Role of Myasthenia Gravis Auto-Antibodies as Predictor of Myasthenic Crisis and Clinical Parameters

Background: The cannabinoid system may be involved in the humoral mechanisms at the neuromuscular junction. Ultramicronized-palmitoylethanolamide (μm-PEA) has recently been shown to reduce the desensitization of Acetylcholine (ACh)-evoked currents in denervated patients modifying the stability of ACh receptor (AChR) function. <p> Objective: To analyze the possible beneficial effects of μm-PEA in patients with myasthenia gravis (MG) on muscular fatigue and neurophysiological changes. <p> Method: The duration of this open pilot study, which included an intra-individual control, was three weeks. Each patient was assigned to a 1-week treatment period with μm-PEA 600 mg twice a day. A neurophysiological examination based on repetitive nerve stimulation (RNS) of the masseteric and the axillary nerves was performed, and the quantitative MG (QMG) score was calculated in 22 MG patients every week in a three-week follow-up period. AChR antibody titer was investigated to analyze a possible immunomodulatory effect of PEA in MG patients. <p> Results: PEA had a significant effect on the QMG score (p=0.03418) and on RNS of the masseteric nerve (p=0.01763), thus indicating that PEA reduces the level of disability and decremental muscle response. Antibody titers did not change significantly after treatment. <p> Conclusion: According to our observations, μm-PEA as an add-on therapy could improve muscular response to fatigue in MG. The possible modulation of AChR currents as a means of eliciting a direct effect from PEA on the conformation of ACh receptors should be investigated. The co-role of cytokines also warrants an analysis. Given the rapidity and reversibility of the response, we suppose that PEA acts directly on AChR, though further studies are needed to confirm this hypothesis.

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Helminth Interactions with Bacteria in the Host Gut Are Essential for Its Immunomodulatory Effect

Microorganisms ◽

10.3390/microorganisms9020226 ◽

2021 ◽

Vol 9 (2) ◽

pp. 226

Author(s):

Milan Jirků ◽

Zuzana Lhotská ◽

Lucia Frgelecová ◽

Oldřiška Kadlecová ◽

Klára Judita Petrželková ◽

...

Keyword(s):

Protective Effect ◽

Intestinal Inflammation ◽

Hymenolepis Diminuta ◽

Clinical Parameters ◽

Th2 Response ◽

Histological Changes ◽

Immunological Markers ◽

Bacterial Microbiota ◽

Inflammatory Effect

Colonization by the benign tapeworm, Hymenolepis diminuta, has been associated with a reduction in intestinal inflammation and changes in bacterial microbiota. However, the role of microbiota in the tapeworm anti-inflammatory effect is not yet clear, and the aim of this study was to determine whether disruption of the microflora during worm colonization can affect the course of intestinal inflammation. We added a phase for disrupting the intestinal microbiota using antibiotics to the experimental design for which we previously demonstrated the protective effect of H. diminuta. We monitored the immunological markers, clinical parameters, bacterial microbiota, and histological changes in the colon of rats. After a combination of colonization, antibiotics, and colitis induction, we had four differently affected experimental groups. We observed a different course of the immune response in each group, but no protective effect was found. Rats treated with colonization and antibiotics showed a strong induction of the Th2 response as well as a significant change in microbial diversity. The microbial results also revealed differences in the richness and abundance of some bacterial taxa, influenced by various factors. Our data suggest that interactions between the tapeworm and bacteria may have a major impact on its protective effect.

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CPAP Therapy Reverses Weakness of Myasthenia Gravis: Role of Obstructive Sleep Apnea in Paradoxical Weakness of Myasthenia Gravis

Journal of Clinical Sleep Medicine ◽

10.5664/jcsm.3626 ◽

2014 ◽

Vol 10 (04) ◽

pp. 441-442 ◽

Cited By ~ 3

Author(s):

Ki-Hwan Ji ◽

Jong Seok Bae

Keyword(s):

Obstructive Sleep Apnea ◽

Sleep Apnea ◽

Myasthenia Gravis ◽

Cpap Therapy ◽

Obstructive Sleep

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Myasthenia gravis in children: analysis of 18 patients

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2001000500005 ◽

2001 ◽

Vol 59 (3B) ◽

pp. 681-685 ◽

Cited By ~ 13

Author(s):

Maria da Penha A. Morita ◽

Alberto A. Gabbai ◽

Acary S.B. Oliveira ◽

Audrey S. Penn

Keyword(s):

Myasthenia Gravis ◽

Intravenous Immunoglobulin ◽

Acetylcholine Receptor ◽

Nerve Stimulation ◽

Chest Ct ◽

Clinical Severity ◽

Myasthenic Crisis ◽

Repetitive Nerve Stimulation ◽

Follicular Hyperplasia ◽

Receptor Antibodies

Myasthenia gravis (MG) in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1). Eleven patients (61%) presented moderate or severe generalized disease and 4 (22%) had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47%) out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested) and the levels had no relation to clinical severity. Nine out of 16 patients (56%) worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia). Three patients (75%) improved markedly after thymectomy and one (25%) worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.

