Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of an anti-SRP myopathy with enlargement of the thymus

Rie Ohta, M.D.1)2), Akihiro Mukaino, M.D.1)2), Ikuo Kinoshita, M.D.1), Mitsuhiro Tsujihata, M.D.3) and Shigeaki Suzuki, M.D., Ph.D.4)

1)Section of Neurology, Japanese Red Cross Nagasaki Genbaku Hospital
2)Present Address: Department of First Internal Medicine, Nagasaki University Hospital
3)Section of Neurology, Nagasaki-Kita Hospital
4)Department of Neurology, School of Medicine, Keio University

A 54-year-old female was admitted to our hospital because of the Raynaud phenomenon and muscle weakness of the upper limbs. The neurological findings showed somatic and proximal limb weakness. Laboratory studies showed a high serum creatine kinase level. Computerized tomography (CT) revealed enlargement of the thymus. A muscle biopsy showed a small number of degenerating and regenerating fibers but no inflammatory infiltrations. At first, she was initially treated with a three-day course of intravenous methylprednisolone (1 g/day). However, the weakness progressed and the serum creatine kinase level remained high. She was subsequently treated with a combination of tacrolimus (3 mg/day) and prednisolone, but showed no any improvement of the muscle weakness. Following additional treatment with intravenous immunoglobulin, she showed improvement in her muscle weakness. Further, anti-signal recognition particle antibodies were identified after treatment. There have been no previous reports of myopathy with antibodies against the signal recognition particle and enlargement of the thymus, so we herein report the details of this unique case.
Full Text of this Article in Japanese PDF (7715K)

(CLINICA NEUROL, 54: 798|802, 2014)
key words: anti-signal recognition particle antibody, enlargement of the thymus, intravenous immunoglobulin, Raynaud phenomenon

(Received: 2-Oct-13)