Comparison of Lewis–Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre. (24th October 2019)

• Record Type: Journal Article
• Authors: Fargeot, G.; Maisonobe, T.; Psimaras, D.; Debs, R.; Lenglet, T.; Adams, D.; Vandendries, C.; Labeyrie, C.; Viala, K.
• Is Part Of: European journal of neurology. Volume 27:Number 3(2020)
• Page Start: 522
• Page End: 528
• Publication Date: 2019-10-24
• Subjects: chronic inflammatory demyelinating polyradiculoneuropathy -- chronic inflammatory polyradiculoneuropathy -- Lewis–Sumner syndrome -- multifocal acquired demyelinating sensory and motor neuropathy; Neurology -- Periodicals; Nervous system -- Diseases -- Periodicals; 616.8
• DOI: 10.1111/ene.14101
• Languages: English
• ISSNs: 1351-5101

Abstract

Abstract : Background and purpose: Whether the Lewis–Sumner syndrome (L‐SS) is a distinct entity from other types of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP‐ot) remains controversial. Method: The clinical/electrophysiological characteristics and long‐term outcomes of 45 L‐SS and 35 CIDP‐ot patients were retrospectively compared. Results: The CIDP‐ot group was composed of 11 patients with a typical CIDP, 17 with a pure sensory form, four with a distal form and three with a pure motor form. In the L‐SS group, asymmetric ( P < 0.001) and monomelic involvement ( P = 0.04) of the upper limbs ( P < 0.001) was significantly more frequent; paucisymptomatic forms (Overall Neuropathy Limitations Scale ≤ 1) were less frequent ( P < 0.001); electroneuromyography showed that conduction block in intermediate nerve segments was the main demyelinating feature, with frequent F‐wave abnormalities on nerves without conduction block (44%). Long‐term prognosis was globally poorer in the L‐SS group with more frequent aggravation during treatment ( P = 0.02), less frequent treatment withdrawal ( P = 0.03) and longer time to achieve successful withdrawal (39 vs. 15 months). Conclusions: Our study suggests that L‐SS patients have a less favourable therapeutic response rate and long‐term outcomes. Rapid differentiation of L‐SS from other forms of CIDP is important in order to anticipate a more complicated disease course management, with from one side the inefficacy or even harmfulness of corticosteroids and from the other side a difficult weaning procedure. A prospective study is necessary to confirm these results.