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The Diagnostic Validity of Paraphrenia

Australian & New Zealand Journal of Psychiatry ◽

10.3109/00048679209068306 ◽

1992 ◽

Vol 26 (1) ◽

pp. 18-29 ◽

Cited By ~ 4

Author(s):

Anne M. Hassett ◽

Nicholas A. Keks ◽

Henry J. Jackson ◽

David L. Copolov

Keyword(s):

Old Age ◽

Classification Systems ◽

Historical Background ◽

Psychotic Symptoms ◽

Clinical Parameters ◽

Diagnostic Validity ◽

Separate Entity ◽

Distinct Entity ◽

Icd 10

It remains a matter of conjecture as to whether a schizophrenia-like syndrome commencing in old age differs from the early-onset disorder in any substantial way. This article reviews both the historical background to the concept of defining paraphrenia as a distinct entity, as well as the current controversies concerning whether it should remain a separate entity: the latter is important as paraphrenia has not been included in the DSM Ill-R and ICD 10 classification systems. Clinical parameters and aetiological factors relevant to an understanding of the syndrome are discussed under the rubrics of descriptive and construct validity. Of particular importance are the role of cerebral organic factors and the pathoplastic effect of the ageing process and how both interface with the development of psychotic symptoms. Whilst the course of this syndrome has been considered relatively benign, outcome studies have yet to establish consistent features that provide a basis to determine predictive validity.

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Potential role of thymoma and other mediastinal tumors in the pathogenesis of myasthenia gravis

Journal of Neuroimmunology ◽

10.1016/0165-5728(93)90039-2 ◽

1993 ◽

Vol 44 (2) ◽

pp. 171-176 ◽

Cited By ~ 3

Author(s):

Makoto Matsui ◽

Hiromi Wada ◽

Mitsuhiro Ohta ◽

Yasuo Kuroda

Keyword(s):

Myasthenia Gravis ◽

Potential Role ◽

Mediastinal Tumors

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Effective Early Treatment of AChR Antibody-Positive Myasthenia Gravis with Rituximab; the Experience from a Neuroimmunology Clinic in a Developing Country

Journal of Central Nervous System Disease ◽

10.1177/11795735211016080 ◽

2021 ◽

Vol 13 ◽

pp. 117957352110160

Author(s):

Thomas Mathew ◽

Kurian Thomas ◽

Saji K John ◽

Shruthi Venkatesh ◽

Raghunandan Nadig ◽

...

Keyword(s):

Observational Study ◽

Myasthenia Gravis ◽

Treatment Option ◽

Early Treatment ◽

Early Stage ◽

Tertiary Care ◽

Response To Treatment ◽

Myasthenic Crisis ◽

Achr Antibody ◽

Significant Difference

Background: Rituximab is reserved for treating refractory myasthenia gravis (MG) patients. Here we report our experience with rituximab in AChR antibody positive generalized MG (gMG) and impending myasthenic crisis (IMC). Methods: This retrospective, observational study, conducted at a tertiary care, neuroimmunology clinic, analyzed the data of patients with AChR antibody positive gMG, treated with rituximab between 1st January 2016 and 30th October 2018. Results: Eleven patients with AChR antibody positive gMG received rituximab. Mean age of the cohort was 50.54 ± 18.71 years with 9 males. Seven out of 11 patients received rituximab in the early stage (<2 years from onset) and had good response to treatment. Four of the 5 patients with IMC improved with rituximab alone. In the 10 patients who regularly followed up, there was a significant difference between the QMG scores at baseline and at 1, 2, 6, 12, and 18 months ( P < .0001). Conclusion: Rituximab appears to be a potentially effective early treatment option for AChR antibody positive generalized MG and impending myasthenic crisis.

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The Role of Adjuvants in the Efficacy of a Peptide Vaccine for Myasthenia Gravis

Experimental Biology and Medicine ◽

10.1177/153537020122600407 ◽

2001 ◽

Vol 226 (4) ◽

pp. 307-311 ◽

Cited By ~ 8

Author(s):

James L. McAnally ◽

Likang Xu ◽

Matteo Villain ◽

J. Edwin Blalock

Keyword(s):

Myasthenia Gravis ◽

Peptide Vaccine ◽

Cell Epitope ◽

Keyhole Limpet Hemocyanin ◽

Peptide Vaccines ◽

Lewis Rat ◽

Cell Responses ◽

Immunogenic Region ◽

Experimental Autoimmune

Myasthenia gravis (MG) and its animal model, experimental autoimmune (EA) MG, are caused by interference with neuromuscular transmission by autoantibodies against the nicotinic acetylcholine receptor (AChR) on muscle. Previously, we have shown that two peptides, denoted RhCA 67-16 and RhCA 611-001, designed to be complementary in structure to the main immunogenic region and the dominant Lewis rat T cell epitope (α-chain residues 100-116) of the AChR, respectively, are effective vaccines that prevent EAMG in rats by inducing anti-idiotypic/clonotypic antibodies (Ab) and lowering levels of AChR Ab. These studies employed keyhole limpet hemocyanin (KLH) as a carrier and complete Freunds adjuvant (CFA). In advance of a clinical trial the present study tested the efficacy of RhCA 611-001 when combined with different adjuvants that are approved for use in humans. Adjuvants chosen for comparison were incomplete Freunds adjuvant (IFA) and aluminum hydroxide (Alum). As a second goal we evaluated diphtheria toxin (DT) as an alternative carrier protein to KLH. Alum was found to be an effective adjuvant, particularly when used with the peptide conjugated to DT. This combination of carrier and adjuvant provided protection against EAMG comparable with that observed with CFA and KLH. Using enzyme-linked immunosorbent assays for Ab against RhCA 611-001, it was found that disease protection is qualitatively, but not quantitatively, related to the anti-peptide Ab response. Our results demonstrate a vaccine formulation that should be useful in the first soon-to-be-conducted clinical trials of peptide vaccines to specifically correct aberrant T and B cell responses in an autoimmune disease.

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