Outpatient clinic of Myasthenia Gravis and Other Disorders of Neuromuscular Transmission
The specialized outpatient clinic for Myasthenia Gravis (MG) and other disorders of the neuromuscular transmission of the1st Neurology Department of National and Kapodistrian University of Athens was started in 2008 and during the last 3 years 1312 patients have been evaluated.
Autoimmune myasthenia gravis is a treatable disorder of the neuromuscular transmission, although it can be a potentially life threatening disease because of bulbar and/or respiratory muscle involvement (myasthenic crisis). In the majority of patients immunosuppressive therapy is highly effective but it may be lifelong. MG is not a single entity and there are clinical subgroups with therapeutic and prognostic implications. In our clinic, the basic therapeutic principles are the individualization and close monitoring of treatment.
Lambert-Eaton myasthenic syndrome (LEMS) is another rare autoimmune disorder of the neuromuscular transmission. It is one of the best characterized paraneoplastic syndromes, frequently associated with small cell lung cancer. In the paraneoplastic LEMS the tumor therapy is the first step, while in the non-paraneoplastic LEMS the therapy is symptomatic and immunosuppressive.
Other even more rare syndromes are Acquired Neuromyotonia and Congenital Myasthenic Syndromes (not autoimmune myasthenia).
Clinical suspicion based on patient’s history and neurological examination is crucial for the diagnosis of neuromuscular transmission defect. The ‘’clinical diagnosis’’ is confirmed by the detection of specific Autoantibodies, which are tested in Private Laboratories and by Electrophysiologial Testing of neuromuscular transmission.
Electrophysiological testing is performed in the Electromyography and Electroneurography Laboratory of 1st Neurology Department of National and Kapodistrian University of Athens.
Thymus gland is frequently involved in the pathophysiology of autoimmune myasthenia gravis and Magnetic Resonance Imaging or Computed Tomography of the Mediastinum is mandatory for detection of thymoma. The radiological evaluation is performed in 2nd Radiology Department of National and Kapodistrian University of Athens or in Radiology Department of Aretaieion Hospital.
Thymectomy, when is indicated, is performed in Cardiothoracic Surgery Department of General Hospital.
When hospital admission is indicated, there is a specialized Ward for Neuromuscular Disorders in the 1st Neurology Department of National and Kapodistrian University of Athens.
Patients with myasthenic crisis are usually hospitalized in Intensive Care Unit of General Hospital of Athens ‘’Alexandra’’.
When there is severe clinical deterioration then Plasma Exchange is indicated for rapid recovery. This short-term immunotherapy is performed in the Renal Unit(s) of General Hospital of Athens ‘’Alexandra’’ and of Aretaieion Hospital.
Respiratory muscle involvement is potentially life threatening and consequently regular monitoring of Respiratory Function is essential. This is performed in the Laboratory of 1st Pulmonary Department of National and Kapodistrian University of Athens.
Dr. Zouvelou Vasiliki is in charge of the outpatient clinic.