scholarly journals Impact of Different Thymic Pathologies on the Clinical Course of Myasthenia Gravis: A Population Wide Study in Latvia / Aizkrūts Dziedzera Patoloģiju Ietekmeu uz Myasthenia Gravis Klīnisko Gaitu: Populācijas Pētījums Latvijā

Author(s):  
Anete Zieda ◽  
Kristīne Rāviņa ◽  
Lelde Pelčere ◽  
Maruta S. Naudiņa ◽  
Inese Kamša ◽  
...  

Abstract Myasthenia gravis (MG) is an autoimmune disease characterized by autoantibody mediated postsynaptic failure of neuromuscular transmission. The thymus gland has a role in the pathogenesis of MG. The aim of this study was to determine (1) the prevalence of different thymic pathologies in the MG patient population of Latvia; (2) the potential impact of different thymus pathologies on the clinical course of MG; and (3) the effectiveness of surgical treatment of thymic pathologies in Latvia. The results showed that the most common thymus pathology among the patients with MG in Latvia is thymoma. Compared to the published data, the proportion of MG patients diagnosed with thymus hyperplasia in Latvia is very small. Thymus hyperplasia is uncommon among patients 60 years of age and older. MG onset for patients with thymoma is significantly later than that of patients with thymus hyperplasia. Almost all patients with thymoma or thymus hyperplasia develop a generalised form of MG. The presence of a thymus pathology does not significantly affect the electrophysiological test results, clinical symptom severity, frequency of disability or hospitalisation of the patients with MG. Thymectomy in patients with thymoma or thymus hyperplasia has no significant effect on relieving MG symptoms

1995 ◽  
Vol 34 (03) ◽  
pp. 302-308 ◽  
Author(s):  
C. J. Luz ◽  
W. Giere ◽  
R. Lüdecke ◽  
D. Jonas ◽  
A. J. W. Goldschmidt

Abstract:The illustration of a patient’s history by a graphical primitive is discussed. Illustration technology is presented which simultaneously represents quantitative examination findings (e. g., laboratory values) and qualitative findings (e. g., from function diagnostics) by a single geometrical figure. Depending on the medical results, this figure takes on characteristic forms which can be identified as patterns typical for a specific disease. The procedure developed is integrated in a user interface which is implemented in the form of a computerized medical record for use on a pentop computer. This portable computer assists the physician during ward rounds, supplies additional, intelligence-based information, serves quality control, and streamlines working procedures making them more efficient.


2021 ◽  
Vol 11 (8) ◽  
pp. 1035
Author(s):  
Maria Pia Giannoccaro ◽  
Patrizia Avoni ◽  
Rocco Liguori

The neuromuscular junction (NMJ) is the target of a variety of immune-mediated disorders, usually classified as presynaptic and postsynaptic, according to the site of the antigenic target and consequently of the neuromuscular transmission alteration. Although less common than the classical autoimmune postsynaptic myasthenia gravis, presynaptic disorders are important to recognize due to the frequent association with cancer. Lambert Eaton myasthenic syndrome is due to a presynaptic failure to release acetylcholine, caused by antibodies to the presynaptic voltage-gated calcium channels. Acquired neuromyotonia is a condition characterized by nerve hyperexcitability often due to the presence of antibodies against proteins associated with voltage-gated potassium channels. This review will focus on the recent developments in the autoimmune presynaptic disorders of the NMJ.


