We present a case that suggests a relationship between primary biliary cirr
hosis and myasthenia gravis. A 43-year-old-Japanese woman was admitted to t
he Nagoya City University Medical School, First Department of Internal Medi
cine with abnormal liver function in August 1991.
She bad bad ptosis of the right eye since 1990. She had not been treated fo
r liver disease. Ptosis of the right eye and hepatomegaly were present. Ser
um laboratory examinations revealed elevated biliary enzymes and IgM levels
; tests were positive for antimitochondrial antibody and antiacetylcholine
antibody. Liver histology revealed chronic non-suppurative destructive chol
angitis and led to a diagnosis of primary biliary cirrhosis. The tensilon t
est was positive. Electromyography with repetitive motor nerve stimulation
revealed a neuromuscular junction defect; i.e., the primary characteristic
of myasthenia gravis. The patient was diagnosed with myasthenia gravis. Alt
hough the development of myasthenia gravis has previously been reported in
patients with primary biliary cirrhosis during D-penicillamine administrati
on, this is a very rare case of the coexistence of both diseases before suc
h treatment.