this patient and there have been no data indicative of other overlapping collagen diseases. Reports of polymyositis which occurred during pregnancy are rare, and the pathogenesis and clinical outcome of these patients need to be carefully investigated.

Acute Disease↗

PubMed2000

Skeletal muscle pathology in 2 siblings infected with Toxoplasma gondii.

Skeletal muscle can be the site of inflammatory diseases that lead to muscle weakness, pain, and increased myogenic serum enzymes. Most of these inflammatory myopathies are idiopathic. In some cases inflammatory myopathies are due to infectious agents. We describe the pathological aspects of muscle biopsies of 2 Brazilian siblings who acquired toxoplasmosis at the same time and in similar conditions. One developed a tetraplegia that was confirmed to be due to inflammatory myositis due to toxoplasma. The other developed myocarditis, with heart failure, without skeletal muscle weakness. In both cases many toxoplasma organisms were observed in the muscle biopsies, but in case 1 only was there an inflammatory myopathy with myofiber necrosis; the inflammatory cells were predominantly macrophages with some CD4+ cells and rare CD20+ cells. In case 1, expression of CD54 was observed in many inflammatory cells as well in endothelial cells, but only in endothelial cells in case 2. After treatment with clindamycin and corticosteroids both cases had only partial improvement, case 1 with a residual muscle weakness and case 2 with residual cardiac insufficiency (requiring digoxin). These cases show that the presence of the parasite in myofibers is not enough to induce an inflammatory myositis with muscle cell necrosis. This suggests that immunological disturbances may contribute to the development of inflammatory myositis due to toxoplasma.

Adolescent↗

PubMed2001

[A patient with vitamin E deficient, myopathy presenting with amyotrophy].

We report a 61-year-old man with vitamin E deficiency, presenting with, myopathy as an only clinical symptom. In 1997, at 59 years of age, he noted mild proxymal-muscle weakness and atrophy in the four extremities, nine years after he received a Billroth II partial gastrectomy for a gastric ulcer. His muscle weakness slowly exacerbated, and he was admitted to our hospital in 1999. On admission, neurological examination confirmed mild proximal-muscle weakness and atrophy in the four extremities. Intelligence, cranial nerves, coordination, sensation and tendon reflexes were all normal. Laboratory examination showed normochromic anemia (Hb 9.9 g/dl, Ht 30.9%, MCV 97.5 fl, MCHC 31.2 pg), hypoproteinemia (5.0 g/dl), and hypocholesterolemia (107 mg/dl). The levels of serum CK, lactate and pyruvate were normal. The serum vitamin E level was markedly reduced (0.17 mg/dl; normal 0.75-1.41). Cerebrospinal fluid was normal. Nerve conduction, sensory evoked potentials (SEP), electromyography (EMG), head CT and electroencephalography (EEG) were all normal. Muscle biopsy from the right deltoid muscle showed both mild myogenic and neurogenic changes. Remarkably, type 1 muscle fiber predominance and granular accumulation of autofluorescent lipofuscin granules in the muscle fibers were found. These pathological findings were compatible with those of vitamin E-deficient myopathy. Thus, he was diagnosed as having vitamin E-deficient myopathy, which was confirmed by apparent effective supplementation of vitamin E. Interestingly, our present case did not show any other neurological manifestations such as deep sensory disturbance, sensory ataxia or polyneuropathy. A long-term workload due to hard physical labor and smoking in our patient may have accelerated oxidative muscle damage, resulting in amyotrophy mainly due to vitamin E deficient myopathy.

Gastrectomy↗

PubMed2002

[Neuromuscular monitoring during anesthesia.].

OBJECTIVE: Muscle relaxants are very important in anesthetic practice but must be used with great care. Studies have shown that 17-40% of patients in postanesthesia care units (PACU) have residual muscle weakness. The purpose of this study was to evaluate whether the use of neuromuscular monitors during anesthesia could reduce the incidence of muscle weakness in the postoperative period. MATERIALS AND METHOD: Eighty patients operated for laparoscopic cholecystectomy or lumbal disc prolapse given muscle relaxants during anesthesia were studied, randomly allocated to four groups. Fourty of these patients were monitored with neuromuscular monitor (TOF-guard") during anesthesia and the set point was a TOF-ratio of at least 70% before extubating the patients. Fourty patients were monitored by usual clinical signs (spontaneus breathing, cough and muscle movement). Twenty patients in each group were given vecuronium as muscle relaxant and 20 patients recieved pancuronium, again patients were randomly selected. In the PACU all patients were evaluated and the "5-sec headlift test" was used to find patients with muscle weakness. Hand grip strength was also measured before anesthesia and in the PACU. Glascow Coma Score (GCS) was used to evaluate if patients were too drowsy to co-operate and patients with GCS < 12 were excluded. Measurements were made after arrival to the PACU and every 30 minutes thereafter until headlift was at least 5 sec. RESULTS: The incidence of restcurarization was 15% on arrival to the PACU. No statistically significant difference was found between those monitored with neuromuscular monitors and those that were not. Similarily no statistical difference was found between short acting neuromuscular blocking agents and longer acting agents. CONCLUSION: Although we didn t find any benefit from neuromuscular monitoring or using shorter acting drugs, the use of nervestimulators and short acting drugs is still recommended, especially for high risk patients. The generally accepted train-of-four (TOF-) ratio of 70% has been questioned by some authors, recommending a higher ratio (85%). Further studies using a higher TOF-ratio are therefore recommended.

