Castleman disease presenting with pseudotumour cerebri and myasthenia gravis: A case report and literature review
ConclusionThe occurrence of acute or subacute descending paralysis with involvement of bulbar muscles and respiratory failure can often divert clinicians to a diagnosis of neuromuscular junction disorders (such as botulism or myasthenia gravis), vascular causes like stroke, or electrolyte and metabolic abnormalities. Early identification of Miller Fisher syndrome with appropriate testing is essential to prompt treatment and prevention of further, potentially fatal, deterioration.
In myasthenia gravis (MG) patients on intravenous methylprednisolone (IVMP) therapy, initial deterioration should be carefully monitored because it may cause myasthenic crisis. The aim of this study was to investigate the onset, duration and related factors of initial deterioration from the first IVMP in MG patients.
Interview with James F. Howard, MD, author of Clinical Effects of Self-administered Subcutaneous Zilucoplan in Patients with Moderate to Severe Generalized Myasthenia Gravis: Results of a Phase 2 Randomized, Double-Blind, Placebo-Controlled, Multicenter Clinical Trial
Discussion Myasthenia gravis (MG) is a problem of the neuromuscular junction which causes muscle weakness. It can occur in all ages and have a range of symptoms from mild localized disease to mortality-threatening respiratory failure. MG occurs in 1.7-30 cases/million, with a prevalence of 77.7 cases/million. Pediatric patients comprise 10-15% of all patients with MG. In various Asian populations, the juvenile MG can be up to 50% of all of the MG cases. Fluctuations in muscle weakness is a hallmark of the disease. As a reminder, “[i]n normal synaptic transmission in the neuromuscular junction, the axon is depolarize...
This randomized clinical trial evaluates the clinical effects of zilucoplan, a subcutaneously self-administered macrocyclic peptide inhibitor of complement component 5, in a broad population of patients with moderate to severe generalized myasthenia gravis.
Authors: Yuan JL, Xing Y, Hu WL PMID: 32051728 [PubMed]
Authors: Aoki S, Nagashima K, Furuta M, Makioka K, Fujita Y, Saito K, Kashima T, Nakajima N, Ikota H, Higuchi O, Ikeda Y Abstract We herein report the case of a 65-year-old woman diagnosed with myasthenia gravis (MG) after complaining of double vision. The patient had anti-low-density lipoprotein receptor-related protein 4 (LRP4) antibody in her serum, although antibodies against the acetylcholine receptor and muscle-specific tyrosine kinase were not detected. Chest computed tomography showed an anterior mediastinal tumor with a high uptake on fluorodeoxyglucose-positron emission tomography. Endoscopic thymectomy s...
In conclusion we redefined the clinical spectrum of muscle involvement in MG-IM association, which represent a continuum among 3 main clinical groups: distal, proximal and subclinical muscle involvement. Minimal muscle involvement and focal myositis could be underestimated among myasthenic patients and early aggressive immunotherapy could be required in focal group.Graphical abstract
CONCLUSIONS These results indicate that grilled nux vomica ameliorates EAMG by depressing the TLR-4/NF-kappaB signaling pathway, and hub genes may serve as potential targets for MG treatment. PMID: 32052794 [PubMed - in process]
AbstractLambert ‐Eaton myasthenic syndrome (LEMS) is an autoimmune neuromuscular junction disease associated with P/Q‐type voltage‐gated calcium channels (P/Q‐type VGCCs) autoantibodies1. P/Q ‐type VGCCs present at the presynaptic motor nerve terminals and their antibodies induce a reduction in neurotransmitter release, leading to the characteristic muscle weakness associated with the disease. Approximately half of LEMS patients have small‐cell lung carcinoma (SCLC) that occurs as a paraneoplastic disorder.