| Autor: |
de Camargo LV; Departamento de Neurologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., de Carvalho MS; Departamento de Neurologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., Shinjo SK; Disciplina de Reumatologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil., de Oliveira ASB; Setor de Doenças Neuromusculares, Disciplina de Neurologia, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil., Zanoteli E; Departamento de Neurologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil. |
| Jazyk: |
angličtina |
| Zdroj: |
BioMed research international [Biomed Res Int] 2018 Jan 29; Vol. 2018, pp. 5069042. Date of Electronic Publication: 2018 Jan 29 (Print Publication: 2018). |
| DOI: |
10.1155/2018/5069042 |
| Abstrakt: |
Sporadic inclusion body myositis (sIBM) is considered the most common acquired myopathy aged over 50 years. The disease is characterized by a particular process of muscle degeneration characterized by abnormal deposit of protein aggregates in association with inflammation. The aim of this study was to present clinical and muscle histopathological findings, including immunostaining for LC3B, p62, α -synuclein, and TDP-43, in 18 patients with sIBM. The disease predominated in males (61%) and European descendants, with onset of clinical manifestations around 59 years old. The most common symptoms were muscle weakness, falls, dysphagia, and weight loss. Hypertension was the main comorbidity. Most of the cases presented with paresis predominantly proximal in lower limbs and distal in upper limbs. Immunosuppressive treatment showed to be not effective. Muscle histological findings included dystrophic changes, endomysial inflammation, increased lysosomal activity, and presence of rimmed vacuoles and of beta-amyloid accumulation, in addition to high frequency of mitochondrial changes. There was increased expression of LC3B, p62, α -synuclein, and TDP-43 in muscle biopsies. The sIBM has characteristic clinical and histological findings, and the use of degeneration and autophagic markers can be useful for the diagnosis. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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