scholarly journals Colon Adenoma Implicating Myasthenia Gravis: A Case Report of a Patient with Postcolectomy Complications

2016 ◽  
Vol 2016 ◽  
pp. 1-2
Author(s):  
Y. Papachatzakis ◽  
E. Tseliou ◽  
I. Tatouli ◽  
I. Dialoupi ◽  
F. Michas ◽  
...  
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Plasma Exchange ◽  
Bowel Obstruction ◽  
Surgical Repair ◽  
Myasthenic Crisis ◽  
Colon Adenoma ◽  
Bulbar Symptoms ◽  
Anastomosis Leakage ◽  
Receptor Antibodies

We report the case of a 63-year-old patient with myasthenia gravis (MG) due to acetylcholine receptor antibodies (AChR) who underwent colectomy due to colon adenoma and developed myasthenic crisis and anastomosis leakage after surgery. The patient underwent two plasma exchanges, 4 and 6 days preoperatively, and received intravenous prednisolone and immunoglobulin infusion due to the crisis, which included primarily bulbar symptoms. The patient developed on the 10th postoperative day bowel obstruction symptoms and anastomosis leakage which required surgical repair and ileostomy. Bowel obstruction occurred in a patient with AChR related myasthenia after plasma exchange and during immunosuppression although it is more commonly reported in patients with thymoma related myasthenia.

Nivolumab-related myasthenia gravis with myositis and myocarditis in Japan

Neurology ◽  
2017 ◽  
Vol 89 (11) ◽  
pp. 1127-1134 ◽  
Author(s):  
Shigeaki Suzuki ◽  
Nobuhisa Ishikawa ◽  
Fumie Konoeda ◽  
Nobuhiko Seki ◽  
Satoshi Fukushima ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitors ◽  
Checkpoint Inhibitors ◽  
Control Group ◽  
Myasthenic Crisis ◽  
Daily Living Activity ◽  
Patients With Cancer ◽  
Bulbar Symptoms ◽  
Receptor Antibodies

Objective:To report the clinical features of myasthenia gravis (MG) induced by treatment with immune checkpoint inhibitors using 2-year safety databases based on postmarketing surveys in Japan.Methods:We studied 10,277 patients with cancer who had received monotherapy with either nivolumab or ipilimumab between September 2014 and August 2016. As the control group, 105 patients with idiopathic MG were used.Results:There were 12 MG cases (0.12%) among 9,869 patients with cancer who had been treated with nivolumab, but none among 408 patients treated with ipilimumab. These 12 patients included 6 men and 6 women with a mean age of 73.5 ± 6.3 years. MG onset occurred in the early phase after nivolumab treatment and rapidly deteriorated. Nivolumab-related MG (nivoMG) included 4 patients with mild involvement and 8 patients with severe involvement. Bulbar symptoms and myasthenic crisis were observed more frequently in nivoMG than idiopathic MG. Ten patients were positive for anti–acetylcholine receptor antibodies. Serum creatine kinase levels were markedly elevated to an average level of 4,799 IU/L. Among the 12 patients with nivoMG, 4 had myositis and 3 had myocarditis, with 1 of these patients having both. Immunosuppressive therapy was effective. Postintervention status showed that pharmacologic remission or minimal manifestations were obtained in 4 patients; however, 2 patients died. Immune-related adverse events triggered by nivolumab impaired the patients' daily living activity.Conclusions:The prompt and correct recognition of MG following treatment with immune checkpoint inhibitors in patients with cancer is important.

scholarly journals Myasthenia gravis in children: analysis of 18 patients

2001 ◽  
Vol 59 (3B) ◽  
pp. 681-685 ◽  
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.

