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Journal of Neurology

, Volume 262, Issue 4, pp 1014–1018 | Cite as

Small-fibre neuropathy related to bulbar and spinal-onset in patients with ALS

  • A. TruiniEmail author
  • A. Biasiotta
  • E. Onesti
  • G. Di Stefano
  • M. Ceccanti
  • S. La Cesa
  • A. Pepe
  • C. Giordano
  • G. Cruccu
  • M. Inghilleri
Original Communication

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 small-nerve fibres with Quantitative Sensory Testing (QST) (assessing thermal-pain perceptive thresholds) and skin biopsy (assessing intraepidermal nerve fibre density) in 24 consecutive patients with ALS, 11 with bulbar-onset and 13 with spinal-onset. In 23 of the 24 patients, regardless of ALS onset, nerve conduction study invariably showed large myelinated fibre sparing. In patients with bulbar-onset ALS, QST found normal thermal-pain perceptive thresholds and skin biopsy disclosed normal intraepidermal nerve fibre density. Conversely, in patients with spinal-onset, thermal-pain thresholds were abnormal and distal intraepidermal nerve fibre density was reduced. Sensory nervous system involvement in ALS differs according to disease onset. Patients with spinal-onset but not those with bulbar-onset ALS have concomitant distal small-fibre neuropathy. Neurologists should therefore seek this ALS-related non-motor feature to improve its diagnosis and treatment.

Keywords

Small-fibre neuropathy Amyotrophic lateral sclerosis Skin biopsy 

Notes

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standard

All patients gave their informed consent.

Supplementary material

415_2015_7672_MOESM1_ESM.docx (16 kb)
Supplementary material 1 (DOCX 16 kb)
415_2015_7672_MOESM2_ESM.docx (17 kb)
Supplementary material 2 (DOCX 16 kb)

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • A. Truini
    • 1
    Email author
  • A. Biasiotta
    • 1
  • E. Onesti
    • 1
  • G. Di Stefano
    • 1
  • M. Ceccanti
    • 1
  • S. La Cesa
    • 1
  • A. Pepe
    • 1
  • C. Giordano
    • 2
  • G. Cruccu
    • 1
  • M. Inghilleri
    • 1
  1. 1.Department of Neurology and PsychiatrySapienza UniversityRomeItaly
  2. 2.Department of Radiological, Oncological and Pathological SciencesSapienza UniversityRomeItaly

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