Rational Therapy of Myasthenia Gravis

Finley, Jon M.; Pascuzzi, Robert M.

doi:10.1055/s-2008-1041256

Cited by 11 publications

(4 citation statements)

References 3 publications

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“…2). Although the transient nature of the response to plasma exchange has been emphasized, occasional patients may have a more prolonged response (24% remained improved to some degree for more than 6 months) (26). Vascular access requires placement of a central line.…”

Section: Treatment Optionsmentioning

confidence: 99%

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Myasthenia Gravis and Lambert‐Eaton Syndrome

Pascuzzi

2002

Therapeutic Apheresis

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Myasthenia gravis is a common autoimmune disorder characterized by the presence of pathogenic antibodies directed against the acetylcholine receptor. Patients present with variable degrees and distribution of fluctuating weakness at times life threatening. Clinical manifestations, establishment of diagnosis, the natural history of myasthenia gravis, and therapeutic options are herein reviewed. Far less common is Lambert‐Eaton syndrome (the myasthenic syndrome), another autoimmune disorder due to the presence of antibodies directed against the PQ‐type voltage‐gated calcium channels. Clinical features and treatment options are summarized.

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Section: Treatment Optionsmentioning

confidence: 99%

“…Patients commonly are hospitalized for initiation of therapy to avoid the risk of early exacerbation. Opinions differ regarding the best method of administration (23)(24)(25)(26).…”

Section: Corticosteroidsmentioning

confidence: 99%

Myasthenia Gravis and Lambert‐Eaton Syndrome

Pascuzzi

2002

Therapeutic Apheresis

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“…Opinions differ regarding the best method of administration. [24][25][26][27] For patients with severe MG, it is best to begin with high-dosage daily therapy of 60 to 80 mg/day by mouth (PO). Early exacerbation occurs in about half of patients, usually within the first few days of therapy and typically lasting 3 or 4 days.…”

Section: Corticosteroidsmentioning

confidence: 99%

“…Although the transient nature of the response to plasma exchange has been emphasized, occasional patients may have a more prolonged response (24% remained improved to some degree for more than 6 months). 27 Vascular access requires placement of a central line. Complications include hypotension, bradycardia, electrolyte imbalance, hemolysis, infection, and access problems (such as pneumothorax from placement of a central line).…”

Section: Plasma Exchangementioning

confidence: 99%

Pearls and Pitfalls in the Diagnosis and Management of Neuromuscular Junction Disorders

Pascuzzi

2001

Semin Neurol

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Myasthenia gravis (MG) is a common autoimmune disorder characterized by the presence of pathogenic antibodies directed against the acetylcholine receptor. Patients present with variable degrees and distribution of fluctuating weakness, at times life-threatening. Clinical manifestations, establishment of diagnosis, the natural history of MG, and therapeutic options are herein reviewed with an emphasis on pearls and pitfalls of clinical relevance. Far less common is Lambert-Eaton syndrome (the myasthenic syndrome), another autoimmune disorder due to the presence of antibodies directed against the PQ-type voltage-gated calcium channels. Clinical features and treatment issues of these and other disorders of neuromuscular transmission are reviewed.

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Safety for preoperative use of steroids for transsternal thymectomy in myasthenia gravis☆

Zieliński¹,

Kużdżał²,

Staniec³

et al. 2004

European Journal of Cardio-Thoracic Surgery

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Objective: Effects of preoperative steroids on morbidity and mortality after transsternal thymectomy are analyzed. Methods: There were 620 transsternal thymectomies for myasthenia gravis performed in the period . There were 547 patients with nonthymomatous myasthenia, including 17 patients submitted to repeated thymectomy (rethymectomy) and 46 patients with thymoma. There were 522 upper median sternotomies performed in the period 1973 -1997 and 98 complete median sternotomies performed in the period 1998-2002 (74 patients without thymoma, 7 thymomas and 17 rethymectomies). One hundred and seventeen of these 620 patients were taking steroids preoperatively. The impact of steroids use on postoperative respiratory insufficiency (ventilator), need for tracheostomy, superficial wound dehiscence, superficial wound suppuration, sternal dehiscence and mortality was analyzed. Results: The rate of morbidity and mortality in the steroids and the no-steroids groups included respiratory insufficiency necessitating the use of a ventilator in 46/503 patients from the nosteroids group (9.1%) and 8/117 patients from the steroids group (6.8%; P ¼ 0:4260), need for tracheostomy in 14/503 patients from the nosteroids group (2.8%) and 3/117 patients from the steroids group (2.6%; P ¼ 0:9054). Complications with wound healing (including sternal dehiscence, superficial wound dehiscence and superficial wound suppuration) occurred in 47/503 (9.3%) patients from the no-steroids group and in 1/117 (0.9%) patient from the steroids group ðP ¼ 0:0023Þ: The overall complications rate was 30.6% (154/503) in the no-steroids group and 11.1% (13/117) in the steroids group ðP ¼ 0:0001Þ: Mortality rate in the no-steroids group was 0.4% (2/503 patients) and none in the steroids group. Morbidity in patients with complete sternotomy was low, respiratory insufficiency in three patients (3.1%), and superficial wound suppuration in one patient (1.0%), no other morbidity and no mortality. Changing morbidity in periods 1973-1980, 1981-1990 and 1991-2002 is analyzed. Conclusions: Preoperative use of steroids for transsternal thymectomy in patients with myasthenia gravis had no negative impact on morbidity and mortality, conversely, the results in patients taking steroids were better, with significant difference of results regarding the overall complication rates and the overall complication rates of wound healing. q

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Rational Therapy of Myasthenia Gravis

Cited by 11 publications

References 3 publications

Myasthenia Gravis and Lambert‐Eaton Syndrome

Myasthenia Gravis and Lambert‐Eaton Syndrome

Pearls and Pitfalls in the Diagnosis and Management of Neuromuscular Junction Disorders

Safety for preoperative use of steroids for transsternal thymectomy in myasthenia gravis☆

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