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Maximal sniff mouth pressure compared with maximal inspiratory pressure in acute respiratory failure.

Inspiratory muscle strength most often is better reflected by sniff Pes than PImax against occlusion. Furthermore, sniff Pes can be estimated noninvasively by the measurement of sniff Pmo in normal subjects and in patients with respiratory muscle weakness. The aim of this study was to compare sniff Pmo and P.PImax to assess inspiratory muscle strength in patients with acute respiratory failure. The highest pressure was produced by P.PImax in 61 percent of measurements, and by sniff Pmo in 39 percent. Above 35 cm H2O P.PImax yielded the highest pressure in 55 percent of cases and the ratio sniff Pmo/P.PImax was 1.20 +/- 0.54. Below 35 cm H2O, P.PImax yielded the highest pressure in 75 percent of cases and the ratio sniff Pmo/P.PImax was 0.76 +/- 0.35 (p less than 0.02). Thus, measurements of sniff Pmo and P.PImax complement one another for assessing inspiratory muscle strength. However, sniff Pmo underestimates inspiratory muscle strength in patients with severe inspiratory muscle weakness.

Acute Disease↗

Metabolic alkalosis with paradoxic aciduria in cattle.

In 4 cases of metabolic alkalosis with paradoxic aciduria in cattle, generalized muscle weakness was the predominant feature. In 3 of the 4 cases, prolonged anorexia preceded development of muscle weakness and aciduria. Plasma electrolyte concentrations varied but, in 3 of 4 cases, a transient decrease in plasma potassium concentration was detected. In the 3 cases in which prolonged therapy with sodium chloride, potassium chloride, and dextrose was offered, resolution of aciduria was rapid, followed by correction of metabolic alkalosis and return of muscle strength.

Acid-Base Imbalance↗

The Boston FICSIT study: the effects of resistance training and nutritional supplementation on physical frailty in the oldest old.

Research indicates that lower extremity muscle weakness in the elderly is consistently related to impaired mobility and fall risk. Reversible components of the muscle weakness of aging include underuse syndromes and undernutrition, both of which are prevalent in nursing home populations. The Boston FICSIT study is a nursing home-based intervention to improve muscle strength through progressive resistance training of the lower extremities and/or multi-nutrient supplementation in chronically institutionalized subjects aged 70-100. Baseline measurements of falls, medical status, psychological variables, functional status, nutritional intake and status, body composition, muscle mass and morphology, muscle function, and gait and balance are taken. The nursing home residents are then randomly assigned to one of four treatment groups for 10 weeks: (1) high intensity progressive resistance training of the hip and knee extensors 3 days per week; (2) multi-nutrient supplementation with a 360-kcal high carbohydrate, low fat liquid supplement every day; (3) a combination of groups (1) and (2); and (4) a control group. Both non-supplemented groups receive a liquid placebo every day, and both non-exercising groups attend three sessions of "leisure activities" every week in order to control for the attentional aspects of the exercise and nutritional interventions. At the end of the 10-week period, all baseline measurements are re-assessed.

Aged↗

Postanesthesia care unit recovery times and neuromuscular blocking drugs: a prospective study of orthopedic surgical patients randomized to receive pancuronium or rocuronium.

UNLABELLED: In this study, we examined the effect of choice of neuromuscular blocking drug (NMBD) (pancuronium versus rocuronium) on postoperative recovery times and associated adverse outcomes in patients undergoing orthopedic surgical procedures. Seventy patients were randomly allocated to a pancuronium or rocuronium group. On arrival to the postanesthesia care unit (PACU) and again 30 min later, train-of-four ratios were quantified by using acceleromyography. Immediately after acceleromyographic measurements, patients were assessed for signs and symptoms of residual paresis. During the PACU admission, episodes of hypoxemia, nausea, and vomiting were recorded. The time required for patients to meet discharge criteria and the time of actual PACU discharge were noted. Forty percent of patients in the pancuronium group had train-of-four ratios <0.7 on arrival to the PACU, compared with only 5.9% of subjects in the rocuronium group (P < 0.001). Patients in the pancuronium group were more likely to experience symptoms of muscle weakness (blurry vision and generalized weakness; P < 0.001) and hypoxemia (10 patients in the rocuronium group versus 21 patients in the pancuronium group; P = 0.015) during the PACU admission. Significant delays in meeting PACU discharge criteria (50 min [45-60 min] versus 30 min [25-40 min]) and achieving actual discharge (70 min [60-90 min] versus 57.5 min [45-61 min]) were observed when the pancuronium group was compared with the rocuronium group (P < 0.001). In conclusion, our study indicates that PACU recovery times may be prolonged when long-acting NMBDs are used in surgical patients. IMPLICATIONS: Clinical recovery may be delayed in surgical patients administered long-acting neuromuscular blocking drugs. During the postanesthesia care unit admission, patients randomized to receive pancuronium (versus rocuronium) were more likely to exhibit symptoms of muscle weakness, develop hypoxemia, and require more time to meet discharge criteria.