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

2004 ◽  
Vol 62 (2b) ◽  
pp. 391-395 ◽  
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.

scholarly journals Familial Autoimmune Myasthenia Gravis: Four Patients Involving Three Generations

2000 ◽  
Vol 27 (4) ◽  
pp. 307-310 ◽  
Author(s):  
R.A. Marrie ◽  
D.J. Sahlas ◽  
G.M. Bray
Keyword(s):  
Myasthenia Gravis ◽  
Medical Records ◽  
Index Case ◽  
Family Members ◽  
Monozygotic Twins ◽  
Bulbar Symptoms ◽  
History Of ◽  
Autoimmune Myasthenia ◽  
Successive Generations ◽  
Receptor Antibodies

Background:Familial autoimmune myasthenia gravis (MG) is rare, although a genetic role for the development of autoimmune MG is suggested by concordance in monozygotic twins and the increased frequency of other autoimmune diseases in family members of myasthenics.Methods:A patient with a family history of MG was evaluated in hospital. Relatives were interviewed and medical records examined for details regarding the diagnosis of MG in three other family members.Results:The index case first experienced symptoms of MG at age 75 years. She developed generalized MG and required corticosteroids and immunosuppressive therapy to control her disease. Her father developed predominantly bulbar symptoms of MG at age 75 years. He died of complications experienced following a gastrostomy placed for continued difficulty swallowing. His brother developed similar symptoms of MG in his early 60s and died shortly after thymectomy. A 46-year-old nephew of the index case is also beginning to exhibit signs of generalized MG. Acetylcholine receptor antibodies were strongly positive in the index case and her nephew. (The assay was not available for her father and uncle).Conclusion:Four individuals in three successive generations had diagnoses of autoimmune MG. Study of familial cases such as these may clarify the contribution of genetic factors to the development of this disease.

scholarly journals Myasthenia Gravis and Stroke in the Setting of Giant Cell Arteritis

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Elli-Sophia Tripodaki ◽  
Sotirios Kakavas ◽  
Ioanna Skrapari ◽  
Dimitrios Michas ◽  
Giorgios Katsikas ◽  
...  
Keyword(s):  
Case Report ◽  
Myasthenia Gravis ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Inflammatory Markers ◽  
Low Grade ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Laboratory Findings ◽  
Receptor Antibodies

This case report concerns the diagnosis of two independent chronic diseases in a patient hospitalized for stroke, myasthenia gravis (MG) and giant cell arteritis (GCA). MG has been found to be associated with several diseases, but there are very few cases documenting its coexistence with GCA. We report the case of a 79-year-old woman initially hospitalized for stroke. Patient’s concurrent symptoms of blepharoptosis, dysphagia, and proximal muscle weakness were strongly suggestive of myasthenia gravis. The persistent low-grade fever and elevated inflammatory markers in combination with the visual deterioration that developed also raised the suspicion of GCA. Histological examination confirmed GCA, while muscle acetylcholine receptor antibodies were also present. Even though in medicine one strives to interpret a patient’s symptoms with one diagnosis, when one entity cannot fully interpret the clinical and laboratory findings, clinicians must consider the possibility of a second coexisting illness.

scholarly journals Two patients with a neuroendocrine tumour of the small intestine and paraneoplastic myasthenia gravis

2014 ◽  
Vol 2014 ◽  
Author(s):  
M A W Hermans ◽  
B M L Stelten ◽  
H R Haak ◽  
W W de Herder ◽  
M W Dercksen
Keyword(s):  
Small Intestine ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Muscle Weakness ◽  
Neuroendocrine Tumour ◽  
Stage Iv ◽  
List Type ◽  
Bulbar Symptoms ◽  
Receptor Antibodies ◽  
Learning Points

Summary This paper reports on two patients with a long-standing diagnosis of an ENETS stage IV neuroendocrine tumour (NET) of the small intestine who developed neurological symptoms. The first patient only had bulbar symptoms and tested positive for acetylcholine receptor antibodies. The second patient had more classical symptoms of fatigable diplopia and muscle weakness of the legs, but no detectable antibodies. The diagnosis of paraneoplastical myasthenia gravis (MG) was postulated. Both patients were treated with pyridostigmine for MG and octreotide for the NETs. Interestingly, treatment of the NETs resulted in improvement of myasthenic symptoms. Paraneoplastic MG has been described to occur with certain malignancies, mainly thymoma. Herein, we prove that the association with gastrointestinal NETs, however, rare, is also one to be considered by clinicians dealing with either of these diseases. The pathogenesis has yet to be elucidated. Learning points NETs are rare malignancies with a wide variety of symptoms. Paraneoplastic MG can occur with various types of malignancies. Herein, we provide evidence of paraneoplastic MG in association with a grade IV NET of the small intestine. Treatment of the NETs resulted in remission of myasthenic symptoms in one patient.

