Rinsho Shinkeigaku (Clinical Neurology)

Symposium 09

Clinicopathological features of neuropathy associated with IgG4-related disease

Ken Ohyama, M.D.1), Haruki Koike, M.D., Ph.D.1), Mie Takahashi, M.D.1), Yuichi Kawagashira, M.D., Ph.D.1), Masahiro Iijima, M.D., Ph.D.1) and Gen Sobue, M.D., Ph.D.1)

1)Department of Neurology, Nagoya University Graduate School of Medicine

The newly recognized entity IgG4-related disease (IgG4-RD) is characterized by an elevated IgG4 serum concentration, swelling of organ, and tissue infiltration by IgG4-positive plasma cells with fibrosis. IgG4-RD has been reported in various organs. In the field of neurology, hypophysitis and hypertrophic pachymeningitis have been known to be related to IgG4-RD, while reported patients with neuropathy manifesting features compatible to IgG4-RD. The features of IgG4-related neuropathy are characterized by sensory-motor neuropathy, mononeuritis multiplex pattern, and predominant involvement of distal portions of the lower extremities. In the sural nerve biopsy specimens, fibrosis and IgG4-positive plasma cell infiltration in the epineurium and decreased myelinated fiber density due to axonal degeneration were observed. IgG4-RD should be considered as the differential diagnosis of neuropathy.
Full Text of this Article in Japanese PDF (262K)

(CLINICA NEUROL, 54: 1047|1049, 2014)
key words: IgG4-related disease, IgG4-related neuropathy, mononeuritis multiplex, vasculitis, fibrosis

(Received: 22-May-14)