![]() There are specific clinical scenarios where one must be extra cautious, for instance the newly diagnosed young female patient, who may be imminently planning a pregnancy, in contrast to a newly diagnosed elderly patient with multiple comorbidities. Now, more than ever, the treating physician must carefully contemplate which treatments are best suited for an individual MG patient since the “one size fits all” approach may not be as relevant. Therapies in the field of MG have significantly advanced over the years. This is a clinical emergency that requires management in an intensive care setting. In myasthenic crisis, the severe end of the disease spectrum, there is neuromuscular dysphagia rapidly evolving into complete loss of swallow function, and often in association with respiratory muscle weakness and type 2 respiratory failure. The majority, however, evolve further into generalized muscle weakness involving the facial and bulbar muscles, the neck and axial muscles and the limbs, with the upper limbs often being more severely affected than the lower limbs. They commonly present first with ocular manifestations such as asymmetrical fatigable ptosis with or without blurred or double vision. In an even smaller cohort of MG patients, no antibodies are detected on conventional antibody assay testing and we refer to these patients as “seronegative.” Patients with MG typically present with fatigable muscle weakness. These antibodies commonly are to the nicotinic acetylcholine receptor (AChR) but in a smaller proportion of cases, antibodies to muscle specific tyrosine kinase (MuSK) or to lipoprotein receptor-related protein 4 (Lrp-4) can be present instead ( 1– 3). Myasthenia gravis (MG) is a rare acquired autoimmune disorder of the neuromuscular junction (NMJ), caused by antibodies that target the post-synaptic membrane ( 1). In this review, we discuss various facets of myasthenia management in adults with ocular and generalized disease, including some practical approaches and our personal opinions based on our experience. ![]() MG patients may also develop dysfunctional breathing and the necessary respiratory physiotherapy techniques need to be implemented to alleviate the patient's symptoms of dyspnoea. Fatigue is common in MG and should be duly identified from fatigable weakness and managed with a combination of physical therapy with or without psychological support. Novel therapies are being developed and trialed, including ones that inhibit complement-induced immunological pathways or interfere with antibody-recycling pathways. In the younger age-groups, particularly in women, consideration must be given to the potential teratogenicity of certain therapies. Treatment needs to be individualized in the older age-group depending on specific comorbidities. There is evidence to support early treatment with corticosteroids when ocular motility is abnormal and fails to respond to symptomatic treatment. Ocular MG is not life-threatening but can be significantly disabling when diplopia is persistent. Minimally invasive thymectomy surgery including robotic-assisted thymectomy surgery has further revolutionized thymectomy and the management of MG. Data from the MGTX trial showed clear evidence that thymectomy is beneficial in patients with acetylcholine receptor (AChR) antibody positive generalized MG, up to the age of 65 years. Rituximab has a role in refractory MG, while plasmapheresis and immunoglobulin therapy are commonly prescribed to treat MG crisis and in some cases of refractory MG. Immunosuppressive therapies, such as azathioprine are prescribed in addition to but sometimes instead of corticosteroids when background comorbidities preclude or restrict the use of steroids. Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission. There is no single universally accepted treatment regimen. Symptom control, with acetylcholinesterase inhibitors such as pyridostigmine, is commonly employed. When the diagnosis of myasthenia gravis (MG) has been secured, the aim of management should be prompt symptom control and the induction of remission or minimal manifestations. ![]() 2Neuroimmunology Laboratory, Laboratory Medicine and Facilities Building, Queen Elizabeth University Hospital, Glasgow, United Kingdom.1Neurology Department, Institute of Neurological Sciences, Queen Elizabeth University Hospital, Glasgow, United Kingdom.
0 Comments
Leave a Reply. |