Ocular myasthenia gravis: response to long-term immunosuppressive treatment.
OBJECTIVE
Ocular myasthenia gravis is a subtype of myasthenia gravis that causes relatively mild disability, but may convert into severe generalised muscle weakness. A universal management plan for ocular myasthenia gravis has not been established. This study was performed to determine the outcome of ocular myasthenia gravis with the currently available therapeutic options.


METHODS
Retrospective analysis of 78 patients with ocular myasthenia gravis with a mean disease duration of 8.3 (range 0.5-58.3) years.


RESULTS
In 54 patients (69%) symptoms and signs remained confined to the extraocular muscles during the observation period. The remaining 24 patients (31%) developed symptoms of generalised myasthenia gravis; 50% of them within two years, 75% within four years after onset. A somewhat reduced risk of generalisation was found in those with mild symptoms, normal repetitive nerve stimulation test, and low or absent antiacetylcholine receptor (AChR) antibodies at the time of diagnosis. Patients receiving immunosuppressive treatment (corticosteroids and/or azathioprine) rarely developed generalised myasthenia gravis (six of 50, 12%). Those without such treatment, usually due to uncertain diagnosis and late referral, converted into generalised myasthenia gravis significantly more often (18 of 28, 64%).


CONCLUSIONS
The prognosis of ocular myasthenia gravis is good. A conventional scheme with short-term corticosteroids and long-term azathioprine seems adequate to achieve remission in most patients. The proportion of patients developing generalised myasthenia gravis was smaller in this population compared with previously published groups (usually 50%-70%). Early immunosuppressive treatment is at least partially responsible for this finding. Thymectomy (performed here in 12 patients with an abnormal chest CT) also correlated with a good outcome, but had no apparent advantage over medical treatment alone.
Sommer
N
N
Department of Neurology, Eberhard-Karls-University Tübingen, Germany.
Sigg
B
B
Melms
A
A
Weller
M
M
Schepelmann
K
K
Herzau
V
V
Dichgans
J
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology; D001379:Azathioprine / Q000008:administration & dosage* / Q000009:adverse effects; D002648:Child; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications / Q000628:therapy*; D015835:Ocular Motility Disorders / Q000150:complications / Q000628:therapy*; D011239:Prednisolone / Q000008:administration & dosage* / Q000009:adverse effects; D011379:Prognosis; D011729:Pyridostigmine Bromide / Q000008:administration & dosage* / Q000009:adverse effects; D012189:Retrospective Studies; D013934:Thymectomy; D013959:Thyroid Diseases / Q000150:complications / Q000453:epidemiology / Q000601:surgery; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D011239:Prednisolone; D011729:Pyridostigmine Bromide; D001379:Azathioprine