Immunosuppressive treatment of ocular myasthenia gravis.
Myasthenia gravis (MG) is caused by autoantibodies against proteins at the neuromuscular junction. This autoimmune process leads to abnormal fatiguability and weakness of striated muscle. Ptosis and diplopia are among the most common manifestations of MG. The term "ocular MG" (OMG) as opposed to "generalised MG" (GMG) is used to define the clinical subtype of MG with isolated eye muscle weakness. Although OMG may appear to cause only moderate disability, it can significantly impair the patient's activities of daily living and progress to generalised myasthenia. Therefore, a clear management plan should be installed early in these patients. Since prospective treatment trials have not been performed, basic management strategies for OMG have to be deduced from retrospective studies, trials in GMG, and generally accepted clinical experience. Cholinesterase inhibitors are used in all types of MG, but are often less helpful in OMG. In the absence of thymoma, thymectomy is usually not considered in OMG, although a few studies have described histological abnormalities in thymuses from patients with OMG. Corticosteroids are of great short term benefit in most patients with OMG but potential adverse effects limit their long term use. Azathioprine is needed to reduce long term corticosteroid adverse effects, but this agent requires about 6 months to be effective. In summary, OMG has a good prognosis in most patients, with corticosteroids and azathioprine being the major treatment options. The challenges for the clinician are to recognise the condition despite the large number of differential diagnoses, to minimise the patient's symptoms using the therapies available and to carefully limit potentially hazardous therapeutic efforts, especially in mild or even uncertain cases.
Tackenberg
B
B
Clinical Neuroimmunology Group, Department of Neurology, Philipps-University, Rudolf-Bultmann-Strasse 8, D-35033 Marburg, Germany.
Hemmer
B
B
Oertel
W H
WH
Sommer
N
N
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use*; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D005128:Eye Diseases / Q000188:drug therapy*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009157:Myasthenia Gravis / Q000150:complications / Q000188:drug therapy*; D011379:Prognosis; D013934:Thymectomy
D000305:Adrenal Cortex Hormones; D002800:Cholinesterase Inhibitors; D007166:Immunosuppressive Agents; D001379:Azathioprine