| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the Department of Neurology (Drs. Lu, Wu, Zhang, and Jiang), Beijing Children’s Hospital, Beijing, P.R.C.; the Department of Neurology (Drs. Sheikh, Cornblath, McKhann, Griffin, and Ho), Johns Hopkins University School of Medicine, Baltimore, MD; the Johns Hopkins University Zanvyl Krieger Mind-Brain Institute (Dr. McKhann), Baltimore, MD; and the Department of Neurology (Dr. Asbury), University of Pennsylvania School of Medicine, Philadelphia, PA.
Address correspondence and reprint requests to Dr. Tony W. Ho, Department of Neurology, Johns Hopkins Hospital, Pathology 509, 600 N. Wolfe St., Baltimore, MD 21287; e-mail: Tony{at}jhmi.edu
OBJECTIVE: To correlate electrophysiologic patterns with sural nerve pathology in children with Guillain–Barré syndrome (GBS).
BACKGROUND: Based on electrophysiologic and pathologic observations, GBS has been divided into demyelinating and axonal subtypes. The acute motor axonal neuropathy (AMAN) involves predominantly motor nerve fibers with a physiologic pattern suggesting axonal damage, whereas the acute inflammatory demyelinating polyneuropathy (AIDP) involves both motor and sensory nerve fibers with a physiologic pattern suggesting demyelination. In this study, we sought to confirm these observations by correlating sural nerve pathology with electrophysiologic findings in GBS patients.
METHODS: Biopsies of sural nerve from 29 of 50 prospectively studied GBS patients were obtained. Nerves were examined by light and electron microscopy, and with immunocytochemistry for macrophages, lymphocytes, and complement activation products.
RESULTS: Sural nerves from AMAN patients were normal or had only a few (0.1% to 0.7%) degenerating fibers without lymphocytic infiltration or complement activation. One patient with reduced sural sensory nerve action potential classified as acute motor sensory axonal neuropathy (AMSAN) had many degenerating fibers (2.3%) in the sural nerve. All three AIDP patients displayed active demyelination, and in two patients, lymphocytic infiltration and complement activation products were observed on the abaxonal Schwann cell surface.
CONCLUSION: Classification of Guillain–Barré syndrome subtypes based on motor conduction studies correlates closely with pathologic changes seen in sural nerve. In acute motor axonal neuropathy cases, the sural nerve is almost completely spared pathologically. In acute inflammatory demyelinating polyneuropathy cases, macrophage-mediated demyelination and lymphocytic infiltration are common in the biopsies of sural nerves.
Key words: Children—Guillain–Barré syndrome—Sural nerve biopsy—Electrodiagnosis—Acute motor axonal neuropathy—Acute inflammatory demyelinating neuropathy
This article has been cited by other articles:
|
|
 |
|
  S Agrawal, D Peake, and W P Whitehouse Management of children with Guillain-Barre syndrome Arch. Dis. Child. Ed. Pract., December 1, 2007; 92(6): 161 - 168. [Full Text] [PDF]
|
|
|
|
 |
|
 J. T. Sladky Guillain-Barre syndrome: Blind men describe an elephant? Neurology, October 23, 2007; 69(17): 1647 - 1649. [Full Text] [PDF]
|
|
|
|
 |
|
 M. R. Molinero, D. Varon, K. R. Holden, J. T. Sladky, I. B. Molina, and F. Cleaves Epidemiology of Childhood Guillain-Barre Syndrome as a Cause of Acute Flaccid Paralysis in Honduras: 1989--1999 J Child Neurol, November 1, 2003; 18(11): 741 - 747. [Abstract] [PDF]
|
|
|
|
|
|
J. Berciano, T. W. Ho, and J. W. Griffin Physiologic-pathologic correlation in Guillain-Barre syndrome in children Neurology, December 12, 2000; 55(11): 1762 - 1762. [Full Text]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
