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Effects of a carrageenan-induced myositis on the discharge properties of group III and IV muscle receptors in the cat.

1. To see how muscle group III and IV receptors are affected by a myositis, the background activity and mechanical excitability of slowly conducting afferent units from normal and inflamed muscles were studied in chloralose-anesthetized cats. The inflammation was induced by infiltrating the gastrocnemius-soleus muscle with a suspension of 2% carrageenan. According to their responsiveness to local pressure stimulation the receptors were classified as touch units, moderate pressure units, and noxious pressure (probably nociceptive) units. The impulse activity in single afferent units was recorded up to 14 h after induction of the inflammation. 2. In inflamed muscle both group III and group IV receptors showed an increase in the proportion of units having a background activity and in the mean background activity. The differences reached statistically significant levels in group III fibers only. 3. A characteristic feature of the background activity of some receptors in inflamed muscle was its intermittent nature: the discharges occurred either as grouped impulses of short duration or as phases of relatively high discharge frequency alternating with long periods of silence. 4. In normal muscle no receptor exhibited intermittent discharges or had a discharge rate exceeding 7 imp/min. Thus the presence of an intermittent background activity or a high frequency of the background discharge can be considered as characteristic for afferent units from an inflamed muscle. 5. The time course of the background activity showed two peaks, one occurring 2-4 h, the other one 6-7 h after induction of the inflammation. Recordings of single units during the transition from the normal to the inflamed state demonstrated that the first increase in background discharge took place 1-1.5 h after injection of carrageenan. 6. The proportion of noxious pressure units was reduced and that of moderate pressure units increased in inflamed muscle. In this case the difference was significant for group IV units only. 7. The response curves upon mechanical stimulation did not show significant differences in normal and inflamed muscle, i.e., sensitized noxious pressure units behaved similar to real touch units or moderate pressure units with respect to their mechanical excitability. 8. No apparent correlation existed between the receptors' mechanical threshold and degree of background activity in inflamed muscle. This suggests that the inflammation-induced change in mechanical excitability and development of background activity are independent phenomena.(ABSTRACT TRUNCATED AT 400 WORDS)

Action Potentials↗

Injection of botulinum toxin A to the upper esophageal sphincter for oropharyngeal dysphagia in two patients with inclusion body myositis.

Inclusion body myositis (IBM) is a progressive degenerative skeletal muscle disease leading to weakening and atrophy of both proximal and distal muscles. Dysphagia is reported in up to 86% of IBM patients. Surgical cricopharyngeal myotomy may be effective for cricopharyngeal dysphagia and there is one published report that botulinum toxin A, injected into the cricopharyngeus muscle using a hypopharyngoscope under general anesthesia, relieved IBM-associated dysphagia. This report presents the first documentation of botulinum toxin A injection into the upper esophageal sphincter using a flexible esophagogastroduodenoscope under conscious sedation, to reduce upper esophageal sphincter pressure and successfully alleviate oropharyngeal dysphagia in two IBM patients.

Aged↗

[Progressive myositis ossificans. Utility of bone scintigraphy].

47 year old woman affected by myositis ossificans, with muscular calcifications in her evolution. Bone scintigraphy showed increased polyfocal activity as well as uptake of different intensity in soft tissues. The bone scintigraphy allows for early identification of ossification areas in soft tissues, as it can even detect injuries earlier than that observed with other image techniques. It is considered to be very useful in the diagnosis and establishment of the extension of the process, as well as follow-up.

Ankylosis↗

Inflammatory myositis: a rare differential diagnosis of a neck lump.

Inflammatory myositis is an extremely rare benign inflammatory condition of skeletal muscle. It usually affects the extremities, and there are only 10 cases reported in the head and neck region. Its significance is that in this region it can be clinically mistaken for a neoplasm. We describe a case and review the literature.

Adult↗

Apolipoprotein E and alpha-1-antichymotrypsin polymorphisms in sporadic inclusion body myositis.

