Inclusion body myopathy
Intravenous immune globulin in hereditary inclusion body myopathy: a pilot study
... Patient 3 is a 39-year-old woman of Bohemian/Czech/ French Canadian ancestry diagnosed with HIBM at the age of 31 years. Following a year of progressive tripping and falling, she awoke one day with numbness from the ...
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Isolated inclusion body myopathy caused by a multisystem proteinopathy–linked hnRNPA1 mutation
... ferences in genetic background may be associated with the Japanese MSP3 phenotype. The selective involve- ment of skeletal muscles reported in this study should be confirmed, particularly by pathologic analyses as a ...
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Clinical Reasoning: A 40-year-old woman presenting with distal leg weakness
... hereditary inclusion body myopathy (hIBM), Laing myopathy (MYH7 mutation), and some myofibrillar ...Laing myopathy (MYH7 mutation) often presents with disproportionate weakness of ...
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Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant
... cause inclusion-body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD) ...for myopathy, 49% for Paget’s disease, and 30% for FTD ...
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Clinical Reasoning: Progressive proximal weakness in a 56-year-old man with bone pain
... Rimmed vacuoles are a nonspecific finding in many myo- pathies, including sporadic IBM (sIBM) and myofibrillar myopathy (MFM). As noted previously, the patient’s pattern of muscle involvement was not typical for ...
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Sporadic inclusion body myositis: the genetic contributions to the pathogenesis
... Sporadic inclusion body myositis (sIBM) is the commonest form of idiopathic inflammatory myopathy among individ- uals aged over 50 [1]. It has a male predominance and a prevalence of 1–71 people per ...
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Pathology and Development of the Grasshopper Inclusion Body Virus in Melanoplus sanguinipes
... Electron microscopic investigations of viral replication showed that at about 8 days after inoculation presumptive viral particles had developed as buds or protrusions from precursor gra[r] ...
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Multicenter questionnaire survey for sporadic inclusion body myositis in Japan
... This was a cross-sectional study targeting patients with sIBM registered at the collaborating institutes, which re- ceived patients with myopathy in Japan. The detailed in- formation was gleaned from 146 patients ...
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Development and validation of a Bayesian survival model for inclusion body myositis
... “lower body severity” status, a multinomial distribution with probabilities equal to that observed in the TMA dataset was used, with the assumption that all individuals with “severe” lower body impairment ...
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STEROID MYOPATHY
... In two of the patients who developed myopathy during therapy with dexamethazone, mild improvement oc curred when treatment with what are gen erally considered equivalent anti-inflamma to[r] ...
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Nemaline Myopathy With Dilated Cardiomyopathy in Childhood
... Nemaline myopathy is a nonpro- gressive congenital skeletal muscle disorder de fi ned by the presence of inclusions known as nemaline rods (Greek nema = thread) in muscle fi ...nemaline myopathy and ...
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Clinical Reasoning: Subacute paresis in a 28-year-old man with HIV
... Weakness with a pattern of more proximal and axial involvement and rather subacute course could suggest HIV-associated neuromuscular disorders including in- flammatory demyelinating polyneuropathy, motor neuron disease, ...
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TIGAR inclusion pathology is specific for Lewy body diseases
... disorder. A key pathological hallmark of the disease is loss of dopaminergic neurons in the substantia nigra, resulting in the clinical triad of tremor, akinesia and rigidity. A further diagnostic pathological feature of ...
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A metastable subproteome underlies inclusion formation in muscle proteinopathies
... sion body myositis for proteins with coverage across sample ...in inclusion body myositis using Z agg ...in inclusion body myositis using ...
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Custom made inclusion bodies: impact of classical process parameters and physiological parameters on inclusion body quality attributes
... that IBs can be produced in high concentration (so that the amount of generated product often outweighs the additional downstream steps), IB based processes are believed to fundamentally boost time/space yields for ...
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Pathogenesis of FUS-associated ALS and FTD: insights from rodent models
... Despite the absence of confirmed genetic cases, inclu- sions immunoreactive for FUS are present in a small proportion FTLD cases (FTLD-FUS) and can be neuro- pathologically sub-categorised as atypical FTLD-U, neur- onal ...
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Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration
... Choreoathetoid involuntary movements are rarely reported in patients with frontotemporal lobar degeneration (FTLD), suggesting their exclusion as a supportive feature in clinical diagnostic criteria for FTLD. Here, we ...
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On the connection between two equilibrium problems for cracked bodies in the cases of thin and volume rigid inclusions
... Let us formulate an equilibrium problem for an elastic body containing a volume rigid inclusion. We consider the case of the partly delaminated inclusion. In this case we have an interfacial crack ...
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Challenges in diagnosis and treatment of sporadic inclusion-body myositis
... Sporadic inclusion body myositis (sIBM) is a rare yet increasingly prevalent disease and the most common cause of inflammatory myopathy in people over the age of ...
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Emerging therapeutic options for sporadic inclusion body myositis
... Sporadic inclusion body myositis is the most common inflammatory muscle disorder preferentially affecting males over the age of 40 ...of inclusion body myositis and the current efforts in ...
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