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Approach to Neuromyopathy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
The association of neuromyopathic change and protein aggregates with lack of inflammation, with or without rimmed vacuoles, suggests myofibrillar myopathies (MFM). The presence of neuromyopathic change and rimmed vacuoles with lack of inflammation and protein aggregates expands the differential diagnoses into MFM type V, oculopharyngeal muscular dystrophy (OPMD), Welander distal myopathy (WDM), Emery-Dreifuss dystrophy type II (EDD), distal myopathy with ADSSL1 mutation, Limb-girdle muscular dystrophy (LGMD) type 1a and 1g, distal dystrophy with rimmed vacuoles (DDRV), and autosomal dominant vacuolar myopathy (ADNM). Treatment history is important in these diseases to rule out healed inflammatory myopathies such as treated inclusion body myositis (IBM). Inflammatory markers (CD4, CD8, and CD68) and major histocompatibility (MHC class-I) expression can enlighten the autoimmune inflammatory process.
Examine the lower limbs
Published in Hani TS Benamer, Neurology for MRCP PACES, 2019
Q: What are the causes of proximal myopathy? Polymyositis or dermatomyositis.Inclusion body myositis, which is the most common form of myopathy after the age of 50 years.Endocrine causes: Cushing’s syndrome and thyrotoxicosis.Drugs: steroids, amiodarone, lithium and statins.Alcoholism.Limb girdle dystrophy.Metabolic myopathies.Osteomalacia.Paraneoplastic myopathy (very rare).
Neurological and neuromuscular disorders of the larynx
Published in Declan Costello, Guri Sandhu, Practical Laryngology, 2015
Inclusion body myositis (IBM) is the most common progressive muscle disease in people over 50.104 It is an inflammatory myopathy of uncertain cause and distinct from immune disorders such as dermatomyositis and polymyositis. Most forms are sporadic, although a hereditary form has been described. The ‘inclusion body’ of the name refers to accumulations of proteinaceous material within muscle fibres that are similar to those in the Alzheimer’s brain, including amyloid-beta, phosphorylated tau protein and 20 other proteins.105 In addition, there is a pronounced inflammatory infiltrate. Diagnosis depends on these and other characteristic histological findings on muscle biopsy.
A case of inclusion body myositis complicated by microscopic polyangiitis
Published in Scandinavian Journal of Rheumatology, 2018
S Yamada, H Yamashita, K Taira, A Hida, N Arai, J Shimizu, Y Miyaji, M Sonoo, A Yashima, Y Takahashi, H Kaneko
Inclusion body myositis (IBM) is characterized by chronic muscle weakness with mononuclear cell infiltration of skeletal muscle. IBM is distinguished from polymyositis and dermatomyositis both histopathologically and clinically. Microscopic polyangiitis (MPA) is a vasculitis related to anti-neutrophil cytoplasmic autoantibody (ANCA) that exhibits various symptoms, such as mononeuritis multiplex. To our knowledge, there have been no previous reports of IBM complicated by MPA. Here, we present the first case of MPA in a patient with IBM.
The Diagnostic Yield of Electromyography at Detecting Abnormalities on Muscle Biopsy: A Single Center Experience
Published in The Neurodiagnostic Journal, 2021
Patrick B. Moloney, Stela Lefter, Aisling M. Ryan, Michael Jansen, Niamh Bermingham, Brian McNamara
There was concordance between EMG and muscle biopsy findings in 134 cases (76.6%). The aforementioned cases with “false positive” and “false negative” investigations, comprised of 31 (17.7%) of those with discordant studies. The remaining 10 cases (5.7%) had conflicting EMG and muscle biopsy findings (myopathic EMG with neurogenic biopsy or neurogenic EMG with myopathic biopsy). Four of these cases were diagnosed with inclusion body myositis (IBM) by muscle biopsy, after initially demonstrating EMG findings consistent with a neurogenic process.
Health care costs and comorbidities for patients with inclusion body myositis
Published in Current Medical Research and Opinion, 2018
Allison Keshishian, Steven A. Greenberg, Neetu Agashivala, Onur Baser, Kristen Johnson
Inclusion body myositis (IBM) is an inflammatory autoimmune disorder of skeletal muscle, with no effective treatment, resulting in progressive limb weakness and loss of function1–3. It is a late-onset disease with an average onset age of 61–66 years4–6 and delayed diagnosis, resulting in an estimated age of 65–70 years at diagnosis. IBM is an orphan disease, as defined by the US Food and Drug Administration, with published prevalence estimates of 11–117 per million7–9.