Open AccessCharacterization of IgG4 anti‐neurofascin 155 antibody‐positive polyneuropathy
Author(s) -
Ogata Hidenori,
Yamasaki Ryo,
Hiwatashi Akio,
Oka Nobuyuki,
Kawamura Nobutoshi,
Matsuse Dai,
Kuwahara Motoi,
Suzuki Hidekazu,
Kusunoki Susumu,
Fujimoto Yuichi,
Ikezoe Koji,
Kishida Hitaru,
Tanaka Fumiaki,
Matsushita Takuya,
Murai Hiroyuki,
Kira Junichi
Publication year - 2015
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.248
Subject(s) - medicine , chronic inflammatory demyelinating polyneuropathy , antibody , gastroenterology , polyneuropathy , immunology , pathology
Objective To investigate anti‐neurofascin 155 ( NF 155) antibody‐positive chronic inflammatory demyelinating polyneuropathy ( CIDP ). Methods Sera from 50 consecutive CIDP patients diagnosed in our clinic, 32 patients with multiple sclerosis, 40 patients with other neuropathies including 26 with Guillain–Barré syndrome ( GBS )/Fisher syndrome, and 30 healthy controls were measured for anti‐ NF antibodies by flow cytometry using HEK 293 cell lines stably expressing human NF 155 or NF 186. Four additional CIDP patients with anti‐ NF 155 antibodies referred from other clinics were enrolled for clinical characterization. Results The positivity rate for anti‐ NF 155 antibodies in CIDP patients was 18% (9/50), who all showed a predominance of IgG4 subclass. No other subjects were positive, except one GBS patient harboring IgG1 anti‐ NF 155 antibodies. No anti‐ NF 155 antibody carriers had anti‐ NF 186 antibodies. Anti‐ NF 155 antibody‐positive CIDP patients had a significantly younger onset age, higher frequency of drop foot, gait disturbance, tremor and distal acquired demyelinating symmetric phenotype, greater cervical root diameter on magnetic resonance imaging neurography, higher cerebrospinal fluid protein levels, and longer distal and F‐wave latencies than anti‐ NF 155 antibody‐negative patients. Marked symmetric hypertrophy of cervical and lumbosacral roots/plexuses was present in all anti‐ NF 155 antibody‐positive CIDP patients examined by neurography. Biopsied sural nerves from two patients with anti‐ NF 155 antibodies demonstrated subperineurial edema and occasional paranodal demyelination, but no vasculitis, inflammatory cell infiltrates, or onion bulbs. Among anti‐ NF 155 antibody‐positive patients, treatment responders more frequently had daily oral corticosteroids and/or immunosuppressants in addition to intravenous immunoglobulins than nonresponders did. Interpretation Anti‐ NF 155 antibodies occur in a subset of CIDP patients with distal‐dominant involvement and symmetric nerve hypertrophy.