Coexistence of myasthenia gravis and serological markers of neurological autoimmunity in neuromyelitis optica

Abstract

We systematically evaluated the frequency of neurological disorders and muscle and neural autoantibodies in 177 patients with neuromyelitis optica (NMO) and in 250 control subjects (173 healthy; 77 multiple sclerosis, MS, patients). An excess of myasthenia gravis (MG, 2%), and muscle-type acetylcholine receptor antibody (11%) was detected among NMO patients. The presence of neural or muscle autoantibodies was more common in NMO patients (34%) than in MS patients or healthy controls (7%), P < 0.0001. The coexistence of NMO and MG should be considered in atypical or refractory presentations of either disorder.

Neuromyelitis Optica Spectrum Disorder in a Chinese Woman with Ocular Myasthenia Gravis: First Reported Case in the Chinese Population

“…17 The current evidence of the coexistence of MG and NMO spectrum disorder is summarised in Table 1. 16,17 Our patient presented with bilateral recurrent optic neuritis prior to the onset of ocular MG and was later confirmed to have NMO spectrum disorder via seropositivity for NMO-IgG. It has been reported in the literature that in 90-98% of subjects, the diagnosis of MG preceded that of NMO spectrum disorder with a lapse time ranging from 4 to 16 years.…”

“…[13][14][15] McKeon et al demonstrated that 34% of NMO patients are harboured with autoimmune antibodies against neural tissues and muscles. 16 Patients with MG may trigger the onset of NMO spectrum disorder FIGURE 4 Spectralis OCT demonstrating generalised RNFL thinning over both eyes. after thymectomy.…”

Multiple sclerosis: Serum anti-CNS autoantibodies

“…Examples of such antibodies are antibodies that recognize conformationally intact human antimyelin-associated glycoprotein (MOG) and that are common in childhood inflammatory demyelinating diseases as well as some cases of aquaporin-4 (AQP4)-negative NMO. 19,20 This study is the first report showing that anti-CNS autoantibodies of different specificities are frequently present in the serum in patients with MS. Why such antibodies have not been detected in previous large IIF studies [15][16][17] relates chiefly to differences in the methods used to fix the tissue. Traditionally, cryostat sections of snap-frozen rodent tissue are exposed to the test serum without fixation or following treatment of the section for 10 minutes with ethanol or acetone or with formalin.…”

“…Previous IIF studies of serum autoantibodies in MS refer to the use of cryostat sections of fresh unfixed tissue or sections fixed in acetone, ethanol, or formalin followed by application of the test serum and a fluorochrome-conjugated anti-human IgG antiserum. 14 With this procedure, anti-CNS autoantibodies have been reported to be absent in the serum of patients with MS. [15][16][17] In this study of serum autoantibodies in MS, a fixation method is used that instantly fixes still vital tissues by whole-body perfusion with chilled paraformaldehyde, a procedure known to optimally preserve some antigens, as well as improve tissue morphology.…”

Conventional and Advanced Imaging in Neuromyelitis Optica

“…23,24 However, autoantibodies directed to antigens other than AQP4 are often also detectable in NMO sera, 25,26 and a proportion of patients have clinical features of systemic autoimmunity. Of 8 patients with Sjögren syndrome-associated myelopathy identified by Kim et al, 27 7 met the diagnostic criteria for NMO, and AQP4 antibodies were present in 4/5 patients whose serum was available for testing.…”