Autonomic dysfunction occurs frequently in multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). Patients with either condition may present with autonomic symptoms such as bladder, sexual, cardiovascular, thermoregulatory, and gastrointestinal dysfunction, and fatigue, but autonomic symptoms that affect quality of life are underrecognized in clinical practice. The immunopathogenesis of MS has been considered to be associated with autonomic dysfunction. Applying appropriate treatment strategies for autonomic dysfunction is important to improve the quality of life of patients. Here we review autonomic dysfunction and how this is managed in patients with MS and NMOSD.
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Sexual function and related predictors in male with multiple sclerosis and neuromyelitis optica spectrum disorder: a case–control study Saeed Vaheb, Mohammad Yazdan Panah, Mohammad Mohammadi, Mohammad Amin Sadri, Narges Ebrahimi, Sarina Loghmani, Marjan Beigi, Vahid Shaygannejad, Omid Mirmosayyeb The Journal of Sexual Medicine.2025; 22(2): 274. CrossRef
Autoantibodies are present in many autoimmune disorders, including diseases impacting the peripheral nerve, neuromuscular junction, and muscle. Some of these autoantibodies play a vital role in pathogenesis, whereas others are unlikely to be directly pathogenic, but may be useful biomarkers. The identification of autoantibodies is valuable in diagnosis, as well as in establishing a treatment plan in antibody-mediated neuromuscular disorders. This review briefly summarizes antibody, autoantibody, and methods of autoantibody testing for clinicians who treat patients with neuromuscular disorders.
Background : The pathogenesis of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), Guillain-Barr?syndrome (GBS) in not clear, but it has been known that the immune mechanisms play an important role. Authors performed this study to establish an animal model of experimental allergic neuritis(EAN) by immunizing the myelin components of peripheral nerves and to understand the electrophysiological and histopathological features as well as the CD5+ B-lymphocyte changes in peripheral bloods in the EAN models. Methods : Lewis rats weighing 150-200 gm were injected subcutaneously in soles two with total myelin, P0, P1, or P2 proteins purified from the bovine cauda eguina. The EAN induction was assessed by evaluating clinical manifestations. The electrophysiological and histopathological features were studied as routine methods. The CD5+ B-lymphocyte were double stained using monoclonal FITC conjugated anti-rat CD45RA and R-PE conjugated anti-rat CD5+antibodies and calculated using a fluorescence activated cell sorter (FACS). Results : The EAN animal models were established. In two out of five, in one out of two, in none out of three, and in none out of one Lewis rats injected with purified total myelin, P0, P1, P2 proteins respectively. They showed slow spontaneous motor activity and weak resistance against pulling back by tails. The typical electrophysiological and histologic findings in total protein and P0 induced EAN animal models were the decreased conduction velocity, the decreased compound muscle action potential (CMAP) amplitude and the dispersion phenomenon. The perivascular infiltrates of lymphocytes with focal demyelinating process were found in light microscopy. The CD5+ B-lymphocyte expression in three EANs were 2.38%, 3.50% 2.50%, which were not significantly increased, compared with those in normal controls. Conclusion : The EAN animal models were successfully established by injecting the total myelin and P0 myelin and they showed electrophysiological and histological features typical of demyelinating process. However they did not show an increased expression of CD5+ B-lymphocyte in peripheral bloods which could be indirect evidence of humoral autoimmunity.
Both myasthenia gravis and Guillain-Barre syndrome are autoimmune disorder, but it is very rare that both of them occur together. A 53-years old woman with history of myasthenia gravis complained of weakness of extremities, worsening progressively for several days. Electrophysiologic study showed findings of motor axonal neuropathy without sensory involvement. She became improved in aspect f of clinical symptoms and electrophysiologic findings after infusion of immunoglobulin. This is a case of Guillain-Barre syndrome in a patient with myasthenia gravis.
Background & Object : Myasthenia gravis(MG) is an autoimmune disease due to binding of antibody to acetylcholine receptors on the muscle membrane. It is well known that other autoimmune disease infrequently accompany myasthenia gravis. The aim of this study was to evaluate the clinical significance of associated autoimmune diseases(AAD) and compare prognosis between MG with AAD and MG without AAD. Method : A total of 65 MG patients(24 men and 41 women) were enrolled at this study. From the clinical records of these patients, we investigated the clinical characteristics and prognosis of MG with AAD and compared thses date with those of MG without other such disease. Results : AAD were found in 10 of 65 cases(15%). 9 cases of10 MG with AAD were generalized MG type. The most common disease was thyroid disorders. The rate of AAD was higher in thymic abnormal patients. There was no significant remission rate difference between MG with AAD and MG without AAD, but the percentage of patients experienced crisis was higher in MG with AAD. Conclusion : The occurrence of AAD may suggest a more generalized autoimmune disturbance that could be associated with a less favorable prognosis.
Although the etiology and pathogenesis of amyotrophic lateral sclerosis(ALS) in unknown, increasing evidence support a role autoimmune machanisms in motor neuron degeneration. The coexistence of immune disease in ALS supports that an altered immune system may contribute to disease pathogenesis. A 55-year-old woman was admitted to our department due to dysarthria and gait disturbance. On physical and neurologic examination, she showed thyroid enlargement, tongue atrophy, muscle weakness, fasciculation, and increased deep tendon reflex. The electrophysiological studies are compatible with motor neuron disease. Cytological findings of thyroid were compatible with hashimoto's thyroiditis. Thus, we report a case of ALS combined with Hashimoto's thyroiditis. And the simultaneous presentation with ALS and Hashimoto's thyroiditis led us to consider whether this was simply a chance association or not.
Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has beenused in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.