We define late-onset myasthenia gravis (LOMG) when symptoms appear at ≥65 years of age. There has been a continuous increase in the incidence of LOMG with a clear male predominance. Commonly, patients present with focal (ocular or bulbar) weakness. A high index of suspicion required to achieve early diagnosis and to improve prognosis. Management options include acetylcholinesterase inhibitors, steroids, and immunosuppressants. The most controversial issue in treatment is thymectomy, because not enough data are available. Successful treatment is associated with improved survival, and death is often secondary to comorbidities.

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ObjectivesThere are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes.MethodsWe performed a retrospective chart review of MGUSN (n = 56) and CIDP (n = 67) patients. Data extracted included: demographics, neurological examination, and nerve conduction studies (NCS) at baseline and last visit. Clinical status was rated as 0 = worse, 1 = unchanged, 2 = stabilized after a declining course, or 3 = improved. The electrophysiology data were rated as 0 = worse, 1 = stable, or 2 = improved. Statistical analyses were performed using JMP (version 9.0.2 for Macintosh, from SAS).ResultsSeventy percent were males, aged 68.1 ± 12.6 years with neuropathy for 9.8 ± 6.8 years and follow-up of 4.0 ± 3.2 years. CIDP patients had more severe neuropathy, and were more likely to receive treatment and to respond. The clinical neuropathy status remained unchanged in 52.8 % of the MGUSN and 24.2 % of the CIDP patients, and stabilized in 7.6 % of MGUSN and 30.3 % of CIDP patients. IgM-MGUSN patients did not differ from other immunoglobulin subclasses in response to treatment. The clinical severity and the number of abnormal NCS parameters were greater in the demyelinating MGUSN in comparison to the axonal group.ConclusionMGUSN patients have less severe neuropathy than CIDP patients, but among the MGUSN patients the severity is greater in the demyelinating and the IgM groups. MGUSN patients may do well without treatment and exposure to potential adverse effects.

Myasthenia gravis is diagnosed at a progressively later age and the incidence continuously increases in the aged with a clear male predominance. People above the age of 65 constitute more than 50% of the newly diagnosed. Commonly, patients present with focal (ocular or bulbar) weakness. A high index of suspicion is needed to achieve early diagnosis and improve prognosis. Management options include acetylcholinesterase inhibitors, steroids, and immunosuppressants. Of the immunosuppressants, azathioprine is one of the most widely used due to its demonstrated effect and favorable side-effect profile. Others include cyclosporine, tacrolimus, mycophenolate mofetil, and cyclophosphamide. The use of mycophenolate mofetil is still controversial. Monoclonal antibodies, like rituximab, eculizumab, and belimumab, are relatively new therapeutic options in autoimmune diseases. Rituximab is of special interest in muscle-specific tyrosine kinase antibody-positive patients. A novel skeletal muscle activator and stem cell transplantation are being studied. There is no place for standard thymectomy in the older age groups except for thymomas and with computer-assisted endoscopy. Generally, the disease responds well to treatment at this age but comorbidities make medication choice more complex. Successful treatment is associated with improved survival, and quality of life can be remarkably improved during the whole survival period. As the median age of onset is 65 years and has been increasing steadily, it appears necessary to review the subject of treatment of myasthenia gravis in the aged.