Classification of late myopathies based on observation of 15 new cases

Summary The author observed 15 cases of chronic progredient myopathy in which the first symptoms appeared after the age of 40. 7 of them belonged to the group of primary, non-purulent polymyositis and the possibility of a polymyositic origin was also present in the 8th case.Cases 9 to 15 appeared to be differentiable both from polymyositis and muscular dystrophy. Thus the author had no case which could have been regarded unequivocally as progressive muscular dystrophy. These 7 cases of unknown etiology do not constitute a uniform group nor do they represent even an independent nosological entity.In the classification of myopathies beginning after the 30th year, the author tried to follow etio-pathogenetic principles. With modern diagnostic techniques, e.g., with the biochemical, histochemical and electron microscopic examination of the muscle biopsy, it is possible to shed light on some conditions underlying late myopathies, such as endocrine changes, dysmetabolic changes, deficiency of muscle enzymes, muscle tuberculosis or sarcoidosis, etc.The residual cases may be grouped under 3 main headings: they are classified as progressive muscular dystrophy or as chronic polymyositis or may be considered as an independent disease entity which can be distinguished from both above conditions. Several authors have an extremely uniform view on these 3 possibilities.Based on literary data and on the author's experience, it can be stated that the most important group of late myopathies consists of polymyositides.Only an insignificant part of late myopathies may be regarded as progressive muscular dystrophy.In the third group, constituted by the remaining late myopathy cases, the only common feature is their unknown etiology. From the standpoint of clinical picture, histopathologic findings and therapeutic responsiveness, we are faced with a heterogenous syndrome.

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Zusammenfassung Es wurde über die Beobachtung von 15 progredienten, chronischen Myopathie-Fällen berichtet, bei denen die ersten Symptome über das 40. Lebensjahr erschienen. 7 Fälle gehörten zu den primären, nicht eitrigen Polymyositiden, und auch im Fall 8 bestand die Möglichkeit eines polymyositischen Ursprungs.Die Fälle 9 bis 15 schienen von den Polymyositiden und auch von der progressiven Muskeldystrophie abtrennbar. Der Autor hatte also keinen Spätmyopathiefall, der als Dystrophia musculorum progressiva betrachtet werden konnte. Die 7 Fälle unbekannter Ätiologie bilden keine einheitliche Gruppe und stellen auch keine selbständige nosologische Einheit dar.In der Klassifikation der nach dem 30. Lebensjahr beginnenden Myopathien wurde versucht, ein ätio-pathogenetisches Prinzip einzuleiten. Moderne diagnostische Verfahren, unter anderem biochemische, histochemische und elektronenmikroskopische Untersuchungen der Muskelbiopsie, vermögen den Hintergrund einiger Spätmyopathien aufzuklären, so z. B. Endokrinopathien, dysmetabolische Veränderungen, Mangel an Muskelenzymen, Muskeltuberkulose, Muskelsarkoidose usw.Die zurückbleibenden Fälle werden in 3 Gruppen eingeteilt: 1. Progressive Muskeldystrophien. 2. Chronische Polymyositiden. 3. Fälle, die eine selbständige nosologische Einheit darstellen und welche von den zwei obenerwähnten Untergruppen abgrenzbar sind. Die Meinung einiger Autoren ist eine äußerst uniformisierte über diese 3 Möglichkeiten.Auf Grund der Literaturangaben und eigener Erfahrungen war es feststellbar, daß die Polymyositiden die wichtigste Gruppe der Spätmyopathien darstellen.Nur einen unbedeutenden Teil der Spätmyopathien kann man für eine Dystrophia musculorum progressiva betrachten.In der dritten Gruppe, welche sich aus den zurückbleibenden Spätmyopathie-Fällen zusammensetzt, ist die einzige gemeinsame Eigenschaft der Fälle ihre unbekannte Ätiologie. Diese Gruppe stellt vom Standpunkt des klinischen Bildes, der histopathologischen Befunde und der therapeutischen Beeinflußbarkeit ein heterogenes Syndrom dar.

     

Fibrodysplasia ossificans progressiva

A 2-year-old boy presented with low-grade fever and multiple progressive painful swellings over upper dorsal trunk and supraclavicular region with progressive stiffening of skin for the last 2 months. Examination revealed dysmorphic face, proximally placed thumb and bilateral hallux valgus. Hence, a diagnosis of Fibrodysplasia Ossificans Progressiva was entertained     

MRI findings of myositis in Behçet disease

We report the magnetic resonance imaging findings in a case of localized myositis in a 23-year-old man with long-standing Behçet disease.     

Muscle involvement in rheumatoid arthritis: an ultrastructural study

An electron microscopic investigation has been carried out on muscle bioptic samples from patients affected by rheumatoid arthritis (RA). This study was undertaken to seek further ultrastructural alterations affecting striated muscles in RA pathology. Bioptic samples were collected on a total of 30 surgical interventions of hip (10), knee(8), and foot (12). This yielded three muscle types: gluteus maximus, vastus lateralis, and extensor digitorum communis. Muscle samples from 12 patients with no RA stigmata, selected to match RA patients by age and gender, constituted the control group. Tissue samples were prepared both for conventional histochemical methods and according to conventional electron microscopic procedures, including morphometric analysis. Although to a different extent in each sample, in muscles from RA vs. controls the authors observed the simultaneous presence of discrete muscular alterations such as wider separation of myofibrils, myelin figures, dilated sarcotubular system, pleomorphic mitochondria, myofibril flaking, and lipofuscin deposition in the subsarcolemmal region. In addition to a progressive atrophy, the above findings are suggestive of rheumatoid myositis and lend further support to the still poorly documented presence of an idiopathic inflammatory myopathy and inclusion body myositis associated with RA.     

Pathophysiology and Treatment of Streptococcal Toxic Shock Syndrome

We have recently encountered three cases of streptococcal toxic shock syndrome, each of which had a different cause. All the patients had inflammation of soft tissue in the lower extremities, and developed shock and multiple organ failure immediately after the clinical visit. The inflammation of soft tissue was necrotising fasciitis in one case, myositis in one case, and phlegmon in one. In the first case the debridement was incomplete, which resulted in an extensive ulceration. Wary of repeating this experience, we made an early diagnosis and did a thorough debridement in the second case. The patient was ultimately discharged without complications. It is rare that a patient with extensive myositis survives without amputation of the extremity. The third patient responded well to early treatment with antibiotics.     

皮肌炎／多发性肌炎127例临床分析

目的 探讨DM/PM的临床表现，主要诊断要点和疗效。方法 分析了127例住院病例资料。结果 初发症状以皮损（52.8％）和皮损肌炎同发（31.5％）多见，而仅有肌炎占14.9％。皮损以眼睑及眶周浮肿性紫红色斑（85.2％），Gottron丘疹（58.3％），甲周红斑（43.5％）和皮肤异色病样改变（43.5％）多见，胆炎以四肢近端（82.3％）和吞咽肌群（44.9％）多见。此外可见血清肌浆酶和24h尿肌酸排出量增加，肌电图异常，少数合并恶性肿瘤及心肌受累。皮质激素治疗有效，与MTX合用能提高疗效。结论 眼睑及眶周浮肿紫红色斑，四肢近端肌无力，肌痛，肌力减退及24h尿肌酸排出量增加为主要诊断要点。皮质激素治疗有效，合用MTX能提高疗效。