Bochdalek Hernia in a Soldier

2006 ◽  
Vol 92 (2) ◽  
pp. 75-77
Author(s):  
D. J. Connor
Keyword(s):  
Case Report ◽  
Epidural Analgesia ◽  
Bowel Obstruction ◽  
Surgical Repair ◽  
Military Hospital ◽  
Bochdalek Hernia ◽  
Diaphragmatic Hernias ◽  
Respiratory Embarrassment

AbstractCongenital diaphragmatic hernias are most often seen in infants. They may rarely be discovered in adulthood. This case report describes the management of a soldier presenting with acute bowel obstruction and respiratory embarrassment in a military hospital. The surgical repair was achieved with a laparotomy. Effective epidural analgesia and early physiotherapy were axiomatic of his un-complicated post-operative course.

Late-onset double-seronegative myasthenia gravis syndrome and myasthenic crisis due to nivolumab use for Hodgkin’s lymphoma

2020 ◽  
pp. 107815522097679
Author(s):  
Thai Dang ◽  
Samir Macwan ◽  
Constantin A Dasanu
Keyword(s):  
Myasthenia Gravis ◽  
Late Onset ◽  
Checkpoint Inhibitors ◽  
Myasthenic Crisis ◽  
Immune Mediated ◽  
Seronegative Myasthenia Gravis ◽  
Programmed Cell Death 1 ◽  
Bulbar Symptoms ◽  
Checkpoint Inhibitor Therapy ◽  
Grade 3

Introduction Insofar, use of programmed cell death-1 (PD-1) immune checkpoint inhibitors in oncology has been linked with several immune-mediated neurologic effects. However, grade 3 to 4 adverse events such as myasthenic crisis have been vanishingly rare. Case presentation: We present herein a unique patient with Hodgkin lymphoma who developed late-onset double-seronegative myasthenia gravis syndrome followed by myasthenic crisis after 16 weeks of therapy with nivolumab. One day prior to this event, she developed ptosis, diplopia, bulbar symptoms of dysphagia, dysarthria, orthopnea as well as extremity weakness. She required intubation, mechanical ventilation, plasmapheresis and steroid therapy. Management and outcome: She gradually achieved a near-complete resolution of neurologic symptoms over the next several weeks. On a follow-up visit eight weeks later, she only has some residual diplopia. Restaging scans showed a continued decrease in size of the mediastinal mass, without abnormal uptake. She remains on prednisone 10 mg orally daily. Discussion Prompt recognition of this rare phenomenon, immediate discontinuation of checkpoint inhibitor therapy and subsequent management with immunosuppressive therapy are necessary steps in order to minimize the considerable rates of morbidity and mortality.

Myasthenic crisis demanding mechanical ventilation

Neurology ◽  
2019 ◽  
Vol 94 (3) ◽  
pp. e299-e313 ◽  
Author(s):  
Bernhard Neumann ◽  
Klemens Angstwurm ◽  
Philipp Mergenthaler ◽  
Siegfried Kohler ◽  
Silvia Schönenberger ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Myasthenia Gravis ◽  
Plasma Exchange ◽  
Noninvasive Ventilation ◽  
Late Onset ◽  
Multiorgan Failure ◽  
Myasthenic Crisis ◽  
Treatment Regimens ◽  
First Line Therapy ◽  
Discharge From Hospital

