Skip to main content

Advertising Disclaimer »

Main menu

  • Journals
    • Pediatrics
    • Hospital Pediatrics
    • Pediatrics in Review
    • NeoReviews
    • AAP Grand Rounds
    • AAP News
  • Authors/Reviewers
    • Submit Manuscript
    • Author Guidelines
    • Reviewer Guidelines
    • Open Access
    • Editorial Policies
  • Content
    • Current Issue
    • Online First
    • Archive
    • Blogs
    • Topic/Program Collections
    • AAP Meeting Abstracts
  • Pediatric Collections
    • COVID-19
    • Racism and Its Effects on Pediatric Health
    • More Collections...
  • AAP Policy
  • Supplements
  • Multimedia
    • Video Abstracts
    • Pediatrics On Call Podcast
  • Subscribe
  • Alerts
  • Careers
  • Other Publications
    • American Academy of Pediatrics

User menu

  • Log in

Search

  • Advanced search
American Academy of Pediatrics

AAP Gateway

Advanced Search

AAP Logo

  • Log in
  • Journals
    • Pediatrics
    • Hospital Pediatrics
    • Pediatrics in Review
    • NeoReviews
    • AAP Grand Rounds
    • AAP News
  • Authors/Reviewers
    • Submit Manuscript
    • Author Guidelines
    • Reviewer Guidelines
    • Open Access
    • Editorial Policies
  • Content
    • Current Issue
    • Online First
    • Archive
    • Blogs
    • Topic/Program Collections
    • AAP Meeting Abstracts
  • Pediatric Collections
    • COVID-19
    • Racism and Its Effects on Pediatric Health
    • More Collections...
  • AAP Policy
  • Supplements
  • Multimedia
    • Video Abstracts
    • Pediatrics On Call Podcast
  • Subscribe
  • Alerts
  • Careers

Discover Pediatric Collections on COVID-19 and Racism and Its Effects on Pediatric Health

American Academy of Pediatrics
Article

Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes

Anne Louise Oaklander and Max M. Klein
Pediatrics April 2013, 131 (4) e1091-e1100; DOI: https://doi.org/10.1542/peds.2012-2597
Anne Louise Oaklander
aDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts; and
bDepartment of Pathology (Neuropathology), Massachusetts General Hospital, Boston Massachusetts
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Max M. Klein
aDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts; and
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • Comments
Loading

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Download PDF

Abstract

OBJECTIVE: We tested the hypothesis that acquired small-fiber polyneuropathy (SFPN), previously uncharacterized in children, contributes to unexplained pediatric widespread pain syndromes.

METHODS: Forty-one consecutive patients evaluated for unexplained widespread pain beginning before age 21 had medical records comprehensively analyzed regarding objective diagnostic testing for SFPN (neurodiagnostic skin biopsy, nerve biopsy, and autonomic function testing), plus histories, symptoms, signs, other tests, and treatments. Healthy, demographically matched volunteers provided normal controls for SFPN tests.

RESULTS: Age at illness onset averaged 12.3 ± 5.7 years; 73% among this poly-ethnic sample were female (P = .001). Sixty-eight percent were chronically disabled, and 68% had hospitalizations. Objective testing diagnosed definite SFPN in 59%, probable SFPN in 17%, and possible SFPN in 22%. Only 1 of 41 had entirely normal SFPN test results. Ninety-eight percent of patients had other somatic complaints consistent with SFPN dysautonomia (90% cardiovascular, 82% gastrointestinal, and 34% urologic), 83% reported chronic fatigue, and 63% had chronic headache. Neurologic examinations identified reduced sensation in 68% and vasomotor abnormalities in 55%, including 23% with erythromelalgia. Exhaustive investigations for SFPN causality identified only history of autoimmune illnesses in 33% and serologic markers of disordered immunity in 89%. Treatment with corticosteroids and/or intravenous immune globulin objectively and subjectively benefited 80% of patients (12/15).

CONCLUSIONS: More than half among a large series of patients with childhood-onset, unexplained chronic widespread pain met rigorous, multitest, diagnostic criteria for SFPN, which extends the age range of acquired SFPN into early childhood. Some cases appeared immune-mediated and improved with immunomodulatory therapies.

