Clinical presentation, evaluation, and management of patients with myasthenia gravis at a tertiary care center in Pakistan

Abstract

Note: The editors of PJNS are aware that this article contains old data. However, we have decided to publish this article because a) the data is still important as there is paucity of data in this regard from Pakistan, and b) the authors have written the article well and have mentioned this limitation of their manuscript. Background and objectives: Little is known about the clinical profile and outcome of myasthenia gravis in Pakistan. The objective of this study is to review the clinical characteristics of patients with myasthenia gravis in Pakistan, and the outcome of investigations and treatment. Methods: The study comprised a retrospective review of charts of patients diagnosed with myasthenia gravis at Aga Khan University Hospital in Karachi, Pakistan, over a period of 16 years from 1987 to 2003. The following features were reviewed: (i) clinical presentation, (ii) investigations especially nerve conduction studies, acetylcholine receptor antibodies, and imaging studies of the thorax, (iii) treatments administered (including thymectomy). Data analysis was done using Excel sheets. Results: Of the 83 patients, 51(61.4%) were males, whereas 32 (38.6%) were females. The age range was from 12 to 81 years, mean age of 43.7 years (SD± 18.2). In the 60 years plus group, there were three times as many males as compared to females. Limb weakness was noted in 58 (69.9%), ocular symptoms in 57(68.7%), and oropharyngeal symptoms in 54(65.1%) patients. Three (3.6%) presented in a state of myasthenic crisis. Tensilon test was performed in 34 patients and was positive in 31(91.2%), repetitive nerve conduction studies (RNS) were performed in 37 patients and was positive in 26(70.3%), acetylcholine receptor antibodies were done in 60 patients, and were positive in 55(91.7%). Thymic enlargement was seen in 28 out of 43 patients who underwent CT-Scan/MRI studies of the thorax. Besides pyridostigmine, most patients received immunosuppressive therapy with either steroids or azathioprine or both. The thymectomy was performed in 44 patients. Conclusion: The general disease pattern of MG, as noted in our series, appears to follow a similar pattern as noted worldwide. However, this retrospective and hospital-based study has its limitations, and more prospective and epidemiological studies are needed.