2020 ◽  
Vol 6 (4) ◽  
pp. 201
Author(s):  
Lívia S. Ramos ◽  
Laura N. Silva ◽  
Marta H. Branquinha ◽  
André L. S. Santos

Candida haemulonii complex (C. haemulonii, C. duobushaemulonii and C. haemulonii var. vulnera) is well-known for its resistance profile to different available antifungal drugs. Although echinocandins are the most effective class of antifungal compounds against the C. haemulonii species complex, clinical isolates resistant to caspofungin, micafungin and anidulafungin have already been reported. In this work, we present a literature review regarding the effects of echinocandins on this emergent fungal complex. Published data has revealed that micafungin and anidulafungin were more effective than caspofungin against the species forming the C. haemulonii complex. Subsequently, we investigated the susceptibilities of both planktonic and biofilm forms of 12 Brazilian clinical isolates of the C. haemulonii complex towards caspofungin and micafungin (anidulafungin was unavailable). The planktonic cells of all the fungal isolates were susceptible to both of the test echinocandins. Interestingly, echinocandins caused a significant reduction in the biofilm metabolic activity (viability) of almost all fungal isolates (11/12, 91.7%). Generally, the biofilm biomasses were also affected (reduction range 20–60%) upon exposure to caspofungin and micafungin. This is the first report of the anti-biofilm action of echinocandins against the multidrug-resistant opportunistic pathogens comprising the C. haemulonii complex, and unveils the therapeutic potential of these compounds.


2004 ◽  
Vol 101 (43) ◽  
pp. 15464-15469 ◽  
Author(s):  
C. Vandiedonck ◽  
G. Beaurain ◽  
M. Giraud ◽  
C. Hue-Beauvais ◽  
B. Eymard ◽  
...  

2012 ◽  
Vol 245 (1-2) ◽  
pp. 75-78 ◽  
Author(s):  
Shuuichi Mori ◽  
Masahiko Kishi ◽  
Sachiho Kubo ◽  
Takuyu Akiyoshi ◽  
Shigeru Yamada ◽  
...  

1976 ◽  
Vol 144 (3) ◽  
pp. 739-753 ◽  
Author(s):  
J M Lindstrom ◽  
A G Engel ◽  
M E Seybold ◽  
V A Lennon ◽  
E H Lambert

Passive transfer of experimental autoimmune myasthenia gravis (EAMG) was achieved using the gamma globulin fraction and purified IgG from sera of rats immunized with Electrophus electricus (eel) acetylcholine receptor (AChR). This demonstrates the critical role of anti-AChR antibodies in impairing neuromuscular transmission in EAMG. Passive transfer of anti-AChR antibodies from rats with chronic EAMG induced signs of the acute phase of EAMG in normal recipient rats, including invasion of the motor end-plate region by mononuclear inflammatory cells. Clinical, eletrophysiological, histological, and biochemical signs of acute EAMG were observed by 24 h after antibody transfer. Recipient rats developed profound weakness and fatigability, and the posture characteristic of EAMG. Striking weight loss was attributable to dehydration. Recipient rats showed large decreases in amplitude of muscle responses to motor nerve stimulation, and repetitive nerve stimulation induced characteristic decrementing responses. End-plate potentials were not detectable in many muscle fibers, and the amplitudes of miniature end-plate potentials were reduced in the others. Passively transferred EAMG more severely affected the forearm muscles than diaphragm muscles, though neuromuscular transmission was impaired and curare sensitivity was increased in both muscles. Some AChR extracted from the muscles of rats with passively transferred EAMG was found to be complexed with antibody, and the total yield of AChR per rat was decreased. The quantitative decrease in AChR approximately paralleled in time the course of clinical and electrophysiological signs. The amount of AChR increased to normal levels and beyond at the time neuromuscular transmission was improving. The excess of AChR extractable from muscle as the serum antibody level decreased probably represented extrajunctional receptors formed in response to functional denervation caused by phagocytosis of the postsynaptic membrane by macrophages. The amount of antibody required to passively transfer EAMG was less than required to bind all AChR molecules in a rat's musculature. The effectiveness of samll amounts of antibody was probably amplified by the activation of complement and by the destruction of large areas of postsynaptic membrane by phagocytic cells. A self-sustaining autoimmune response to AChR was not provoked in animals with passively transferred EAMG.


