Endoscopic full-thickness resection (EFTR) is a powerful option for resection of colorectal lesions not amenable to conventional endoscopic resection. The full-thickness resection device (FTRD) allows clip-assisted EFTR with a single-step technique. We report on results of a large nationwide FTRD registry. The "German colonic FTRD registry" was created to further assess efficacy and safety of the FTRD System after approval in Europe. Data were analyzed retrospectively. Sixty-five centers contributed 1,178 colorectal FTRD procedures. https://www.selleckchem.com/products/tng908.html Indications for EFTR were difficult adenomas (67.1%), early carcinomas (18.4%), subepithelial tumors (6.8%), and diagnostic EFTR (1.3%). Mean lesion size was 15 × 15 mm and most lesions were pretreated endoscopically (54.1%). Technical success was 88.2% and R0 resection was achieved in 80.0%. R0 resection was significantly higher for subepithelial tumor compared with that for other lesions. No difference in R0 resection was found for smaller vs larger lesions or for colonic vs rectal procedures. Adverse events occurred in 12.1% (3.1% major events and 2.0% required surgical treatment). Endoscopic follow-up was available in 58.0% and showed residual/recurrent lesions in 13.5%, which could be managed endoscopically in most cases (77.2%). To date, this is the largest study of colorectal EFTR using the FTRD System. The study demonstrated favorable efficacy and safety for "difficult-to-resect" colorectal lesions and confirms results of previous studies in a large "real-world" setting. Further studies are needed to compare EFTR with other advanced resection techniques and evaluate long-term outcome. To date, this is the largest study of colorectal EFTR using the FTRD System. The study demonstrated favorable efficacy and safety for "difficult-to-resect" colorectal lesions and confirms results of previous studies in a large "real-world" setting. Further studies are needed to compare EFTR with other advanced resection techniques and evaluate long-term outcome.Senile systemic amyloidosis (SSA), or wild-type transthyretin (wtATTR) amyloidosis, is associated most commonly with cardiomyopathy and carpal tunnel syndrome. SSA-associated skeletal myopathy is rare. We describe the case of a patient with SSA who exhibited asymmetric quadriceps and finger flexor weakness, a phenotype usually seen in inclusion body myositis.Inclusion body myositis (IBM) is the most common acquired myopathy in adults older than 50 years. Muscle biopsy remains the gold standard for diagnosis. Recently described serum antibodies against cytosolic 5-nucleotidase 1A (cN1A) are considered highly specific for IBM. However, positive cN1A antibodies in diseases other than IBM are recently reported. We review 2 cases in which serum antibodies were positive but ancillary testing revealed motor neuron disease. A 68-year-old man presented with asymmetric quadriceps and handgrip weakness prompting concern for IBM. However, electromyography showed purely chronic neurogenic abnormalities, and muscle biopsy was consistent with post-polio syndrome. A 60-year-old woman reported a history of progressive muscle weakness. Despite positive antibodies, examination and electromyography were indicative of amyotrophic lateral sclerosis. Serum cN1A antibodies are not 100% specific for the diagnosis of IBM. Careful clinical, electrophysiologic, and histopathologic correlation is required in workup of individuals with neuromuscular weakness and positive antibodies.What is in the Literature focuses on peripheral neuropathies with new and practical information related to the diagnosis, treatment, and management. Diagnostic and treatment guidelines are available for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but not all clinicians follow them resulting in erroneous diagnoses and prolonged treatment. Secondary axonal loss in CIDP causes increased connective tissue in muscle. Antibodies to proteins at the node of Ranvier are found in a small percentage of patients with CIDP. The differential diagnosis for CIDP-like neuropathies includes amyloid neuropathy and POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) and amyloidosis. Upper limits for cerebral spinal fluid protein are 0.45 g/L and cell count less then 10/µL, but both may be too low. Hyperactive reflexes may occur in Guillain-Barré syndrome and should not exclude the diagnosis. In severely affected Guillain-Barré syndrome patients, a second dose of intravenous immune globulin within 4 weeks of onset is not likely to be effective.Laing distal myopathy (LDM) is an autosomal dominant disorder caused by mutations in the slow skeletal muscle fiber myosin heavy chain (MYH7) gene on chromosome 14q11.2. The classic LDM phenotype-including early-onset, initial involvement of foot dorsiflexors and great toe extensors, followed by weakness of neck flexors and finger extensors-is well documented. Since the original report by Laing et al in 1995, the spectrum of MYH7-related myopathies has expanded to include congenital myopathies, late-onset myopathies, myosin storage myopathy, and scapuloperoneal myopathies. Most patients with LDM harbor mutations in the midrod domain of the MYH7 gene, but rare cases document disease-associated mutations in the globular head region. In this report, we add to the medical literature by describing the clinicopathological findings in 8 affected family members from 4 new LDM families-including 2 with novel MYH7 mutations (Y162D and A1438P), one with dual mutations (V39M and K1617del), and one family (E1508del) with severe early-onset weakness associated with contractures, respiratory insufficiency, and dilated cardiomyopathy. Our families highlight the ever-expanding clinical spectrum and genetic variation of the skeletal myopathies related to MYH7 gene mutations. To compare temporal trends in clinical and health care resource utilization (HRU) outcomes in people with refractory and nonrefractory generalized myasthenia gravis (gMG). A retrospective analysis of data from adults with gMG in the Myasthenia Gravis Foundation of America Patient Registry. gMG status (ever-refractory or always nonrefractory) and clinical (Myasthenia Gravis-Activities of Daily Living [MG-ADL] scores, exacerbations) and HRU outcomes were determined from questionnaires self-completed 6-monthly for up to 4 years. The probability of each outcome was compared for the 2 groups over time. The mean MG-ADL score and the probability of experiencing each outcome were significantly greater in the ever-refractory versus nonrefractory groups during each year of follow-up. Between-group differences in time trends were statistically significant for intensive care and feeding-tube use. People who have ever had refractory gMG may have worse functional status, more exacerbations, and higher HRU than people with consistently nonrefractory disease.