Onion-bulb patterns predict acquired or inherited demyelinating polyneuropathy

Jennifer A. Tracy, Peter J Dyck, Christopher Jon Klein, Ja Nean K. Engelstad, Jane E. Meyer, P. James B Dyck

Research output: Contribution to journalArticle

Abstract

Introduction: Onion-bulbs (OB) are concentrically layered Schwann-cell processes, surrounding nerve fibers, occurring in both inherited and acquired demyelinating polyneuropathies. We investigated whether OB patterns (generalized, mixed, or focal) correlate with acquired or inherited neuropathies. Methods: One hundred thirty-one OB-rich nerve biopsies were graded for OB pattern and inflammation without knowledge of clinical history. We classified inherited (n = 49) or acquired (n = 82) neuropathies based solely on clinical history. Results: Fifty-one biopsies had generalized (34 inherited vs. 17 acquired, P < 0.001), 54 mixed (48 acquired vs. 6 inherited, P < 0.001), and 26 focal/multifocal (inherited [n = 9], acquired [n = 17]) OB. Inflammation occurred more frequently in acquired (n = 54) than inherited (n = 14) neuropathy (P = 0.004). Discussion: Generalized OB correlates with inherited neuropathy; mixed OB with acquired. Inflammation occurs more in acquired neuropathy cases. OB patterns are best explained by ubiquitous Schwann-cell involvement in inherited and multifocal Schwann-cell involvement in acquired neuropathies and predict the electrophysiology of uniform demyelination in inherited and unequal demyelination in acquired neuropathies. Muscle Nerve, 2019.

Original languageEnglish (US)
JournalMuscle and Nerve
DOIs
StatePublished - Jan 1 2019

Keywords

  • chronic inflammatory demyelinating polyradiculoneuropathy
  • demyelinating neuropathy
  • hypertrophic neuropathy
  • inherited neuropathy
  • onion bulbs
  • peripheral neuropathy

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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