These results indicate that combined treatment with C-peptide and insulin for 3 months may improve renal function by diminishing urinary albumin excretion and ameliorate autonomic and sensory nerve dysfunction in patients with Type 1 diabetes mellitus.

The etiology of upper airway collapsibility in patients with snoring and obstructive sleep apnea (OSA) remains unclear. Local muscular abnormalities, including neurogenic lesions, could be a contributory factor. The aim of this study was to histologically evaluate the hypothesis of a progressive snorers disease. Biopsies of palatopharyngeal muscle were obtained from 21 patients with habitual snoring and different degrees of upper airway obstruction (10 patients with OSA) and 10 nonsnoring control subjects. Morphological abnormalities, including neurogenic signs (e.g., type grouping), were blindly quantified. The degree of abnormality was significantly increased in patients compared with control subjects. The individual score of abnormalities was significantly correlated to the percentage periodic obstructive breathing but not to oxygen desaturation index. Analyses of the individual fiber-size spectra demonstrated a significantly increased number of hypertrophied and/or atrophied fibers in patients compared with controls. The subjects were also divided into three groups according to their type of nocturnal breathing, i.e., nonsnorers, patients with < 20%, and patients with > or = 45% obstructive breathing. These groups correlated significantly with the degree of abnormality and pathological fiber-size spectra. In conclusion, these results support the hypothesis of a progressive local neurogenic lesion, caused by the trauma of snoring, as a possible contributory factor to upper airway collapsibility.

Post‐polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e‐mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non‐swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non‐invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow‐up and patient education are useful for the patients’ mental status and well‐being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential‐specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long‐term effects of muscular training.

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In some myopathies of distal onset, the intermediate filament desmin is abnormally accumulated in skeletal and cardiac muscle. We report the first point mutation in desmin cosegregating with an autosomal dominant form of desmin-related myopathy. The L345P desmin missense mutation occurs in a large, six generation Ashkenazi Jewish family. The mutation is located in an evolutionarily highly conserved position of the desmin coiled-coil rod domain important for dimer formation. L345P desmin is incapable of forming filamentous networks in transfected HeLa and SW13 cells. We conclude that the L345P desmin missense mutation causes myopathy by interfering in a dominant-negative manner with the dimerization-polymerization process of intermediate filament assembly.

Muscle biopsies were obtained from the anterior tibial muscle (TA) of 15 healthy, sedentary young (23-37 years) and 13 healthy and physically active elderly (66-77 years) volunteers. The mean frequency of type I fibres was lower in the young subjects compared with the elderly, but the mean type I fibre cross-sectional area was equal in the two groups. The type IIA fibres were, however, smaller in the elderly than in young subjects. Capillary density, capillary per fibre ratio, capillaries in contact with type I fibres (CC) and CC in relation to type I and type II fibre area did not differ in the two groups. The number of capillaries in contact with type IIA fibres was higher in the younger group. Only occasional and minor pathological changes were observed in the young subjects. In the elderly, such changes were much more common, including central nuclei, ring fibres, fibre splitting, scattered highly atrophic fibres, moth-eaten fibres and vacuoles. Ring fibres were most easily identified with anti-desmin labelling and highly atrophic fibres exhibited a rough network of labelling. Increased content of actin and spectrin was also observed at the periphery of ring fibres. In the elderly group, a qualitative ultrastructural analysis was also obtained and obvious changes included some myofilament loss, collections of lipofuscin which were also observed in satellite cells, proliferation of the SR-T systems and increased wrinkling of nuclear membranes and sarcolemma.

Objective. High mobility group box chromosomal protein 1 (HMGB-1) is an endogenous nuclear protein that can be translocated to the cytoplasm and then released extracellularly. It can induce tumor necrosis factor and interleukin-1 production in myeloid cells. Increased expression of these 2 cytokines has been observed in muscle tissue of patients with polymyositis (PM) and dermatomyositis (DM). The present study was therefore undertaken to investigate how HMGB-1 is expressed in muscle tissue of patients with myositis and, if so, whether such expression is modulated by prednisolone therapy.Methods. Muscle biopsy specimens from 5 patients with PM and 4 patients with DM, obtained before and 3-6 months after initiation of prednisolone therapy, were assessed by conventional microscopic evaluation and computerized image analysis, and HMGB-1 expression was investigated by immunohistochemical staining.Muscle biopsy specimens from 7 healthy controls were also studied.Results High mobility group box chromosomal protein 1 (HMGB-1; previously termed HMG-1 or amphoterin) is a structural nuclear protein that binds DNA. It is ubiquitously present in the nuclei of all mammalian cells and is highly conserved between species (1). HMGB-1 can be released extracellularly and then has proinflammatory properties (2,3). It has recently been discovered that monocyte/macrophages activated by endotoxin, tumor necrosis factor (TNF), or interleukin-1 (IL-1) secrete HMGB-1 in substantial amounts (4). Furthermore, recent evidence implicates HMGB-1 as an important proinflammatory molecule in several conditions, such as