Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A case of anti-neurofascin 155 antibody-positive combined central and peripheral demyelination successfully treated with plasma exchange

Mikito Shimizu, M.D.1), Tohru Koda, M.D., Ph.D.2), Yuji Nakatsuji, M.D., Ph.D.1), Hidenori Ogata, M.D., Ph.D.3), Jun-ichi Kira, M.D., Ph.D.3) and Hideki Mochizuki, M.D., Ph.D.1)

1)Department of Neurology, Osaka University Graduate School of Medicine
2)Department of Neurology, Osaka General Medical Center
3)Department of Neurology, Kyushu University Graduate School of Medicine

A 21-year-old man was admitted to our hospital in June 2015. He felt paresthesia of toes in April 2015, which had been spreading upward, and he became difficult to walk in June. Nerve conduction study showed peripheral demyelinating neuropathy that met the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), and the cerebrospinal fluid (CSF) examination revealed the remarkably increased protein level. In addition, magnetic resonance imaging of his brain showed a few plaques in white matter, so he was finally diagnosed with combined central and peripheral demyelination (CCPD). Moreover, anti-neurofascin155 (NF155) antibodies assayed in his serum and CSF turned out to be positive. Although he was treated with intravenous immunoglobulin and intravenous methylprednisolone, his symptoms were not ameliorated. However, plasma exchange therapy was apparently effective, and the titer of anti-NF155 antibody was reduced. Recently, the number of case reports of CIDP with CNS lesions has gradually been increasing, while the information about the diagnosis and the treatment responses are not enough. Thus, we reported our case with CCPD who was successfully treated with plasma exchange.
Full Text of this Article in Japanese PDF (641K)

(CLINICA NEUROL, 57: 41|44, 2017)
key words: combined central and peripheral demyelination, anti-neurofascin155 antibody, chronic inflammatory demyelinating polyneuropathy, simple plasma exchange

(Received: 15-Sep-16)