Adult↗

Oculopharyngeal muscular dystrophy in Hispanic New Mexicans.

CONTEXT: Oculopharyngeal muscular dystrophy (OPMD) is a rare myopathy caused by polyalanine triplet repeat expansion in the gene for poly(A) binding protein 2 (PABP2) and is found in isolated cohorts throughout the world. We have observed numerous cases of OPMD in New Mexico. OBJECTIVE: To characterize the clinical, genetic, and demographic features of the OPMD population in New Mexico. DESIGN, SETTING, AND PARTICIPANTS: Cohort study with analysis of outpatient clinic medical records from 1965 to 2001 at the University of New Mexico Hospital and the New Mexico VA Health Care System in Albuquerque, which serve the entire state. MAIN OUTCOME MEASURES: Clinical phenotype, supplemented with genetic confirmation (n = 10 patients) and in-depth clinical evaluations (n = 49 patients). RESULTS: We identified 216 cases of OPMD (99 women and 117 men) from 39 kindreds of New Mexicans spanning up to 4 generations. All patients were Hispanic, and the majority of probands came from northern New Mexico. In patients who had both ocular and pharyngeal muscle weakness, ptosis was just as likely to occur before or concurrent with dysphagia. Proximal limb muscle weakness and gait abnormalities were common and occurred later than ocular or pharyngeal weakness. The clinical expression of OPMD caused marked debility, although life-table analysis showed no decrease in life expectancy compared with unaffected family members (P =.81). Ten individuals from different kindreds were found to have an identical polyalanine triplet repeat expansion ([GCG](9)) in the PABP2 gene. CONCLUSIONS: Individuals in this cohort had clinical and genetic characteristics of classic OPMD. Longevity was not affected, but patients experienced considerable morbidity. The origin of the PABP2 mutation in New Mexican OPMD patients is unclear, although the geographic and genetic isolation of northern New Mexicans with a long ancestry in this region may have contributed to the development of this cohort. This disease cohort represents a large and previously unrecognized health care issue in the state of New Mexico and should serve to raise the awareness of this disorder among clinicians who treat Hispanics in the Southwest and throughout the United States.

Adult↗

Mitochondrial abnormalities in selenium-deficient myopathy.

We report a man who developed selenium-deficient myopathy during long-term parenteral nutrition. Muscle biopsy showed marked mitochondrial depletion in the deep sarcoplasm and enlarged mitochondria at the periphery mainly in type 2 fibers. Muscle weakness improved gradually after the second course of selenium supplementation. The peculiar mitochondrial abnormalities in muscle fibers appear to play a key role in the pathogenesis of selenium-deficient myopathy.

Adult↗

Dermatomyositis.

Dermatomyositis is one of the idiopathic inflammatory myopathies. It is characterized clinically by progressive symmetrical proximal muscle weakness and a characteristic rash. There are patients with rash who have little or no muscle disease. Although the process primarily attacks the skin and the muscles, it is a systemic disease with frequent manifestations in the gastrointestinal tract and pulmonary system. Dermatomyositis has been linked to internal malignancy in somewhere between 15% and 25%. Therapy for the muscle disease includes systemic corticosteroids with or without an immunosuppressive agent. Therapy of the skin disease begins with photoprotection and topical corticosteroids, but also includes the use of antimalarial agents and immunomodulatory therapies.

Adrenal Cortex Hormones↗

Sporadic amyotrophic lateral sclerosis of long duration mimicking spinal progressive muscular atrophy exists: additional autopsy case with a clinical course of 19 years.

