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Acute-onset small fiber neuropathy (AOSFN) often presents as painful, non-length-dependent neuropathy. Immune system involvement is suggested by in vitro studies, with many patients showing improvement after corticosteroid treatment.

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Area of Science:

  • Neurology
  • Immunology
  • Neuroscience

Background:

  • Immune-mediated small fiber neuropathy (SFN) is increasingly recognized, but acute-onset SFN (AOSFN) is poorly described.
  • This study investigates a series of AOSFN cases where immune origins are debated.

Purpose of the Study:

  • To describe the clinical characteristics, diagnostic findings, and disease course of patients with acute-onset small fiber neuropathy.
  • To explore potential immune-mediated mechanisms in AOSFN.

Main Methods:

  • Included consecutive patients with probable or definite AOSFN diagnosed by NEURODIAB criteria, with symptom onset within 28 days.
  • Utilized clinical examination, nerve conduction studies, laser-evoked potentials (LEPs), warm detection thresholds (WDTs), electrochemical skin conductance (ESC), epidermal nerve fiber density (ENF), and serum reactivity assays.
  • Analyzed serum reactivity against nerve tissue and compared with healthy and diseased controls.

Main Results:

  • Twenty patients (60% women, median age 44.2 years) presented with AOSFN, predominantly non-length-dependent (85%) and painful (85%), with 60% experiencing autonomic involvement.
  • Diagnosis was confirmed by abnormal LEPs (60%), ENF (55%), WDT (39%), or ESC (31%).
  • In vitro studies revealed IgG immunoreactivity against nerve tissue in 70% of patients, with antibodies binding to various nerve components. Patients showed improvement with corticosteroids (3/3), and 65% achieved partial or complete recovery.

Conclusions:

  • Acute-onset small fiber neuropathy typically manifests as an acute, non-length-dependent, symmetric, painful neuropathy with a variable clinical course.
  • In vitro immunohistochemical studies suggest a potential immune-mediated origin for AOSFN.
  • Early treatment with corticosteroids may lead to favorable outcomes in some AOSFN patients.