Neuromuscular manifestations of L-tryptophan-associated eosinophilia-myalgia syndrome: A histomorphologic analysis of 14 patients

Verity, M. Anthony; Bulpitt, K. J.; Paulus, Harold E.

doi:10.1016/0046-8177(91)90054-s

Cited by 24 publications

(5 citation statements)

References 27 publications

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“…We enrolled 57 consecutive DM patients (median and range of age at biopsy: 38.5 [28–84] years, sex ratio [male:female] 1:1.8 in adult; 6 years , 1:0.8 in children) who were diagnosed with definite, probable or possible DM based on the European Neuromuscular Centre (ENMC) criteria in the French, German and Japanese biopsied muscle repositories (Pitié‐Salpêtrière University Hospital/Institute of Myology, Charité‐Universitätsmedizin, and National Center of Neurology and Psychiatry). A total of 40 patients were positive for any DM‐specific autoantibody [anti‐Mi‐2, transcription intermediary factor 1 gamma (TIF1‐γ), nuclear matrix protein 2 (NXP2), melanoma differentiation‐associated gene 5 (MDA5) or small ubiquitin‐like modifier activating enzyme (SAE)] and 17 had no detectable autoantibody.…”

Section: Methodsmentioning

confidence: 99%

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Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis

Uruha

Allenbach

Charuel

et al. 2018

Neuropathology Appl Neurobio

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Aims To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analysing different DM subforms, and to test the superiority of MxA to other markers. Methods Immunohistochemistry for MxA and retinoic acid‐inducible gene I (RIG‐I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti‐Mi‐2 (n = 6), ‐transcription intermediary factor 1 gamma (n = 10), ‐nuclear matrix protein 2 (n = 13), ‐melanoma differentiation‐associated gene 5 (MDA5) (n = 10) or ‐small ubiquitin‐like modifier activating enzyme (n = 1) autoantibodies and with no detectable autoantibody (n = 17). Among the patients, nine suffered from cancer and 22 were juvenile‐onset type. Disease controls included antisynthetase syndrome (ASS)‐associated myositis (n = 30), immune‐mediated necrotizing myopathy (n = 9) and inclusion body myositis (n = 5). Results Sarcoplasmic MxA expression featured 77% sensitivity and 100% specificity for overall DM patients, while RIG‐I staining and PFA reached respectively 14% and 59% sensitivity and 100% and 86% specificity. In any subset of DM, sarcoplasmic MxA expression showed higher sensitivity than RIG‐I and PFA. Some anti‐MDA5 antibody‐positive DM samples distinctively showed a scattered staining pattern of MxA. No ASS samples had sarcoplasmic MxA expression even though six patients had DM skin rash. Conclusions Sarcoplasmic MxA expression is more sensitive than PFA and RIG‐I expression for a pathological diagnosis of DM, regardless of the autoantibody‐related subgroup. In light of its high sensitivity and specificity, it may be considered a pathological hallmark of DM per se. Also, lack of MxA expression in ASS supports the idea that ASS is a distinct entity from DM.

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Section: Methodsmentioning

confidence: 99%

“…Although mostly small regenerating fibres in perifascicular regions make up the lesion, it may occasionally be difficult to clearly differentiate between PFA of DM and that of ASS‐associated myositis or other inflammatory myopathies harbouring atrophic fibres in the periphery of the fascicles (e.g. Shulman Syndrome) .…”

Section: Introductionmentioning

confidence: 99%

Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis

Uruha

Allenbach

Charuel

et al. 2018

Neuropathology Appl Neurobio

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“…hluscle biopsies showed a characteristic histopathological picture, with extensive inflanimation and fibrosis in the connective tissue surrounding the muscle, but scant evidence of muscle fiber damage (Fig 3) [39,42,43]. hlyalgia was a salient finding in acute EMS in essentially all patients and persisted in the form of intermittent severe spasms.…”

Section: Clinical Features Of Emsmentioning

confidence: 98%

L-Tryptophan-Associated Eosinophilia-Myalgia Syndrome: Clinical and Pathological Features of an Evolving New Disease and Current Concepts of Etiology

Varga

1993

J Intensive Care Med

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Varga J. L-tr)ptophan-associated eosinophilia-niyalgia syndrome: clinical and pathological features of an evolving new disease and current concepts of etiology. J Intensive Care hled 1993;8:229-242. ~~ ~ The eosinophilia-myalgia syndrome (ENS) is a newly recognized illness that occurred in the United States and in other countries in an'epidemic form during 1989. The outbreak was associated with ingestion of the essentid amino acid Ltryptophan. T h e illness appears to be heterogeneous; it has an early phase characterized by myalgia, skin rashes, constitutional symptoms, and marked peripheral blood eosinophilia, f o l l o n d by a late phase characterized by chronic cutaneous, neuromusculx, pulmonary, and other manifestations. Because EMS has no pathognomonic findings, the diagnosis is based on recognizing the characteristic clinical and laboratory features of the syndrome, supported by the histopathological findings of inflammation and fibrosis of the subcutaneous fascia and the connective tissue structures surrounding skeletal muscle and peripheral nerve fibers. Toxicoepidemiological studies suggest that the epidemic of EXIS was caused by a point-source contamination of Ltryptophan preparations. Analysis of L-tryptophan preparations identified a tryptophan-related novel amino acid present only in batches associated n-ith EMS. but extensive studies with animals have thus far failed to establish an etiologic role for this contaminant in EXIS. A recently identified second contaminant is chemically similar to analide derivatives isolated from samples of cooking oils that caused the toxic oil epidemic in Spain. Experimental studies suggest a major role for eosinophil and fibroblast activation in the pathogenesis of EMS. This newly recognized illness has focused intense interest on the role of chemical and environmental agents in the pathogenesis of various idiopathic illnesses characterized by inflammation and fibrosis.

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“… 38 Although the pathogenesis of this disease is still poorly understood, cutaneous and muscle biopsies of patients with EMS showed accumulation of acid mucopolysaccharide deposits in the fascial and perimysial regions, specifically around the arterioles and small arteries, without evidence of arteritis. 39 , 40 Perineural fibrosis and loss of microvasculature were noted on histopathological studies of EMS patients. 39 Focal perifascicular atrophy was also reported in the progression of the disease.…”

Section: Hyaluronan and Painmentioning

confidence: 99%

Hyaluronan homeostasis and its role in pain and muscle stiffness

Amir

Kim

Stecco

et al. 2022

PM&R

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Hyaluronan (HA) is a glycosaminoglycan that consists of single-chain polymers of disaccharide units of glucuronic acid and N-acetylglucosamine. It is a chief constituent of the extracellular matrix. About 27% of the total HA in the body is expressed in the skeleton and connective tissue, while 8% is expressed in muscles. In physiological conditions, HA functions as a lubricant and viscoelastic shock absorber. Additionally, HA is part of complex cellular signaling which modulates nociception and inflammation. This study aims to understand the role that HA plays in the musculoskeletal system, specifically in muscles and the surrounding fascia. This review is also intended to further understand HA homeostasis and the process of its synthesis, degradation, and clearance from the local tissue. The authors examined muscle pain and stiffness as pathological conditions associated with HA accumulation.

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Neuromuscular manifestations of L-tryptophan-associated eosinophilia-myalgia syndrome: A histomorphologic analysis of 14 patients

Cited by 24 publications

References 27 publications

Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis

Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis

L-Tryptophan-Associated Eosinophilia-Myalgia Syndrome: Clinical and Pathological Features of an Evolving New Disease and Current Concepts of Etiology

Hyaluronan homeostasis and its role in pain and muscle stiffness

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