Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes

Daniele Cazzato, Marco Castori, Raffaella Lombardi, Francesca Caravello, Eleonora Dalla Bella, Antonio Petrucci, Paola Grammatico, Chiara Dordoni, Marina Colombi, Giuseppe Lauria

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

Objective: To investigate the involvement of small nerve fibers in Ehlers-Danlos syndrome (EDS). Methods: Patients diagnosed with EDS underwent clinical, neurophysiologic, and skin biopsy assessment. We recorded sensory symptoms and signs and evaluated presence and severity of neuropathic pain according to the Douleur Neuropathique 4 (DN4) and ID Pain questionnaires and the Numeric Rating Scale (NRS). Sensory action potential amplitude and conduction velocity of sural nerve was recorded. Skin biopsy was performed at distal leg and intraepidermal nerve fiber density (IENFD) obtained and referred to published sex- and age-adjusted normative reference values. Results: Our cohort included 20 adults with joint hypermobility syndrome/hypermobility EDS, 3 patients with vascular EDS, and 1 patient with classic EDS. All except one patient had neuropathic pain according to DN4 and ID Pain questionnaires and reported 7 or more symptoms at the Small Fiber Neuropathy Symptoms Inventory Questionnaire. Pain intensity was moderate (NRS ≥4 and

Original languageEnglish
Pages (from-to)155-159
Number of pages5
JournalNeurology
Volume87
Issue number2
DOIs
Publication statusPublished - Jul 12 2016

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Ehlers-Danlos Syndrome
Neuralgia
Nerve Fibers
Pain
Biopsy
Joint Instability
Sural Nerve
Skin
Action Potentials
Signs and Symptoms
Blood Vessels
Leg
Reference Values
Equipment and Supplies
Small Fiber Neuropathy
Surveys and Questionnaires

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Cazzato, D., Castori, M., Lombardi, R., Caravello, F., Bella, E. D., Petrucci, A., ... Lauria, G. (2016). Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes. Neurology, 87(2), 155-159. https://doi.org/10.1212/WNL.0000000000002847

Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes. / Cazzato, Daniele; Castori, Marco; Lombardi, Raffaella; Caravello, Francesca; Bella, Eleonora Dalla; Petrucci, Antonio; Grammatico, Paola; Dordoni, Chiara; Colombi, Marina; Lauria, Giuseppe.

In: Neurology, Vol. 87, No. 2, 12.07.2016, p. 155-159.

Research output: Contribution to journalArticle

Cazzato, D, Castori, M, Lombardi, R, Caravello, F, Bella, ED, Petrucci, A, Grammatico, P, Dordoni, C, Colombi, M & Lauria, G 2016, 'Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes', Neurology, vol. 87, no. 2, pp. 155-159. https://doi.org/10.1212/WNL.0000000000002847
Cazzato D, Castori M, Lombardi R, Caravello F, Bella ED, Petrucci A et al. Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes. Neurology. 2016 Jul 12;87(2):155-159. https://doi.org/10.1212/WNL.0000000000002847
Cazzato, Daniele ; Castori, Marco ; Lombardi, Raffaella ; Caravello, Francesca ; Bella, Eleonora Dalla ; Petrucci, Antonio ; Grammatico, Paola ; Dordoni, Chiara ; Colombi, Marina ; Lauria, Giuseppe. / Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes. In: Neurology. 2016 ; Vol. 87, No. 2. pp. 155-159.
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AU - Petrucci, Antonio

AU - Grammatico, Paola

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