2018 ◽  
Vol 50 (3) ◽  
pp. 229-235 ◽  
Author(s):  
Suresh Bokoliya ◽  
Shripad Patil ◽  
Madhu Nagappa ◽  
Arun Taly

AbstractObjectiveTo assess the practicality of dot-blot testing for rapid and sensitive detection of the antiacetylcholine receptor (anti-AChR) antibodies in myasthenia gravis (MG).MethodsIn this case-control study, we tested serum specimens of 85 patients with MG, 85 healthy control individuals, and 85 patients without MG who have other autoimmune and neurological illnesses. All the serum specimens were tested for anti-AChR antibodies using 3 assays: in-house enzyme-linked immunosorbent assay (ELISA), the dot-blot assay, and commercial ELISA.ResultsIn-house ELISA, commercial ELISA, and dot-blot test results were positive for anti-AChR antibodies in 65 (76.5%) patients with MG. The results of all 3 tests were negative for anti-AChR antibodies in healthy controls and patients without MG. We observed perfect concordance (K = 1, P <.001) between all 3 tests. In-house ELISA correlated significantly (r = 0.873, P <.001) with commercial ELISA. In-house ELISA and the dot-blot test demonstrated similar diagnostic performance in detecting anti-AChR antibodies.ConclusionsThe dot-blot assay is a simple, nonradioactive immune assay for rapid detection of anti-AChR antibodies in MG.


2021 ◽  
Vol 11 (11) ◽  
pp. 1444
Author(s):  
Mattia Fonderico ◽  
Emilio Portaccio ◽  
Lorenzo Razzolini ◽  
Luisa Pastò ◽  
Angelo Bellinvia ◽  
...  

The presence of intrathecal IgM synthesis (ITMS) has been associated with an aggressive multiple sclerosis (MS) clinical course. In the present systematic review, we aimed at assessing the prevalence of ITMS among different MS phenotypes. Moreover, we aimed at quantifying the risk of a second relapse in ITMS positive and oligoclonal IgG bands (OCGBs)-positive patients. We selected clinical studies reporting the ITMS prevalence assessed as oligoclonal IgM Bands (OCMBs), lipid-specific OCMBs (LS-OCMBs), and/or as an intrathecal IgM production > 0% (IgMLoc, Reiber formula). The overall prevalence of ITMS was higher in relapsing-remitting (RR) than clinically isolated syndrome (CIS) patients (40.1% versus 23.8%, p < 0.00001), while was in line with that detected in primary progressive MS (PPMS, 26.7%). Almost all patients (98%) with ITMS had also OCGBs. The risk of having a second relapse was higher in OCGBs positive patients (HR = 2.18, p = 0.007) but much higher in ITMS positive patients (HR = 3.62, p = 0.0005). This study revealed that the prevalence of ITMS is higher in RRMS patients. It suggests that the risk of having a second relapse, previously ascribed to OCGBs, may, to a certain extent, be related to the presence of intrathecal IgM.


2020 ◽  
Vol 12 (3) ◽  
pp. 65-70
Author(s):  
Kenneth Chua ◽  
Sakumura J

Vestibular Assessment in patients with Myasthenia Gravis (MG) is challenging, as diagnostic evaluation requires good recording of eye movements. Reports on Vestibular Function Testing (VFT) in MG patients have been scant and it is arguable that VFT will have little clinical value in the MG population. A 75-year-old man, with late onset acquired autoimmune MG presented with dizziness for evaluation. He completed VFT with no significant abnormalities in all tests and was elated to have vestibular ruled out as a contributing factor to his dizziness and imbalance. However, his functional impairments were still addressed and managed regardless of the test results. MG is a heterogenous condition that may be well-controlled with treatment. Patients with dizziness can still be diagnostically evaluated to rule in or out a vestibular involvement and should not be precluded from VFT. Patients should also be assessed for their functional impairments and not based on symptom checklist and objective test results alone. Hence, patients with normal VFT results can still benefit from a hybrid of vestibular rehabilitation therapy (VRT) with focus on habituation.


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