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• Vol.62 No.3
• CLINICA NEUROL, 62: 173−177, 2022
• Vol.62 No.3 contents

Original Article

Clinical and long-term characteristics of the subtypes of chronic inflammatory demyelinating polyneuropathy

Fumitaka Shimizu, M.D., Ph.D.¹⁾, Jo Nemoto, M.D.¹⁾, Yukio Takeshita, M.D., Ph.D.¹⁾, Toshihiko Maeda, M.D., Ph.D.¹⁾, Michiaki Koga, M.D., Ph.D.¹⁾ and Takashi Kanda, M.D., Ph.D.¹⁾

¹⁾ Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine

Objective: To clarify the clinical and long-term characteristic of each subtype of chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: We evaluated data from 30 consecutive CIDP patients who met the criteria proposed by the European Federation of Neurological Societies and the Peripheral Nerve Society. Results: Patients were classified as having typical CIDP (t-CIDP) (10/30, 33%), multifocal acquired demyelinating sensory and motor (MADSAM) (12/30, 40%), DADS (4/30, 13%), sensory CIDP (3/30, 10%) or motor CIDP (1/30, 3%). Nerve conduction studies showed more prolonged distal motor latencies/F-wave latencies and slower motor nerve conduction in the typical CIDP group than in the MADSAM group. Intravenous immunoglobulin (IVIg) was effective in 80% (8/10) of t-CIDP, 100% (12/12) of MADSAM, 100% (4/4) of DADS, and 100% (3/3) of sensory CIDP cases. Maintenance therapy with immunoglobulin was administered in patients with t-CIDP (5/10, 50%), MADSAM (9/12, 75%), DADS (1/4, 25%), and sensory CIDP (2/3, 67%). There were no patients with CIDP, in whom CIDP subtype was transformed from the initial diagnosis during five years of follow-up. Discussion: Percentage of MADSAM was the most common phenotype in our cohort of CIDP patients, and IVIg/immunoglobulin maintenance was effective for MADSAM as well as t-CIDP in contrast to findings from the previous reports.
Full Text of this Article in Japanese PDF (443K)

(CLINICA NEUROL, 62: 173−177, 2022)
key words: chronic inflammatory demyelinating polyneuropathy, clinical characteristic, long-term characteristic

(Received: 10-Jul-21)