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Research paper
A nationwide survey of combined central and peripheral demyelination in Japan
  1. Hidenori Ogata1,
  2. Dai Matsuse1,
  3. Ryo Yamasaki2,
  4. Nobutoshi Kawamura1,3,
  5. Takuya Matsushita2,
  6. Tomomi Yonekawa1,
  7. Makoto Hirotani4,
  8. Hiroyuki Murai1,
  9. Jun-ichi Kira1
  1. 1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  2. 2Department of Neurological Therapeutics, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  3. 3Department of Neurology, Kawamura Hospital, Gifu, Japan
  4. 4Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan
  1. Correspondence to Professor Jun-ichi Kira, Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan; kira{at}neuro.med.kyushu-u.ac.jp

Abstract

Objectives To clarify the clinical features of combined central and peripheral demyelination (CCPD) via a nationwide survey.

Methods The following characteristics were used to define CCPD: T2 high-signal intensity lesions in the brain, optic nerves or spinal cord on MRI, or abnormalities on visual-evoked potentials; conduction delay, conduction block, temporal dispersion or F-wave abnormalities suggesting demyelinating neuropathy based on nerve conduction studies; exclusion of secondary demyelination. We conducted a nationwide survey in 2012, sending questionnaires to 1332 adult and paediatric neurology institutions in Japan.

Results We collated 40 CCPD cases, including 29 women. Age at onset was 31.7±14.1 years (mean±SD). Sensory disturbance (94.9%), motor weakness (92.5%) and gait disturbance (79.5%) were common. Although cerebrospinal fluid protein levels were increased in 82.5%, oligoclonal IgG bands and elevated IgG indices were detected in 7.4% and 18.5% of cases, respectively. Fifteen of 21 patients (71.4%) had abnormal visual-evoked potentials. Antineurofascin 155 antibodies were positive in 5/11 (45.5%). Corticosteroids, intravenous immunoglobulins and plasmapheresis resulted in an 83.3%, 66.7% and 87.5% improvement, respectively, whereas interferon-β was effective in only 10% of cases. CCPD cases with simultaneous onset of central nervous system (CNS) and peripheral nervous system (PNS) involvement exhibited greater disability, but less recurrence and more frequent extensive cerebral and spinal cord MRI lesions compared to those with temporarily separated onset, whereas optic nerve involvement was more common in the latter.

Conclusions CCPD shows different characteristics from classical demyelinating diseases, and distinctive features exist between cases with simultaneous and temporarily separated onset of CNS and PNS involvement.

  • NEUROIMMUNOLOGY
  • MULTIPLE SCLEROSIS
  • NEUROPATHY
  • EPIDEMIOLOGY
  • NEUROMUSCULAR

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