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Title of Journal: J Neurol

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Abbravation: Journal of Neurology

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Springer Berlin Heidelberg

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DOI

10.1002/chin.201518248

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1432-1459

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Smallfibre neuropathy related to bulbar and spina

Authors: A Truini A Biasiotta E Onesti G Di Stefano M Ceccanti S La Cesa A Pepe C Giordano G Cruccu M Inghilleri
Publish Date: 2015/02/17
Volume: 262, Issue: 4, Pages: 1014-1018
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Abstract

We aimed at seeking more precise diagnostic information on the sensory nervous system involvement described in patients with amyotrophic lateral sclerosis ALS We investigated large myelinated nerve fibres with nerve conduction study and smallnerve fibres with Quantitative Sensory Testing QST assessing thermalpain perceptive thresholds and skin biopsy assessing intraepidermal nerve fibre density in 24 consecutive patients with ALS 11 with bulbaronset and 13 with spinalonset In 23 of the 24 patients regardless of ALS onset nerve conduction study invariably showed large myelinated fibre sparing In patients with bulbaronset ALS QST found normal thermalpain perceptive thresholds and skin biopsy disclosed normal intraepidermal nerve fibre density Conversely in patients with spinalonset thermalpain thresholds were abnormal and distal intraepidermal nerve fibre density was reduced Sensory nervous system involvement in ALS differs according to disease onset Patients with spinalonset but not those with bulbaronset ALS have concomitant distal smallfibre neuropathy Neurologists should therefore seek this ALSrelated nonmotor feature to improve its diagnosis and treatment

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