  • peripheral nervous system disease
  • widespread chronic pain
  • dysautonomia
  • Abbreviations:
    AFT —
    autonomic function testing
    CIDP —
    chronic inflammatory demyelinating polyneuropathy
    CWP —
    chronic widespread pain
    ENF —
    epidermal nerve fiber
    ESR —
    erythrocyte sedimentation rate
    GBS —
    Guillain-Barré syndrome, (acute inflammatory demyelinating polyneuropathy)
    IVIG —
    intravenous immune globulin
    POTS —
    postural orthostasis tachycardia syndrome
    RR —
    reference range (of normal values for laboratory tests)
    SFPN —
    small-fiber polyneuropathy
    • Accepted December 12, 2012.
    • Copyright © 2013 by the American Academy of Pediatrics

    Individual Login

    Log in
    You will be redirected to aap.org to login or to create your account.

    Institutional Login

    via Institution

    You may be able to gain access using your login credentials for your institution. Contact your librarian or administrator if you do not have a username and password.

    Log in through your institution

    If your organization uses OpenAthens, you can log in using your OpenAthens username and password. To check if your institution is supported, please see this list. Contact your library for more details.

    Pay Per Article - You may access this article (from the computer you are currently using) for 2 days for US$25.00

    Regain Access - You can regain access to a recent Pay per Article purchase if your access period has not yet expired.

    Offer Reprints

    PreviousNext
    Back to top

    Advertising Disclaimer »

    In this issue

    Pediatrics
    Vol. 131, Issue 4
    1 Apr 2013
    • Table of Contents
    • Index by author
    View this article with LENS
    PreviousNext
    Email Article

    Thank you for your interest in spreading the word on American Academy of Pediatrics.

    NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

    Enter multiple addresses on separate lines or separate them with commas.
    Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes
    (Your Name) has sent you a message from American Academy of Pediatrics
    (Your Name) thought you would like to see the American Academy of Pediatrics web site.
    CAPTCHA
    This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
    Request Permissions
    Article Alerts
    Log in
    You will be redirected to aap.org to login or to create your account.
    Or Sign In to Email Alerts with your Email Address
    Citation Tools
    Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes
    Anne Louise Oaklander, Max M. Klein
    Pediatrics Apr 2013, 131 (4) e1091-e1100; DOI: 10.1542/peds.2012-2597

    Citation Manager Formats

    • BibTeX
    • Bookends
    • EasyBib
    • EndNote (tagged)
    • EndNote 8 (xml)
    • Medlars
    • Mendeley
    • Papers
    • RefWorks Tagged
    • Ref Manager
    • RIS
    • Zotero
    Share
    Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes
    Anne Louise Oaklander, Max M. Klein
    Pediatrics Apr 2013, 131 (4) e1091-e1100; DOI: 10.1542/peds.2012-2597
    del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
    Print
    Download PDF
    Insight Alerts
    • Table of Contents

    Jump to section

    • Article
      • Abstract
      • Methods
      • Results
      • Discussion
      • Conclusions
      • Acknowledgments
      • Footnotes
      • References
    • Figures & Data
    • Info & Metrics
    • Comments

    Related Articles

    • No related articles found.
    • PubMed
    • Google Scholar

    Cited By...

    • Diagnosis and management of sensory polyneuropathy
    • Small Fiber Neuropathy in Children: Two Case Reports Illustrating the Importance of Recognition
    • Google Scholar

    More in this TOC Section

    • Neighborhood Child Opportunity Index and Adolescent Cardiometabolic Risk
    • Neonates Born to Mothers With COVID-19: Data From the Spanish Society of Neonatology Registry
    • Comparison of Manual and Automated Sepsis Screening Tools in a Pediatric Emergency Department
    Show more Article

    Similar Articles

    Subjects

    • Neurology
      • Neurology
      • Neurologic Disorders

    Keywords

    • peripheral nervous system disease
    • widespread chronic pain
    • dysautonomia
    • Journal Info
    • Editorial Board
    • Editorial Policies
    • Overview
    • Licensing Information
    • Authors/Reviewers
    • Author Guidelines
    • Submit My Manuscript
    • Open Access
    • Reviewer Guidelines
    • Librarians
    • Institutional Subscriptions
    • Usage Stats
    • Support
    • Contact Us
    • Subscribe
    • Resources
    • Media Kit
    • About
    • International Access
    • Terms of Use
    • Privacy Statement
    • FAQ
    • AAP.org
    • shopAAP
    • Follow American Academy of Pediatrics on Instagram
    • Visit American Academy of Pediatrics on Facebook
    • Follow American Academy of Pediatrics on Twitter
    • Follow American Academy of Pediatrics on Youtube
    • RSS
    American Academy of Pediatrics

    © 2021 American Academy of Pediatrics