English Abstract↗

PubMed2005

An alphaTropomyosin mutation alters dimer preference in nemaline myopathy.

Nemaline myopathy is a human neuromuscular disorder associated with muscle weakness, Z-line accumulations (rods), and myofibrillar disorganization. Disease-causing mutations have been identified in genes encoding muscle thin filament proteins: actin, nebulin, slow troponin T, betaTropomyosin, and alphaTropomyosin(slow). Skeletal muscle expresses three tropomyosin (Tm) isoforms from separate genes: alphaTm(fast)(alphaTm, TPM1), betaTm (TPM2), and alphaTm(slow) (gammaTm, TPM3). In this article, we show that the level of betaTm, but not alphaTm(fast) protein, is reduced in human patients with mutations in alphaTm(slow) and in a transgenic mouse model of alphaTm(slow)(Met9Arg) nemaline myopathy. A postnatal time course of Tm expression in muscles of the mice indicated that the onset of alphaTm(slow)(Met9Arg) expression coincides with the decline of betaTm. Reduction of betaTm levels is independent of the degree of pathology (rods) within a muscle and is detected before the onset of muscle weakness. Thus, reduction in the level of betaTm represents an early clinical diagnostic marker for alphaTm(slow)-based mutations. Examinations of tropomyosin dimer formation using either recombinant proteins or sarcomeric extracts show that the mutation reduces the formation of the preferred alpha/beta heterodimer. We suggest this perturbation of tropomyosin isoform levels and dimer preference alters sarcomeric thin filament dynamics and contributes to muscle weakness in nemaline myopathy.

Animals↗

PubMed1995

[Non-Fukuyama type merosin-positive congenital muscular dystrophy with delayed muscle fiber type differentiation: a case report].

A patient with non-Fukuyama type merosin-positive congenital muscular dystrophy (nonFCMD) who had severe muscle weakness leading to early death was reported. He was the first product of epileptic mother who had been placed on phenobarbital and phenytoin. The patient had severe respiratory failure and muscle weakness at the neonatal period, and died at 4 months of age. Multiple joint contractures were also noted at birth. Serum creatine kinase was within normal limits (123 IU/l). Electromyography showed a myogenic pattern. Brain computed tomographic (CT) scan and magnetic resonance imaging (MRI) were normal without white matter lucency or pachygyria. Muscle biopsy revealed dystrophic changes and type 2C fiber predominance. Dystrophin, dystrophin-associated glycoproteins and merosin were all positively demonstrated. Although patients with merosin-positive nonFCMD have relatively mild clinical course, our patient had severe muscle weakness with fatal outcome. Defect in muscle fiber maturation and differentiation, such as an increase of undifferentiated type 2C fibers, may be a major factor to influence muscle symptoms in non FCMD.

Humans↗

PubMed2000

Mitochondrial cytopathy combined with Fanconi's syndrome.

Severe muscle weakness in Fanconi's syndrome is rarely the result of mitochondrial cytopathy. We describe a rare case of a 9-year-old male with early onset of Fanconi's syndrome. He developed severe proximal muscle weakness exacerbated by hypokalemia and hypophosphatemia in childhood. The muscle biopsy revealed increased accumulation of abnormal mitochondria and fat droplets in histochemical stains and electron microscopy. Mitochondrial cytopathy cannot be excluded in Fanconi's syndrome with late onset of muscular impairment. Long-term follow-up of his clinical course is suggested to understand the natural history of this unusual case.

Acidosis, Renal Tubular↗

PubMed1991

Muscular dystrophy with separate clinical phenotypes in a large family.

This report describes a large consanguineous family with muscular dystrophy in 23 patients showing intrafamilial variation of clinical expression. One main variant appeared in the first decade with proximal muscle weakness progressing over the next 20 years to wheelchair confinement, and appeared compatible with classical limb-girdle muscular dystrophy. The other main variant showed onset of distal muscle weakness in lower limbs in the third or fourth decade, progressing very slowly without greater disability throughout the lifetime. Tibial muscle weakness and wasting were clinical landmarks in this variant, but computed tomography of skeletal muscle revealed focal areas of fatty degeneration also in truncal, pelvifemoral, and distal leg muscles in a way not previously reported in distal myopathy. The overall difference in clinical findings between these main variants would suggest 2 separate genetic entities, genealogical data makes a common genetic background possible.

Adult↗

PubMedDate not supplied

Hypothyroidism presenting as a polymyositis-like syndrome. Report of two cases.

Two patients with proximal muscle weakness and marked elevations of serum muscle enzymes were initially believed to have polymyositis; however electromyography and muscle biopsies were normal. Both patients were subsequently found to have hypothyroidism. Each regained her muscle strength, and serum enzymes normalized with thyroid hormone replacement. Because muscle weakness and an elevated creatine phosphokinase occur in most patients with hypothyroidism, thyroid dysfunction must be considered in the differential diagnosis of polymyositis.

Adult↗