Comparison of Lewis–Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.
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| Title: | Comparison of Lewis–Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre. |
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| Authors: | Fargeot, G. (AUTHOR), Maisonobe, T. (AUTHOR), Psimaras, D. (AUTHOR), Debs, R. (AUTHOR), Lenglet, T. (AUTHOR), Adams, D. (AUTHOR), Vandendries, C. (AUTHOR), Labeyrie, C. (AUTHOR), Viala, K. (AUTHOR) |
| Source: | European Journal of Neurology. Mar2020, Vol. 27 Issue 3, p522-528. 7p. 1 Diagram, 4 Charts. |
| Subjects: | Tertiary care, Arm, Motor neuron diseases, Syndromes, Neural conduction |
| 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. [ABSTRACT FROM AUTHOR] |
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| Database: | Psychology and Behavioral Sciences Collection |
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