This report concerns an autopsy case of sporadic amyotrophic lateral sclerosis (ALS) clinically diagnosed as having spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without hereditary burden. She developed muscle weakness in the distal part of the right upper extremity at age 52, followed by muscle weakness in the left upper extremity and lower extremities at age 54 and 64, respectively. At age 66 she could not walk, even with assistance. Fasciculation and atrophy of the tongue appeared at age 68, followed by dysphagia and dysarthria at age 70. She died of respiratory disturbance at age 71. During the clinical course, neurological examination revealed neither Babinski sign nor hyperreflexia. No respirator administration was performed throughout the clinical course. Neuropathological examination disclosed not only neuronal loss with gliosis in the hypoglossal nucleus and anterior horns of the spinal cord, but also loss of Betz cells and degeneration of the pyramidal tract. Based on these clinicopathological findings and a literature review of sporadic autopsy cases of ALS with long clinical course (10 years or more), including four cases without pyramidal signs, we believe that sporadic ALS of long clinical course mimicking SPMA exists.

Aged↗

Multi-minicore disease: a report of 5 patients from Kuwait.

OBJECTIVE: To describe the characteristic clinical features and the diagnostic findings in muscle histochemistry of multi-minicore disease in 5 children from two unrelated families in Kuwait. CLINICAL PRESENTATION: The 5 children who presented with muscle weakness, 2 siblings from family 1 and the remaining 3 from the other (family 2), represent the classical type of multi-minicore disease; however, the two families differ in the course of the disease. Family 1 had the non-progressive form while family 2 had progressive weakness with respiratory complications and scoliosis. The diagnosis was confirmed in the index patients by muscle histochemistry, which demonstrated the typical minicores, which are devoid of oxidative enzyme activity. CONCLUSION: Both families represent the classical form, however, clinical variability in the course of the illness was demonstrated in these two families.

Child↗

[A patient with neuroborreliosis presenting gadolinium-enhanced MRI lesions in bilateral facial nerves].

We report a 33-year-old man with bilateral facial paralysis due to neuroborreliosis. About three weeks after rhinorrhea and fever lasting four days, he noticed fatigue in the legs and paresthesia in all four extremities. Another week later, he developed paresthesia in his tongue and bilateral facial muscle weakness, and was admitted to our hospital. On admission, neurological examination revealed moderate bilateral facial muscle weakness, mild paresthesia in the tongue and four extremities, and decreased Achilles tendon reflex bilaterally. Mild pleocytosis and increased protein were found in the cerebrospinal fluid (CSF). IgM antibodies that reacted with the antigens of Borrelia garinii and Borrelia afzelii were found in his serum. Clinically and serologically, he was thus diagnosed as having neuroborreliosis. Brain MRI revealed gadolinium-enhanced lesions of the bilateral facial nerves in the facial nerve canal portion. After three weeks of treatment with 100 mg/day doxycycline and 2 g/day ceftriaxone sodium, his symptoms and CSF abnormalities were rapidly improved. Although facial nerve paralysis is a major symptom of neuroborreliosis, the present report is the first to detect the inflammatory lesions of the facial nerves in the facial nerve canal portion by MRI.

Adult↗

[Case report of sodium valproate treatment of aggression associated with Alzheimer's disease].

Aggressive behaviors represent a major management problem in patients with dementia. Neuroleptics are commonly the selected psychotherapeutics for controlling severe aggression, especially the violent outbursts often seen in severely demented patients. But their efficacy for this problem is limited and their use is frequently complicated by side effects. Anticonvulsants such as carbamazepine have been advocated as alternatives. Although sodium valproate can also be a potential alternative, only a few cases were reported. A 69-year-old man was admitted for the control of agitation, wandering and violence. He had an 8-year history of amnesia. The mental status examination revealed severe global cognitive loss. Cranial computed tomography showed moderate brain atrophy and dilatation of bilateral temporal ventricles. A diagnosis of Alzheimer's disease was made. Initially he was treated with nuroleptics. Sulpride in doses up to 600 mg/day had no positive effect, but led to confusion and worsened aggressive behavior. Haloperidol in doses up to 4 mg/day or propericiazine 40 mg/day showed the same result as sulpiride. Carbamazepine had a moderate ameliorative effect on the agitation and violence, but the patient could not tolerate it because of muscle weakness. Then, we used sodium valproate 400mg/day and found this was effective. After one week muscle weakness appeared. Therefore, the dose was decreased to 200mg/day, achieving a blood level of 20.7 micrograms/ml. The patient's agitation was well controlled at that point and had no apparent side effects. Sodium valproate may be a useful agent in the treatment of aggression in patients with dementia.

Aged↗

Follow-up study on F-wave in patients with lumbosacral radiculopathy. Comparison between before and after surgery.

