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• Vol.54 No.12
• CLINICA NEUROL, 54: 1047−1049, 2014
• Vol.54 No.12 contents

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)