Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of refractory IgG4-related peripheral neuropathy with severe axonal damage

Yu Suzuki, M.D.1), Makoto Shiraishi, M.D.1), Koji Yamada, M.D.1), Masatomo Doi, M.D.2), Masayuki Kato, M.D.3) and Yasuhiro Hasegawa, M.D.1)

1)Department of Internal Medicine, Division of Neurology, St Marianna University School of Medicine
2)Department of Diagnostic pathology, Division of Diagnostic pathology, St Marianna University School of Medicine
3)Department of Internal Medicine, Division of Hematology, St Marianna University School of Medicine

A 78-year-old man presented complaining of tingling and pain. Neurological examination revealed dysesthesia and hypothermesthesia below both knees and areflexia in the lower extremities. Laboratory data revealed elevated serum levels of immunoglobulin IgG4 and para-aortic, and mesenteric lymphadenopathy was evident on plain computed tomography of the abdomen. Microscopic findings of a bone marrow biopsy specimen showed occlusion of blood vessels with IgG4-positive plasma cells. IgG4-related disease was diagnosed because the bone marrow biopsy exhibited > 10 IgG4-positive plasma cells per high-power field. Treatment was initiated with prednisolone starting at 30 mg/day, but no improvement in neurological symptoms was achieved. Sural nerve biopsy demonstrated obstructive thromboangiitis with severe loss of myelin and axons. Further investigations are needed to elucidate the relationship between obstructive thromboangiitis and steroid-resistant IgG4-related peripheral neuropathy.
Full Text of this Article in Japanese PDF (780K)

(CLINICA NEUROL, 56: 323|327, 2016)
key words: IgG4-related disease, axonal neuropathy, occlusion of blood vessels, steriod-resistant

(Received: 29-Oct-15)