Analysis of the tremor in patients with anti-NF155 antibody-positive CIDP

2020 Fiscal Year Final Research Report

• Project/Area Number: 19K17036
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 52020:Neurology-related
• Research Institution: Kyushu University
• Principal Investigator: Inamizu Saeko 九州大学, 大学病院, 医員 (30822994)
• Project Period (FY): 2019-04-01 – 2021-03-31
• Keywords: neurofascin 155 / CIDP / 自己抗体 / 振戦 / 表面筋電図

Outline of Final Research Achievements

Surface electromyography was performed on five patients to reveal the nature and source of tremor in anti-neurofascin 155 (NF155) antibody-positive chronic inflammatory demyelinating polyneuropathy (CIDP). As a result, patients could be divided into two groups according to the frequencies of hand tremor, indicating that several mechanisms and anatomical sites might cause the tremor in anti-NF155 antibody-positive CIDP.
To elucidate the frequency and patterns of optic, trigeminal, and facial nerve involvement in anti-NF155 antibody-positive CIDP, 13 patients were subjected to neurological examination, visual-evoked potential (VEP) testing, and blink reflex. Absent or prolonged VEPs were observed in 10/13 (76.9%) patients and 17/26 (65.4%) eyes. All 12 patients tested for blink reflex had abnormalities. These results suggest that demyelination frequently occurs in the optic, trigeminal, and facial nerves in this condition.

Free Research Field

電気生理学

Academic Significance and Societal Importance of the Research Achievements

抗NF155抗体陽性CIDPは、同抗体陰性CIDPと異なる臨床的特徴や、治療反応性を有することが明らかとなっており、今後独立した疾患単位となる可能性がある。今回明らかにした振戦の様式により抗NF155抗体陽性CIDP症例を更に細分化できる可能性が示唆された。また、抗NF155抗体陽性CIDPでは脳神経障害が高頻度に生じることが明らかとなったため、同疾患と診断された症例については、積極的に電気生理学的検査を行い、早期に脳神経障害を同定することが望まれる。これらの所見が治療効果判定にも利用できる可能性がある。