New-Onset Myasthenia Gravis Mimics Spinal Cord Injury After Zenker's Diverticulum Repair.
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ABSTRACT:
SUBMITTER: Meiler SE
PROVIDER: S-EPMC10248725 | biostudies-literature | 2023 Apr-Jun
REPOSITORIES: biostudies-literature
Publications
New-Onset Myasthenia Gravis Mimics Spinal Cord Injury After Zenker's Diverticulum Repair.
Meiler Steffen E SE Siwemuke Tylin J TJ Postma Gregory N GN
OTO open 20230401 2
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Project description:ImportanceUse of biologic agents in generalized myasthenia gravis is generally limited to therapy-refractory cases; benefit in new-onset disease is unknown.ObjectiveTo assess rituximab in refractory and new-onset generalized myasthenia gravis and rituximab vs conventional immunotherapy in new-onset disease.Design, setting, and participantsA retrospective cohort study with prospectively collected data was conducted on a county-based community sample at Karolinska University Hospital, Stockholm, Sweden. Participants included 72 patients with myasthenia gravis, excluding those displaying muscle-specific tyrosine kinase antibodies, initiating rituximab treatment from January 1, 2010, to December 31, 2018, and patients with new-onset disease initiating conventional immunotherapy from January 1, 2003, to December 31, 2012, with 12 months or more of observation time. The present study was conducted from March 1, 2019, to January 31, 2020.ExposuresTreatment with low-dose rituximab (most often 500 mg every 6 months) or conventional immunosuppressants.Main outcomes and measuresTime to remission (main outcome) as well as use of rescue therapies or additional immunotherapies and time in remission (secondary outcomes).ResultsOf the 72 patients included, 31 patients (43%) were women; mean (SD) age at treatment start was 60 (18) years. Twenty-four patients had received rituximab within 12 months of disease onset and 48 received rituximab at a later time, 34 of whom had therapy-refractory disease. A total of 26 patients (3 [12%] women; mean [SD] age, 68 [11] years at treatment start) received conventional immunosuppressant therapy. Median time to remission was shorter for new-onset vs refractory disease (7 vs 16 months: hazard ratio [HR], 2.53; 95% CI, 1.26-5.07; P = .009 after adjustment for age, sex, and disease severity) and for rituximab vs conventional immunosuppressant therapies (7 vs 11 months: HR, 2.97; 95% CI, 1.43-6.18; P = .004 after adjustment). In addition, fewer rescue therapy episodes during the first 24 months were required (mean [SD], 0.38 [1.10] vs 1.31 [1.59] times; mean difference, -1.26; 95% CI, -1.97 to -0.56; P < .001 after adjustment), and a larger proportion of patients had minimal or no need of additional immunotherapies (70% vs 35%; OR, 5.47; 95% CI, 1.40-21.43; P = .02 after adjustment). Rates of treatment discontinuation due to adverse events were lower with rituximab compared with conventional therapies (3% vs 46%; P < .001 after adjustment).Conclusions and relevanceClinical outcomes with rituximab appeared to be more favorable in new-onset generalized myasthenia gravis, and rituximab also appeared to perform better than conventional immunosuppressant therapy. These findings suggest a relatively greater benefit of rituximab earlier in the disease course. A placebo-controlled randomized trial to corroborate these findings is warranted.
| S-EPMC7199182 | biostudies-literature
Project description:Background and aimsZenker's diverticulum (ZD) is attributed to a poorly compliant cricopharyngeus muscle, and the mainstay of treatment is a cricopharyngeal myotomy. We present a video series summarizing endoscopic treatment options for ZD and related conditions.MethodsWe review the rationale and key technique for various endoscopic treatment modalities for ZD, cricopharyngeal bar, and other esophageal diverticula.ResultsStandard flexible endoscopic cricopharyngeal myotomy involves the division of the common wall or septum of the ZD, aiming for complete transection of the cricopharyngeus. However, recurrence rates are high, likely owing to incomplete myotomy. Zenker's peroral endoscopic myotomy (Z-POEM) uses a proximal submucosal tunnel to provide direct visualization of the cricopharyngeus and septum, allowing confirmation of complete myotomy. We demonstrate an over-the-septum modification to simplify the technique. Submucosal fibrosis, commonly seen in patients with prior treatment, limits submucosal dissection. We present a hybrid technique to overcome this, whereby a traditional septotomy is performed until submucosal tissue is visualized. The intact mucosal flap after Z-POEM in a large ZD may contribute to residual dysphagia. We propose Z-POEM with mucosotomy for large ZD. Finally, we demonstrate modifications for treatment of other esophageal diseases, including cricopharyngeal bar and non-Zenker's esophageal diverticula.ConclusionEndoscopic treatment options for ZD and related conditions are rapidly expanding. With careful tailoring to individual patient characteristics, our expanding arsenal of options allows effective and safe treatment of a broad spectrum of patients.
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Project description:Nowadays there are at present no efficient therapies for spinal cord injury (SCI), and new approaches have to be proposed. Recently, a new regenerative medicine strategy has been suggested using smart biomaterials able to carry and deliver cells and/or drugs in the damaged spinal cord. Among the wide field of emerging materials, research has been focused on hydrogels, three-dimensional polymeric networks able to swell and absorb a large amount of water. The present paper intends to give an overview of a wide range of natural, synthetic, and composite hydrogels with particular efforts for the ones studied in the last five years. Here, different hydrogel applications are underlined, together with their different nature, in order to have a clearer view of what is happening in one of the most sparkling fields of regenerative medicine.