ObjectiveTo determine demographic characteristics, clinical features, treatment regimens, and outcome of myasthenic crisis (MC) requiring mechanical ventilation (MV).MethodsAnalysis of patients who presented with MC between 2006 and 2015 in a German multicenter retrospective study.ResultsWe identified 250 cases in 12 participating centers. Median age at crisis was 72 years. Median duration of MV was 12 days. Prolonged ventilation (>15 days) depended on age (p = 0.0001), late-onset myasthenia gravis (MG), a high Myasthenia Gravis Foundation of America Class before crisis (p = 0.0001 for IVb, odds ratio [OR] = infinite), number of comorbidities (>3 comorbidities: p = 0.002, OR 2.99), pneumonia (p = 0.0001, OR 3.13), and resuscitation (p = 0.0008, OR 9.15). MV at discharge from hospital was necessary in 20.5% of survivors. Patients with early-onset MG (p = 0.0001, OR 0.21), thymus hyperplasia (p = 0.002, OR 0), and successful noninvasive ventilation trial were more likely to be ventilated for less than 15 days. Noninvasive ventilation in 92 cases was sufficient in 38%, which was accompanied by a significantly shorter duration of ventilation (p = 0.001) and intensive care unit (ICU) stay (p = 0.01). IV immunoglobulins, plasma exchange, and immunoadsorption were more likely to be combined sequentially if the duration of MV and the stay in an ICU extended (p = 0.0503, OR 2.05). Patients who received plasma exchange or immunoadsorption as first-line therapy needed invasive ventilation significantly less often (p = 0.003). In-hospital mortality was 12%, which was significantly associated with the number of comorbidities (>3) and complications such as acute respiratory distress syndrome and resuscitation. Main cause of death was multiorgan failure, mostly due to sepsis.ConclusionMortality and duration of MC remained comparable to previous reports despite higher age and a high disease burden in our study. Prevention and treatment of complications and specialized neurointensive care are the cornerstones in order to improve outcome.

scholarly journals Assessment of the risks of a myasthenic crisis after thymectomy in patients with myasthenia gravis: a systematic review and meta-analysis of 25 studies

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Chaoying Liu ◽  
Peng Liu ◽  
Xiao jing Zhang ◽  
Wen qian Li ◽  
Guoyan Qi
Keyword(s):  
Risk Factors ◽  
Blood Loss ◽  
Myasthenia Gravis ◽  
Who Classification ◽  
Meta Analysis ◽  
Lung Infection ◽  
Myasthenic Crisis ◽  
Bulbar Symptoms ◽  
History Of ◽  
Meta Analyses

Abstract Background Despite the burgeoning literature describing preoperative and postoperative risks of a myasthenic crisis after thymectomy (MCAT) in patients with myasthenia gravis, substantial differences exist in the risk factors identified by previous studies. We conducted a meta-analysis to assess the reported risk factors and MCAT risk. Methods We collected relevant studies on the risk factors for MCAT by searching the PubMed, Embase, The Cochrane Library, China Biology Medicine (CBM), WanFang Data, VIP and CNKI databases. The search period ranged from the establishment of the database to November 2019. Results Twenty-five of the 458 identified studies were eligible for the meta-analysis. Seven retrospective cohort studies and 18 case-control studies were included, and 14 risk factors for MCAT were extracted. Meta-analyses of the association between MCAT and risk factors related to the patient’s preoperative condition included a preoperative history of MC, preoperative bulbar symptoms, IIa + IIb + III + VI, IIb + III + VI, VI + V, dosage of pyridostigmine bromide prior to the operation, a preoperative AchR-Ab level > 100 (nm/L), preoperative pulmonary function, preoperative complications, and preoperative disease course. Meta-analyses of the association between MCAT and surgery-related risk factors included intraoperative blood loss > 1000 mL and the mode of operation. Meta-analyses of the association between MCAT and postoperative risk factors included postoperative lung infection, thymoma and the WHO classification. The operation time was not an independent risk factor for MCAT. Conclusions The independent risk factors for MCAT were a preoperative history of MC, preoperative bulbar symptoms, preoperative MG Osserman stage, preoperative dosage of pyridostigmine bromide, preoperative serum AchR-Ab level, lung function, major postoperative complications, disease duration before thymectomy, blood loss, thoracotomy, postoperative lung infection, thymoma, and WHO classification.

Sign in / Sign up

Export Citation Format

Share Document