Small fiber neuropathy common in Ehlers-Danlos

Small fiber neuropathy is a common feature of Ehlers-Danlos syndromes. – PubMed – NCBI – 2016 Jul

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 <7) in 8 patients and
  • severe (NRS ≥7) in 11 patients.

Sural nerve conduction study was normal in all patients.

All patients showed a decrease of IENFD consistent with the diagnosis of small fiber neuropathy (SFN),

regardless of the EDS type.

Conclusions:

SFN is a common feature in adults with EDS.

Skin biopsy could be considered an additional diagnostic tool to investigate pain manifestations in EDS.

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