Clinical heterogeneity of seronegative myasthenia gravis.
We studied 38 patients affected by seronegative myasthenia gravis (SNMG) with age at the onset of the disease ranging from 6 to 66 yr. Clinical follow-up lasted at least 2 yr. Patients' lymphocyte cultures showed in no case anti-acetylcholine receptor antibody production; HLA associations did not differ significantly from those in seropositive MG. In most cases (33 out of 38) the disease differed from seropositive MG only in the low incidence of thymic pathology. In five cases the clinical picture was characterized by prevalent involvement of ocular and bulbar muscles and by a more stable course. These last group of patients did not respond satisfactorily to immunosuppressants, but they clearly improved with plasma-exchange. Mice injected with plasma IgG from four patients (two with typical clinical picture and two with prevalent oculobulbar involvement) showed a defect of neuromuscular transmission. In our experience, SNMG is a heterogeneous disease. A humorally mediated pathogenesis appears to be operating in both groups of patients we described.
Evoli
A
A
Institute of Neurology, Catholic University, Rome, Italy.
Batocchi
A P
AP
Lo Monaco
M
M
Servidei
S
S
Padua
L
L
Majolini
L
L
Tonali
P
P
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000818:Animals; D001323:Autoantibodies / Q000097:blood*; D002478:Cells, Cultured; D002648:Child; D004491:Edrophonium; D005260:Female; D015795:HLA-B8 Antigen / Q000032:analysis; D006681:HLA-D Antigens / Q000032:analysis; D006650:Histocompatibility Testing; D006801:Humans; D007116:Immunization, Passive; D007074:Immunoglobulin G / Q000008:administration & dosage / Q000097:blood*; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000276:immunology*; D008297:Male; D051379:Mice; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000097:blood / Q000276:immunology* / Q000503:physiopathology; D011950:Receptors, Cholinergic / Q000276:immunology*; D014403:Tubocurarine
D001323:Autoantibodies; D015795:HLA-B8 Antigen; D006681:HLA-D Antigens; D007074:Immunoglobulin G; D011950:Receptors, Cholinergic; D004491:Edrophonium; D014403:Tubocurarine