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Project description:Neural regeneration after spinal cord injury (SCI) closely relates to the microvascular endothelial cell (MEC)-mediated neurovascular unit formation. However, the effects of central nerve system-derived MECs on neovascularization and neurogenesis, and potential signaling involved therein, are unclear. Here, we established a primary spinal cord-derived MECs (SCMECs) isolation with high cell yield and purity to describe the differences with brain-derived MECs (BMECs) and their therapeutic effects on SCI. Transcriptomics and proteomics revealed differentially expressed genes and proteins in SCMECs were involved in angiogenesis, immunity, metabolism, and cell adhesion molecular signaling was the only signaling pathway enriched of top 10 in differentially expressed genes and proteins KEGG analysis. SCMECs and BMECs could be induced angiogenesis by different stiffness stimulation of PEG hydrogels with elastic modulus 50-1650 Pa for SCMECs and 50-300 Pa for BMECs, respectively. Moreover, SCMECs and BMECs promoted spinal cord or brain-derived NSC (SNSC/BNSC) proliferation, migration, and differentiation at different levels. At certain dose, SCMECs in combination with the NeuroRegen scaffold, showed higher effectiveness in the promotion of vascular reconstruction. The potential underlying mechanism of this phenomenon may through VEGF/AKT/eNOS- signaling pathway, and consequently accelerated neuronal regeneration and functional recovery of SCI rats compared to BMECs. Our findings suggested a promising role of SCMECs in restoring vascularization and neural regeneration.
| S-EPMC10444976 | biostudies-literature
Project description:Background and aimsZenker's diverticulum is a false diverticulum through Killian's dehiscence. Symptoms include halitosis, dysphagia, regurgitation, cough, and aspiration pneumonia. Treatment options include open transcervical cricopharyngeal myotomy, trans-oral rigid endoscopic stapling, and minimally invasive endoscopic myotomy. Although open surgical techniques have historically been the criterion standard for treatment, endoscopic options have become increasingly used. We propose the use of flexible endoscopy in the management of Zenker's diverticulum.MethodsWe present a retrospective case series of 9 patients undergoing endoscopic cricopharyngeal myotomy from 2014 to 2018 using our endoscopic technique.ResultsWe demonstrate that endoscopic technique provided adequate symptomatic relief in 7 of 9 patients, with no operative adverse events.ConclusionsCricopharyngeal myotomy using flexible endoscopy is a safe and effective technique for the management of Zenker's diverticulum. Potential benefits of this approach include shorter operative times, shorter postoperative admissions, and earlier progression of diet. Initial treatment with endoscopic technique does not preclude future open repairs.
| S-EPMC6363821 | biostudies-literature
Project description:A high prevalence of sleep-disordered breathing (SDB) after spinal cord injury (SCI) has been reported in the literature; however, the underlying mechanisms are not well understood. We sought to determine the effect of the withdrawal of the wakefulness drive to breathe on the degree of hypoventilation in SCI patients and able-bodied controls. We studied 18 subjects with chronic cervical and thoracic SCI (10 cervical, 8 thoracic SCI; 11 males; age 42.4 ± 17.1 years; body mass index 26.3 ± 4.8 kg/m(2)) and 17 matched able-bodied subjects. Subjects underwent polysomnography, which included quantitative measurement of ventilation, timing, and upper airway resistance (RUA) on a breath-by-breath basis during transitions from wake to stage N1 sleep. Compared to able-bodied controls, SCI subjects had a significantly greater reduction in tidal volume during the transition from wake to N1 sleep (from 0.51 ± 0.21 to 0.32 ± 0.10 L vs. 0.47 ± 0.13 to 0.43 ± 0.12 L; respectively, P < 0.05). Moreover, end-tidal CO2 and end-tidal O2 were significantly altered from wake to sleep in SCI (38.9 ± 2.7 mmHg vs. 40.6 ± 3.4 mmHg; 94.1 ± 7.1 mmHg vs. 91.2 ± 8.3 mmHg; respectively, P < 0.05), but not in able-bodied controls (39.5 ± 3.2 mmHg vs. 39.9 ± 3.2 mmHg; 99.4 ± 5.4 mmHg vs. 98.9 ± 6.1 mmHg; respectively, P = ns). RUA was not significantly altered in either group. In conclusion, individuals with SCI experience hypoventilation at sleep onset, which cannot be explained by upper airway mechanics. Sleep onset hypoventilation may contribute to the development SDB in the SCI population.
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Project description:Spinal cord injury results from an insult inflicted on the spinal cord that usually encompasses its 4 major functions (motor, sensory, autonomic, and reflex). The type of deficits resulting from spinal cord injury arise from primary insult, but their long-term severity is due to a multitude of pathophysiological processes during the secondary phase of injury. The failure of the mammalian spinal cord to regenerate and repair is often attributed to the very feature that makes the central nervous system special-it becomes so highly specialized to perform higher functions that it cannot effectively reactivate developmental programs to re-build novel circuitry to restore function after injury. Added to this is an extensive gliotic and immune response that is essential for clearance of cellular debris, but also lays down many obstacles that are detrimental to regeneration. Here, we discuss how the mature chromatin state of different central nervous system cells (neural, glial, and immune) may contribute to secondary pathophysiology, and how restoring silenced developmental gene expression by altering histone acetylation could stall secondary damage and contribute to novel approaches to stimulate endogenous repair.
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