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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.
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]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Comparison of Lewis–Sumner syndrome with chronic inflammatory demyelinating polyradiculoneuropathy patients in a tertiary care centre.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Fargeot%2C+G%2E%22&quot;&gt;Fargeot, G.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Maisonobe%2C+T%2E%22&quot;&gt;Maisonobe, T.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Psimaras%2C+D%2E%22&quot;&gt;Psimaras, D.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Debs%2C+R%2E%22&quot;&gt;Debs, R.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Lenglet%2C+T%2E%22&quot;&gt;Lenglet, T.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Adams%2C+D%2E%22&quot;&gt;Adams, D.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Vandendries%2C+C%2E%22&quot;&gt;Vandendries, C.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Labeyrie%2C+C%2E%22&quot;&gt;Labeyrie, C.&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Viala%2C+K%2E%22&quot;&gt;Viala, K.&lt;/searchLink&gt; (AUTHOR)
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22European+Journal+of+Neurology%22&quot;&gt;European Journal of Neurology&lt;/searchLink&gt;. Mar2020, Vol. 27 Issue 3, p522-528. 7p. 1 Diagram, 4 Charts.
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  Data: &lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Tertiary+care%22&quot;&gt;Tertiary care&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Arm%22&quot;&gt;Arm&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Motor+neuron+diseases%22&quot;&gt;Motor neuron diseases&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Syndromes%22&quot;&gt;Syndromes&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Neural+conduction%22&quot;&gt;Neural conduction&lt;/searchLink&gt;
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: 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 &lt; 0.001) and monomelic involvement (P = 0.04) of the upper limbs (P &lt; 0.001) was significantly more frequent; paucisymptomatic forms (Overall Neuropathy Limitations Scale ≤ 1) were less frequent (P &lt; 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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  Data: &lt;i&gt;Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder&#39;s express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.&lt;/i&gt; (Copyright applies to all Abstracts.)
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        Value: 10.1111/ene.14101
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        Text: English
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      – SubjectFull: Tertiary care
        Type: general
      – SubjectFull: Arm
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      – SubjectFull: Motor neuron diseases
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      – SubjectFull: Syndromes
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      – SubjectFull: Neural conduction
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              Text: Mar2020
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