Predictive factors of myasthenic crisis after extended thymectomy for patients with myasthenia gravis

Absztrakt Célkitűzés: A myasthenia gravis (MG) kezelésében számos nyitott, illetve minimálisan invazív thymectomia ismert. A tanulmány ugyanazon intézeten belül a transsternalis (TS), illetve kétféle minimálisan invazív thymectomia (video-assisted thoracoscopic extended thymectomy – VATET; unilateral video-assisted thoracoscopic surgery – UL-VATS) eredményeit hasonlítja össze. Anyag és módszerek: Három különböző időintervallumban 71 betegnél történt thymectomia MG miatt (60 nő, 11 férfi): 23 transsternalis thymectomia (1995. január–2004. szeptember), 22 VATET (2004. szeptember – 2009. augusztus) és 26 UL-VATS thymectomia (2009. szeptember – 2011. december). Az eredmények értékelésénél a műtéti idő, MG-hez társuló neurológiai és a műtét utáni sebészi szövődmények, valamint az MG státuszában az egyéves utánkövetéskor észlelt neurológiai változások szerepeltek. Eredmények: Perioperatív mortalitás nem fordult elő. A műtéti idő 112, 211, 116 perc (p = 0,001), a kórházi napok száma: 8,9, 5,6 és 4 nap (p = 0,001) volt a TS-, VATET- és UL-VATS-csoportban. Az MG-hez kapcsolódó postoperativ neurológiai szövődmények 21,7%, 18,2% és 7,7% (p = 0,365) értékeket mutattak. A sebészi szövődmény 4,3%, 13,7%, 0% (p = 0,118) volt. Az MG tüneteinek javulása 91,3%, 94,7%, 87,5% (p = 0,712), míg komplett remisszió 13%, 10,5%, 11,5% (p = 0,917) volt a TS-, VATET- és UL-VATS-csoportokban. Következtetések: A műtéti idő, valamint a kórházban eltöltött napok száma UL-VATS esetében volt a legrövidebb. A kisebb sebészi beavatkozáshoz alacsonyabb sebészi, illetve MG-s neurológiai szövődmények társultak. Az MG-tünetek javulásában mindhárom módszernél kiváló eredményt értek el.

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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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Predictive factors of prolonged mechanical ventilation, overall survival, and quality of life in patients with post-thymectomy myasthenic crisis

World Journal of Surgical Oncology ◽

10.1186/s12957-017-1209-1 ◽

2017 ◽

Vol 15 (1) ◽

Cited By ~ 4

Author(s):

Kun-Kun Li ◽

Kai Qian ◽

Yong-Geng Feng ◽

Wei Guo ◽

Qun-You Tan ◽

...

Keyword(s):

Quality Of Life ◽

Mechanical Ventilation ◽

Overall Survival ◽

Predictive Factors ◽

Prolonged Mechanical Ventilation ◽

Myasthenic Crisis

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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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Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2004000300003 ◽

2004 ◽

Vol 62 (2b) ◽

pp. 391-395 ◽

Cited By ~ 9

Author(s):

Rosana Carandina-Maffeis ◽

Anamarli Nucci ◽

José F.C. Marques Jr ◽

Eduardo G. Roveri ◽

Beatriz H.M. Pfeilsticker ◽

...

Keyword(s):

Retrospective Study ◽

Side Effects ◽

Myasthenia Gravis ◽

Plasma Exchange ◽

Mortality Rates ◽

Motor Deficit ◽

Myasthenic Crisis ◽

Clinical Hospital ◽

The Mean ◽

Mean Time

We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE) therapy in myasthenia gravis (MG). About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G) or IIIa (MGFA scale). One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD ± 1.4) each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD ± 35.2); median 100.5 (extremes of 55 and 215). The mean volume of plasma exchanged in each session was 2396 ml (SD ± 561); median 2225 (extremes 1512 and 4500). Side effects occurred: reversible hypotension (seven cases), mild tremor or paresthesias (seven cases). Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.

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