Sporadic inclusion body myositis (s-IBM) is a progressive muscle disease of unknown aetiology. Characteristically, intracellular amyloid deposits are detectable, including beta-amyloid precursor protein, phosphorylated tau, alpha1-antichymotrypsin (alpha1-ACT) and apolipoprotein E (ApoE). Polymorphisms and mutations of the encoding genes have been identified in a variety of neurodegenerative diseases including Alzheimer's disease (AD). Beside other factors, polymorphisms may lead to protein accumulation in both diseases. In particular, polymorphisms within the ApoE and alpha1-ACT gene have been implicated in the aetiology of AD and s-IBM. We analysed ApoE and alpha1-ACT gene polymorphisms in 35 s-IBM patients. We could not identify any statistical significant correlation between distinct ApoE and alpha1-ACT genotypes and the risk of developing s-IBM. Additionally, ApoE and alpha1-ACT genotypes seem not to influence the onset age of s-IBM. A combination of different alpha1-ACT and ApoE genotypes appears not to enhance the risk of developing s-IBM. Therefore, allelic variations of alpha1-ACT and ApoE are unlikely to be genetic key factors in the aetiology of s-IBM.

Adolescent↗

Parasitic myositis by Echinococcus alveolaris. Report of a family with myotonia congenita.

Echinococcus alveolaris infections were found in 5 members of a family and at least 3 of them also suffered from myotonia congenita. A muslce biopsy, performed in 1 case because of a pathological EMG finding allowed a diagnosis of parastic myositis caused by E. alveolaris to be made. In a further case, X-rays demonstrated calcification of an Echinococcus cyst in the thigh. Since is it only on rare occasions that these parasites are found in muscle tissue, factors are then discussed which might in some way be responsible for this high rate of E. alveolaris lesions in the muscles of patients with myotonia congenita.

Adolescent↗

Selenium responsive myositis during prolonged home total parenteral nutrition in cystic fibrosis.

The need for routine supplementation of total parenteral nutrition solutions with selenium (Se) has not been clearly defined. Although clinical selenium deficiency in patients on prolonged total parenteral nutrition has been reported, it is rarely observed in the United States. We report a 19-year-old woman with cystic fibrosis who developed muscle pain and weakness after 3 months on total parenteral nutrition which was not supplemented with Se. Coincident with her onset of symptoms, markedly elevated serum creatine kinase values were observed compared to baseline levels. Subsequent evaluations revealed undetectable (less than 0.02 microgram/ml) serum and urine Se levels in this patient. In addition, electromyographic evidence of myositis and nonspecific membrane irritability was documented. Therapy with oral Se rapidly reversed her symptoms and normalized with serum creatine kinase values over a 10-day period. Prolonged treatment with Se was required to achieve normal values of Se in the serum. Patients with severe pancreatic insufficiency, such as cystic fibrosis, may be at risk for clinical Se deficiency if on prolonged total parenteral nutrition without supplementation. Elevated creatine kinase levels should alert physicians to the possibility of Se deficiency in such patients.

Adult↗

Treatment of myositis ossificans traumatica in athletes.

Myositis ossificans traumatica, although it is one of a number of heterotopic bone formation entities, is a specific lesion with a clearly recognized etiology and natural evolutionary pattern. Early conservative treatment with rest, elevation, and immobilization minimizes additional trauma and decreases the likelihood of incapacitating bone formation. Subsequently, active range of motion exercises progressing to resistive exercises usually effect a satisfactory recovery and return to full athletic participation. With this treatment regimen, few athletes will be left with significant functional impairment. However, surgery does have a definite role in the treatment of this condition and is indicated in those athletes who develop a large mass of mature lamellar bone which is painful and is associated with muscle weakness and a significant loss of joint motion. Four patients are presented in which surgery resulted in satisfactory recovery and return to full participation in football.

Adult↗

Myositis of the hip in a professional soccer player. A case report.

Myositis in the hip joint is a rare entity in sportsmen. Repeated trauma or massage can be detrimental. Successful outcome depends upon the recognition of mature, well-organized new bone, and full excision. Best results can be obtained by performing surgery on a biologically sound joint which contains no trace of active or latent inflammation. The joint should be immobilized till the soft tissue trauma has completely healed. Rehabilitation must be in stages.

Adult↗

Unfolding story of inclusion-body myositis and myopathies: role of misfolded proteins, amyloid-beta, cholesterol, and aging.