The aim of this study is to explore the clinical value of follow-up examinations of F-wave in patients with lumbosacral radiculopathy. Postoperative changes in the initial abnormalities and their relationship with improvement in clinical findings were examined. Results from conventional motor nerve conduction study at knee-ankle segment are also discussed. The subjects were 30 patients with unilateral lumbosacral radiculopathy caused by herniated disc. F-waves were obtained from abductor hallucis and extensor digitorum brevis by supramaximal stimulation to tibial and peroneal nerves, respectively, at the ankle. Minimal latency (Fmin), maximal latency (Fmax), chronodispersion (Fdif) and duration (Fdur) were used as the parameters. No subject had abnormal motor nerve conduction velocity (MCV) throughout the course of this study, and there was no significant difference between preoperative and postoperative levels. Peroneal Fmin, Fmax, Fdif and tibial Fdur all showed significant (p < 0.05 in Wicoxon signed-rank test) improvement after surgery. Moreover, these overall improvements significantly (p < 0.05 in chi 2 test) correlated with recovery in muscle weakness. However, normalization of F-wave was not always associated with full recovery in muscle weakness at that time. These findings suggested that follow-up examination of F-wave was of value for objective assessment after operation.

Adult↗

Trichinosis with severe myopathic involvement mimicking polymyositis. Report of a family outbreak.

We describe an outbreak of trichinosis in 3 members of a rural family. In the 3 patients eating raw pork was the source of infection. They presented with myalgias and severe proximal muscle weakness mimicking polymyositis. The diagnosis was made by demonstration of larvae of Trichinella spiralis in the muscle biopsy and also by the presence of anti-Trichinella antibodies detected by double immunodiffusion in their sera. We call attention to the unusual clinical presentation of trichinosis in our patients that was manifested by severe muscle weakness that may be confused with polymyositis.

Adult↗

Lifestyle and late effects after poliomyelitis. A risk factor study of two populations.

BACKGROUND: Patients with polio often experience new symptoms (muscle weakness, pain, fatigue and respiratory problems) many years after the acute disease. This study examined possible interactions between lifestyle factors (overweight, physical inactivity, smoking) and late polio with new symptoms. METHODS: A total of 148 patients hospitalized for acute polio in 1950-1954 at Haukeland University Hospital, Norway and 128 patients, hospitalized for acute polio in 1958 at Tartu University Hospital, Estonia responded to a mailed questionnaire regarding lifestyle and late polio with new symptoms. Multiple regression analysis, two samples t-test and chi-square analysis were undertaken. RESULTS: Mean body mass index (BMI) and percentage of smokers did not differ in the two cohorts, while polio patients were physically less active in Estonia. The physically active patients in both cohorts had significantly lower odds for experiencing polio-related late muscle pain (OR = 0.21; 95% CI = 0.08-0.55) and fatigue (OR = 0.32; 95% CI = 0.14-0.75). With increasing age the patients had significantly higher odds for experiencing new muscle weakness (OR = 1.03; 95% CI = 1.00-1.07), fatigue (OR = 1.04;95% CI = 1.01-1.07) and breath shortness (OR = 1.04; 95% CI = 1.00-1.07). CONCLUSION: Physically inactive patients are at a higher risk for late polio-related symptoms. An active lifestyle should be recommended for patients with polio sequels.

Adult↗

Treatment of acute post-infective polyneuropathy by means of plasma exchange.

15 patients with acute post-infective polyneuropathy of the Guillain-Barré type have been treated by means of plasma exchange. The clinical course and outcome of these patients is compared to that in a retrospectively matched control series who were treated with supportive therapy only. All patients had severe rapidly evolving muscle weakness and approximately half the patients in each group required ventilatory assistance. Both the duration of muscle weakness and the hospitalisation time was significantly shorter in the patients treated by means of plasma exchange. These results suggest that plasma exchange is of significant benefit in the treatment of patients with the Guillain-Barré syndrome.

Adolescent↗

Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy.

