Myasthenia gravis: presentation and outcome in 104 patients managed in a single institution

Abstract

BACKGROUND AND OBJECTIVESFew studies have attempted to delineate the clinical profile of myasthenia gravis (MG) among people of Arab ancestry. Therefore, we sought to clarify the clinical profile, the outcome of treatment and the role of thymectomy in non-thymomatous MG in Saudi Arabia.PATIENTS AND METHODSWe retrospectively studied 104 patients followed over a mean period of 7.2 years (range, 1 to 22 years) at the King Khaled University Hospital, Riyadh, Saudi Arabia. Disease outcomes were compared among thymectomized and non-thymectomized patients according to the post-intervention status criteria of the Myasthenia Gravis Foundation of America (MGFA).RESULTSAge of onset was 22.5±9.3 years (mean±SD) in females and 28.2±15.9 years in males, with peaks in the second and third decades among females and the third and fourth decades among males. At diagnosis, a majority of patients had moderate generalized weakness, equivalent to MGFA class III severity. After medical treatment with or without thymectomy, 9.6% of all patients had achieved complete stable remission, 3.8% had pharmacological remission, 27.9% had minimal manifestations, 23.1% were improved, 20.2% were unchanged and 15.4% were worse. Only thymectomized patients without a thymoma achieved remission, a significant benefit over those who had no thymectomy (P=.02).CONCLUSIONMG presents at a younger age among Saudi Arabs compared to other racial groups. Thymectomy conferred significant benefits towards achievement of remission.

Course and prognosis of myasthenia gravis: a systematic review

“…No additional report was identified by searching relevant reference lists or by hand searches of the main journals of neurology. Of the 97 reports that were reviewed in full text, 13 studies fulfilled the inclusion criteria and were included in this review [6,17–28].…”

“…The agreement between reviewers for inclusion of articles was 93.8%, which gave kappa = 0.86 (95% CI, 0.74–0.98; almost perfect agreement) [29]. Of these, 10 studies were retrospective [6,17–22,25–27], study study was prospective [24], one study combined prospective and retrospective [28], and one study was not clear about the design [23]. All studies included patients with all disability levels of MG (data not shown).…”

“…Sample size varied between 41 patients and 1152 patients [20,25]. Most studies used composite diagnostic criteria [anti‐acetylcholine receptor (anti‐AChR) antibodies, electrophysiological, and pharmacological test], whilst one study made diagnosis based on at least one index test [17]. Only two studies reported disease duration from onset to baseline with median duration ranged from 9 to 20.5 years [28], and four studies presented disease duration from onset to diagnosis (time of diagnosis) [6,19,21,25].…”

“…Many variables were evaluated regarding demographic (in 12 studies), clinical (in eight studies), biochemical (in four studies), and therapeutic (in seven studies) (Tables 1 and 2, and Appendix S4). The prognostic factors most frequently reported were age at onset (12 studies) [6,17–26,28], thymectomy (seven studies) [6,17,19,21–23,25], thymus status (six studies) [6,19,22,23,26,28], severity of disease (five studies) [6,19,21,23,25], and sex (five studies) [6,19,21,23,25].…”

Myasthaenia Gravis: Clinical management issues before, during and after pregnancy

“…[1][2][3] In Western countries, the prevalence of MG is 50-125 cases per million individuals and can present at any age. 1 However, the incidence is twice as common among women (ratio: 3:2) and typically occurs during the second and third decades of life; in contrast, the disease most commonly presents in males during the sixth and seventh decades of life. 2 The majority of myasthaenic patients are seropositive for AChR antibodies, while seronegative patients may have antibodies to other targets at the neuromuscular junction (NMJ), such as muscle-specific kinase (MuSK).…”