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      The effect of prednisone on the progression from ocular to generalized myasthenia gravis.

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      Publication date:
      Journal: Journal of the Neurological Sciences
      Keywords: Cholinesterase Inhibitors, therapeutic use, Disease Progression, Dose-Response Relationship, Drug, Drug Administration Schedule, Female, Humans, Immunosuppressive Agents, Male, Middle Aged, Myasthenia Gravis, drug therapy, physiopathology, prevention & control, Oculomotor Muscles, drug effects, immunology, Prednisone, Pyridostigmine Bromide, Receptors, Cholinergic, Retrospective Studies, Treatment Outcome

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          Abstract

          Fifty percent of ocular myasthenia gravis (OMG) patients will progress to generalized myasthenia, 90% within 3 years from the onset of ocular symptoms. This study was performed to determine whether treatment with oral prednisone initiated and completed within 2 years from the onset of ocular symptoms would affect the progression of ocular myasthenia to generalized myasthenia gravis (GMG). Fifty-six patients were included in this review, with 27 patients in the prednisone-treated group and 29 patients in the untreated group. The treated group was initiated on 60 mg of prednisone daily with a slow taper over 3-6 months. At 2 years, significantly fewer patients in the treated group (3 of 27) progressed to generalized myasthenia when compared to the untreated group (10 of 29) (chi(2), p=0.04). Our results suggest that the early use of steroids may decrease progression of ocular to generalized myasthenia gravis. The decision to use steroids should be considered early in the course of patients diagnosed with ocular myasthenia gravis. This study should be considered preliminary and a prospective trial is warranted to confirm our observations.

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          Myasthenia gravis.

          D Drachman (1994)
          Article 0 comments Cited 139 times – based on 0 reviews     Review now
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            Development of generalized disease at 2 years in patients with ocular myasthenia gravis.

            Mark Kupersmith, Robert Latkany, Peter Homel (2003)
            Generalized myasthenia gravis will develop in more than 50% of patients who present with ocular myasthenia gravis, typically within 2 years. The optimal treatment of ocular myasthenia gravis, including the use of corticosteroids, remains controversial. In addition, the prevalence of thymoma and the optimal performance of the edrophonium chloride test for ocular myasthenia remain unknown. To assess the effect of oral corticosteroid therapy on the frequency of development of generalized myasthenia gravis within 2 years, the incidence of thymoma, and the amount of edrophonium needed for a positive test result in patients with ocular myasthenia gravis. We reviewed an ocular myasthenia gravis database of 147 patients. Patients underwent measurement of acetylcholine receptor (AChR) antibody levels and chest computed tomography. Unless contraindicated, patients with diplopia were recommended for therapy with prednisone, up to 40 to 60 mg/d, with the dosage tapered for 5 to 6 weeks. Most continued to receive daily or alternate-day doses of 2.5 to 10 mg to prevent diplopia. Patients not given prednisone (untreated group) received pyridostigmine bromide or no medication. After the diagnosis, we documented the signs and symptoms of ocular and generalized myasthenia gravis and performed 2-year follow-up in 94 patients. The mean dose of edrophonium chloride to give a positive response was 3.3 mg (SD, 1.6 mg) for ptosis and 2.6 mg (SD, 1.1 mg) for ocular motor dysfunction. Thymoma occurred in 1 patient (0.7%). Generalized myasthenia gravis developed within 2 years in 4 of 58 treated and 13 of 36 untreated patients. The odds ratio (OR) for development of generalized disease in the treated group was 0.13 (95% confidence interval [CI], 0.04-0.45) compared with the untreated group. The AChR antibody level was not predictive of development of generalized myasthenia gravis at 2 years, but the risk was greater in patients with abnormal AChR antibody levels (OR, 6.33; 95% CI, 1.71-23.42). Logistic regression that included age, abnormal AChR antibody level, and prednisone therapy yielded significance only for abnormal AChR antibody level (OR, 7.03; 95% CI, 1.35-36.64) and treatment (OR, 0.06; 95% CI, 0.01-0.30). At 2 years, prednisone treatment appears to reduce the incidence of generalized myasthenia gravis to 7% in contrast to 36% of patients who did not receive prednisone. Thymoma, although uncommon, occurs in ocular myasthenia gravis. Only small amounts of edrophonium are needed to diagnose ocular myasthenia gravis.
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              Prognosis of ocular myasthenia.

              Manuel A. S. Aquino, Lauren A. Penn, C F Bever … (1983)
              A retrospective study of 108 patients with myasthenia gravis who had solely ocular symptoms and signs at onset was carried out to identify factors influencing prognosis. Increasing duration of pure ocular myasthenia was associated with a decreasing risk of late generalized symptoms; only 9 (15%) of the observed generalizations occurred after more than 2 years of solely ocular symptoms. Increasing age at onset was associated with greater risk of respiratory crisis or death caused by myasthenia, whereas patients younger at onset had a greater chance of a benign outcome. Neither systemic curare tests nor responses to repetitive nerve stimulation had prognostic value.
              Article 0 comments Cited 48 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                PubMed ID:: 14706214
                DOI:: 10.1016/j.jns.2003.08.017

                ScienceOpen disciplines: Chemistry
                Keywords: Cholinesterase Inhibitors,therapeutic use,Disease Progression,Dose-Response Relationship, Drug,Drug Administration Schedule,Female,Humans,Immunosuppressive Agents,Male,Middle Aged,Myasthenia Gravis,drug therapy,physiopathology,prevention & control,Oculomotor Muscles,drug effects,immunology,Prednisone,Pyridostigmine Bromide,Receptors, Cholinergic,Retrospective Studies,Treatment Outcome
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                ScienceOpen disciplines: Chemistry
                Keywords: Cholinesterase Inhibitors, therapeutic use, Disease Progression, Dose-Response Relationship, Drug, Drug Administration Schedule, Female, Humans, Immunosuppressive Agents, Male, Middle Aged, Myasthenia Gravis, drug therapy, physiopathology, prevention & control, Oculomotor Muscles, drug effects, immunology, Prednisone, Pyridostigmine Bromide, Receptors, Cholinergic, Retrospective Studies, Treatment Outcome

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