Sporadic inclusion-body myositis and hereditary inclusion-body myopathies are progressive muscle diseases leading to severe disability. We briefly summarize their clinical pictures and pathologic diagnostic criteria and discuss the latest advances in illuminating their pathogenic mechanism(s). We emphasize how different etiologies might lead to the strikingly similar pathology and possibly similar pathogenic cascade. On the basis of our research, several processes seem to be important in relation to the still speculative pathogenesis, including (a) increased transcription and accumulation of amyloid-beta precursor protein and accumulation of its proteolytic fragment amyloid-beta; (b) abnormal accumulation of components related to lipid metabolism, for example, cholesterol, accumulation of which is possibly owing to its abnormal trafficking; (c) oxidative stress; (d) accumulations of other Alzheimer's disease-related proteins; and (e) a milieu of muscle cellular aging in which these changes occur. We discuss a potentially very important role of unfolded and/or misfolded proteins as a possible mechanism in the formations of the inclusion bodies and other abnormalities.

Aging↗

Inclusion body myositis in spring peepers (Pseudacris crucifer).

In 2000, 2 adult captive spring peepers (Pseudacris crucifer) from the same zoological park were humanely euthanized. Histologically, both frogs had degeneration, atrophy, and necrosis of striated myofibers of the tongue admixed with chronic lymphohistiocytic inflammation. One frog had similar lesions in the skeletal muscles of the body wall. Several degenerate and necrotic myofibers contained single, eosinophilic, intranuclear inclusion bodies. Ultrastructural examination of the inclusions revealed nonenveloped, icosahedral, virus-like particles averaging 20-24 nm in diameter. This is the first reported case of inclusion body myositis in frogs and is believed to be due to parvoviral infection.

Animals↗

Fatal necrotizing fasciitis and myositis in a captive common bottlenose dolphin (Tursiops truncatus) associated with Streptococcus agalactiae.

A common bottlenose dolphin (Tursiops truncatus) was presented for necropsy after acute onset of gastrointestinal signs and cutaneous lesions that rapidly progressed to death. Gross and microscopic findings were characterized by locally extensive severe necrohemorrhagic fasciitis and cellulitis, and severe necrotizing myositis in the head and dorsocranial thorax, with numerous disseminated gram-positive cocci. Streptococcus agalactiae was isolated from the lesions and from visceral organs (liver and lung), and it was identified by standard microbiology techniques. This communication is the first report of necrotizing fasciitis in a marine mammal associated with S. agalactiae.

Animals↗

Myositis ossificans and fibroosseous pseudotumor of digits: a clinicopathological review of 64 cases with emphasis on diagnostic pitfalls.

Myositis ossificans (MO) and fibroosseous pseudotumor of digits (FP) are pseudotumoral mimics of malignancy. A review of 50 cases of MO and 14 cases of FP showed that a malignant diagnosis was suggested by referring pathologists in 23% of MO and 9% of FP. The most common misdiagnosis was osteosarcoma. Awareness of the spectrum of clinicopathological features of MO and FP will help pathologists avoid misdiagnoses. A comparison of the clinicopathological features of MO and FP showed that most features were similar, but FP involved an older age group (p<0.001). MO showed a statistically significant higher tendency to contain fibrinous material (p=0.007), edematous lymphangioma-like areas (p=0.013), and cartilage (p=0.017) and FP to contain excessive immature osteoid (p=0.029). These differences may be related to the site of occurrence.

Adolescent↗

Intraabdominal myositis ossificans: a report of 9 new cases.

Intraabdominal myositis ossificans (IMO) is a rare benign disorder characterized by reactive bone formation in intraabdominal soft tissue that should be distinguished from a malignant condition. We retrospectively searched our patient records and report 9 new cases of IMO. The lesions occurred in 7 men and 2 women with a mean age of 50 years (range, 24--76 years), 5 of whom had previous abdominal surgery. Histologically, all the cases were similar, consisting of a reactive mesenchymal process in adipose tissue. Mitosis was observed, but with no atypical forms, and the lesions lacked malignant cytologic features. IMO is an uncommon benign lesion that develops relatively rapidly. The pathogenesis is related to intraabdominal surgical procedures, but the exact mechanism remains to be determined.

Adult↗

Calcitriol dosage in osteomalacia, hypoparathyroidism and attempted treatment of myositis ossificans progressiva.

