Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis

Qureshi, Adnan I; Choudhry, Mashkoor A.; Akbar, Muhammad Shoaib; Mohammad, Yaseen; Chua, H C; Yahia, Abutaher M.; Ulatowski, John A.; Krendel, David A.; Leshner, Robert T.

doi:10.1212/wnl.52.3.629

Cited by 167 publications

(126 citation statements)

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“…Patients often qualify for thymectomy, which improves the prognosis and long-term treatment outcomes [5,8,9]. The histopathological examinations usually reveal thymus hyperplasia; thymoma is observed less frequently [9].…”

Section: Discussionmentioning

confidence: 99%

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Postępowanie u dziecka w przełomie miastenicznym

Rybojad¹,

Lesiuk²,

Fijałkowska-Nestorowicz³

et al. 2013

Anaesthesiol Intensive Ther

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Myasthenia gravis is an autoimmune disorder of peripheral nervous system, leading to fluctuating muscle weakness. It is caused by circulating antibodies that block acetylcholine nicotinic postsynaptic receptors at the postsynaptic neuromuscular junction. Myasthenic crisis is a life-threatening complication, which is defined as weakness from acquired myasthenia gravis. In this paper we described a 15-year-old boy who was admitted to the Paediatric Intensive Care Unit due to myasthenic crisis. He had suffered not only from myasthenia gravis but also hypothyroidism, cerebral palsy and epilepsy. The patient required mechanical ventilation and was successfully treated with both plasmapheresis and intravenous immunoglobulins. He recovered from the crisis and then thymectomy was performed. Perioperative period and anaesthesia passed uncomplicated. Discharged home from the hospital after 2.5 month-treatment, for the last 4 years, he has only come on scheduled outpatient medical appointments. This case reveals that myasthenic crisis, albeit rare, may occur in male adolescents. In such cases multidisciplinary care followed by surgery becomes a procedure of choice. Concomitant medical problems, if well controlled, do not affect the results of outcome of the underlying disease.Key words: myasthenia gravis, children; myasthenia gravis, myasthenic crisis; myasthenia gravis, general anaesthesia; myasthenic crisis, management; myasthenic crisis, plasmapheresis Anaesthesiology Intensive Therapy 2013, vol. 45, no 2, 82-84 Myasthenia gravis (MG) is an autoimmune disease in which neuromuscular transmission is impaired due to a partial blocking of acetylcholine receptors in the postsynaptic membrane by circulating antibodies. Clinically, MG manifests as an abnormal fatigability of skeletal muscles that is relieved by rest and anticholinesterase drugs [1]. Its incidence rate is approximately 50-125: 1 000 000. The two major groups of patients include women aged 20-35 years and men aged 60-75 years. Children < 16 years of age constitute approximately 10-16% of all patients. Moreover, the prevalence of MG is three-fold higher in girls than in boys [1].When this disease progresses rapidly or the dosage of anticholinesterase drugs is too low and the susceptibility to them is consequently reduced, MG can progress to a life--threatening condition known as myasthenic crisis. CASE REPORTA 15-year-old boy (with a weight of 70 kg and a height of 165 cm) was admitted to the ICU of the Children's University Hospital from the neurology ward due to progressive hypodynamic respiratory failure and bulbar symptoms. His history revealed myasthenia of the lower limbs and articulation disorders that had been detected a year earlier. Based on clinical examinations, the patient was diagnosed with MG, and treatment with pyridostigmine at a dose of 60 mg day -1 was initiated. Moreover, a diagnosis of hypothyroidism was made, and levothyroxine was started 1 × 50 μg day -1 (euthyreosis for 3 months). The patient had a history of infantile cerebral ...

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Section: Discussionmentioning

confidence: 99%

“…The histopathological examinations usually reveal thymus hyperplasia; thymoma is observed less frequently [9]. To date, ectopic parathyroid glands have not been described in literature as accompanying thymus removal due to myasthenia gravis.…”

Section: Discussionmentioning

confidence: 99%

Postępowanie u dziecka w przełomie miastenicznym

Rybojad¹,

Lesiuk²,

Fijałkowska-Nestorowicz³

et al. 2013

Anaesthesiol Intensive Ther

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“…Another retrospective, multicenter study included 54 patients with myasthenic crisis and, again, compared the efficacy of PE versus IVIG. 42 This trial pointed to a better tolerability and a shorter time of hospitalization for patients treated with IVIG. Yet, the need of artificial ventilation after 14 days was lower in patients receiving PE.…”

Section: Plasma Exchange In Multiple Sclerosis Relapsesmentioning

confidence: 91%

Plasmapheresis for neuroinflammatory disorders

Schröder

Linker

Gold

2010

Clinical & Exp Neuroim

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Nowadays, therapeutic plasma exchange (PE) is the most common apheresis procedure. Plasma is separated from corpuscular blood constitutents and replaced with a substitution fluid. Thus, PE is a non‐specific treatment modality. Its therapeutic effect is based on the removal of circulating, pathogenic immune factors, including autoantibodies. To date, PE is well established for several immune mediated neurological disorders. Whilst the first experience was acquired in acute life‐threatening conditions, such as the treatment of Guillain–Barré syndrome or myasthenic crisis, therapeutic success was later achieved in other chronic autoimmune diseases; PE was applied successfully in chronic inflammatory demyelinating polyneuropathy, paraproteinaemic polyneuropathy, stiff‐person syndrome and might also be tried in autoimmune diseases of paraneoplastic origin. From a novel aspect, PE was also established as an escalation therapy for steroid unresponsive relapses of multiple sclerosis, and thus has gained even more widespread attention. Humoral disease mechanisms dominate even more in neuromyelitis optica, as a subtype of MS, and usually respond to PE. Adding to its increasing application in clinical practice, the procedure is usually well tolerated. Possible adverse reactions of PE arise from the large‐size vascular access site, the use of replacement fluids and the need for anticoagulation. (Clin. Exp. Neuroimmunol. doi: 10.1111/j.1759‐1961.2010.0010.x, 2010)

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“…In a recent randomized study (114) with 66 patients with myasthenic crisis, intravenous immunoglobulin and plasma exchange were found to be equally effective. A multi-center chart review study to compare the efficacy and tolerance of plasma exchange and intravenous immunoglobulin in the treatment of episodes of myasthenic crisis has shown that both treatments were effective but plasma exchange was associated with higher complication rate compared with intravenous immunoglobulin (115). Juvenile myasthenia gravis has been treated with intravenous immunoglobulin and thymectomy (116).…”

Section: Myasthenia Gravismentioning

confidence: 99%