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease that presents in the fifth or sixth decade with dysphagia, ptosis and proximal limb weakness. OPMD is caused by the abnormal expansion of a polyalanine tract within the coding region of polyA binding protein nuclear 1 (PABPN1). The resultant mutant PABPN1 forms aggregates within the nuclei of skeletal muscle fibres. We have previously described a transgenic mouse model of OPMD that recapitulates the human disease and develops progressive muscle weakness accompanied by the formation of aggregates in skeletal muscle nuclei. The chemical chaperone trehalose has been used effectively to alleviate symptoms in a mouse model of Huntington's disease and is thought to elicit its effect by binding and stabilizing partially folded polyglutamine proteins and inhibiting the formation of aggregates. Here, we show that trehalose reduces aggregate formation and toxicity of mutant PABPN1 in cell models. Furthermore, oral administration of trehalose attenuated muscle weakness, reduced aggregate formation and decreased the number of TUNEL-labelled nuclei in skeletal muscle in an OPMD transgenic mouse model. Thus, anti-aggregation therapy may prove effective in the treatment of human OPMD.

Analysis of Variance↗

[Assessment of function recovery in patients with total knee prosthesis].

The aim of this study was to analyse the evolution of motor performance in a group of patients who underwent surgical knee replacement. We also assessed patient satisfaction at 3 and 9 months after the operation. Sixty-two patients (40 women, 22 men, mean age 72.4 years) underwent isokinetic evaluation associated with surface EMG. All the patients had undergone a total prosthetic knee joint replacement for arthrosis, and had followed a standardised 30-day rehabilitation program at our centre for recovery of knee joint function, strengthening of the flexor-extensor muscles and restoration of ambulation as best as possible. Each of these patients underwent isokinetic evaluations 3 months and 9-10 months after their operation. The isokinetic test consisted of carrying out 5 flexion-extensions of the knee at an angular velocity of 60 degrees/second, followed by an endurance test of 30 repetitions of flexion-extension of the knee at 120 degrees/second. This isokinetic test provided data on extensor strength and flexor strength; furthermore, specifically designed software allowed simultaneous visualisation of the surface EMG tracing, torque and joint excursion. The first analysis showed a macroscopic decrease in the strength of the flexor-extensor muscles of the knee. This muscle weakness was clearly evident 3 months after the operation and was particularly pronounced for the extensor muscles of the operated limb compared with the muscles of contralateral, unoperated limb. Muscle weakness was still present 9 months after the operation although there had been a considerable improvement compared with 6 months previously; the imbalance in the flexor/extensor ratio, which differed from that in the contralateral, unoperated limb, was noted to be still present. The surface EMG demonstrated persistent myoelectrical activity in the flexors even when the extensor activity was predominant: this is an expression of imbalance between agonists-antagonists. A questionnaire administered to the patients about their satisfaction with the operation revealed that 9 months after the surgery 23 patients still complained of continuous joint pain with increased loads, 27 complained of frequent pain with load-bearing and only 12 no longer complained of any disturbance, manifesting full satisfaction with the operation. Total knee replacement is thus a valid treatment in those cases in which degenerative joint disease necessitates a radical solution. Nevertheless, dissatisfaction with the operation is common, and may be due to persistence of joint pain and incomplete joint recovery of joint function or muscle strength. In our series of patients, we found that although there was progressive recovery of strength of both the flexor and extensor muscles, a considerable imbalance remained between the operated and unoperated limbs; this finding was also confirmed by the surface EMG investigation.

Aged↗

A comparative freeze-fracture study of plasma membrane of dystrophic skeletal muscles in dy/dy mice with merosin (laminin 2) deficiency and mdx mice with dystrophin deficiency.

The intramembranous particle (IMP), orthogonal array (OA) and orthogonal array subunit particle (OASP) densities of skeletal muscle plasma membranes of merosin deficient dy/dy mice and their control mice at 7, 14 and 28 days after birth were analysed by freeze-fracture electron microscopy. Similar studies were performed on dystrophin-deficient mdx mice with mild muscle weakness at 28 days after birth for the comparison with those of dy/dy mice with severe muscle weakness at the same age. In the pre-clinical stage of dy/dy mice at 14 days after birth, the membranes showed a significantly decreased density of OAs (P<0.01 by Wilcoxon rank-sum test) as compared with control mice, while those in the clinical stage of dy/dy mice at 28 days after birth showed normal IMP density but a marked depletion of OA density (P<0.01). Moreover, at 28 days after birth, the reduction of OAs in the plasma membranes of dy/dy mice was more marked than that of mdx mice (P<0.05 by Wilcoxon rank-sum test). These results provided us with the information that the OA density was affected more severely with merosin deficiency than with dystrophin deficiency, and again supported our previously proposed concept that the clinical severity in muscular dystrophies correlated with the OA density.

Animals↗