Mineral retention was measured during 39 metabolic balance studies in 34 patients with nutritional osteomalacia or late rickets; they were divided into 5 treatment groups consisting of oral vitamin D, artificial ultra-violet irradiation, 25-hydroxycholecalciferol (calcifediol), 1 alpha-hydroxycholecalciferol (alfacalcidol) and 1 alpha, 25-hydroxycholecalciferol (calcitriol). With the 1 alpha-hydroxylated derivatives, initial dosage of 2 to 6 micrograms daily was required to achieve optimal healing rates by comparison with other responses. Mineral retention was markedly enhanced by supplementation with microcrystalline hydroxyapatite compound (MCHC); untreated X-linked hypophosphataemic rickets healed in 7 weeks on 10 micrograms alfacalcidol daily and 6 grams MCHC daily without developing hypercalcaemia. By contrast, adult-presenting hypophosphataemic osteomalacia developed early hypercalcaemia on the same treatment; additional phosphate supplementation, without changing other treatment, abolished hypercalcaemia and improved calcium retention. A long-term crossover trial of the vitamins D in 6 patients with hypoparathyroidism suggested that relative potencies were as follows (assigning to vitamin D an arbitrary potency of l): vitamin D2 (or D3) l: dihydrotachysterol (DHT) 3: calcifediol 10: alfacalcidol 750: calcitriol 1500. The two-fold superiority of calcitriol over alfacalcidol was evident. Calcifediol and vitamin D controlled plasma calcium at comparable levels of circulating 25-hydroxyvitamin D (25-OH-D), elevated 25-OH-D persisting at least 1 to 2 years after discontinuing long-term (greater than 4 years) vitamin D. In 2 patients with myositis ossificans progressiva treated with 10 to 20 micrograms calcitriol daily, hypercalcaemia was minimized by a low-calcium diet supplemented with cellulose phosphate, suggesting that bone resorption did not play a major role in vitamin D intoxication. Net mineral loss was documented in a young male patient but not in a menopausal female, suggesting that calcitriol treatment was not likely to produce post-menopausal osteoporosis.

Bone Resorption↗

Transthyretin Val122Ile, accumulated Abeta, and inclusion-body myositis aspects in cultured muscle.

Cultured muscle fibers (CMF) from a patient with inclusion-body myositis (IBM) and cardiac amyloidosis associated with the transthyretin (TTR) Val122Ile mutation contained aspects of the IBM phenotype: vacuolation, congophilic inclusions, and clusters of immunocolocalizing amyloid beta-peptide (Abeta) and TTR accumulations. These abnormalities are never present in normal human CMF. These perturbations were greatly increased after Abeta precursor protein gene transfer. The TTR mutation may be a genetic predisposition factor for the patient's IBM.

Aged↗

Anti-T-lymphocyte globulin treatment in inclusion body myositis: a randomized pilot study.

The authors performed an open, randomized trial in patients with inclusion body myositis comparing 1) 12-month treatment with oral methotrexate 7.5 mg/week alone (MTX group) with 2) 12-month MTX treatment preceded by 7 days of IV anti-T-lymphocyte immunoglobulin treatment (ATG group). Eleven patients were randomized; 10 patients completed 12 months follow-up. Myometry showed that patients in the ATG group (n = 6) increased in mean muscle strength by 1.4% compared with the MTX group (n = 5), whose muscle strength decreased by 11.1% (p = 0.021).

Aged↗

MAP kinase phosphatase-1 is induced in abnormal fibers in inclusion body myositis.

OBJECTIVE: To investigate alterations in protein kinases and phosphatases that regulate the activity of mitogen activated protein kinase (MAPK) in sporadic inclusion body myositis (IBM). BACKGROUND: In vacuolated fibers in IBM, several studies reported upregulation of the extracellular regulated kinase (ERK) subclass of MAPK family. Whereas MAPK kinases (MKK) activate MAPK, MAPK phosphatases (MKP) inactivate MAPK. MKP-1 is involved in muscle fiber differentiation and it is downregulated during myotube formation. METHODS: Immunolocalization of MKK1 through MKK4 and MKP-1 to MKP-3 was tested in muscle specimens from 10 patients with IBM and controls. RESULTS: In IBM, strong and focal deposits of MKP-1 were observed in vacuolated fibers. The MKP-1-positive deposits were colocalized with ERK. MKP-2, MKP-3, and MKK were not associated with vacuolated fibers. CONCLUSIONS: In IBM, MKP-1 is abnormally induced in vacuolated fibers probably to inactivate ERK. Although direct activators other than those tested in the current study might induce ERK, the absence of activation of MKK suggests that the aggregation of ERK protein itself causes the seeming upregulation of the protein kinase in IBM. Like ERK and its nuclear substrate, MKP-1 is an enzyme that forms aggregates in vacuolated fibers and is involved in myogenesis.

Adult↗