Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection

T. W. Ho, S. T. Hsieh, I. Nachamkin, H. J. Willison, K. Sheikh, J. Kiehlbauch, K. Flanigan, Justin Charles McArthur, David Cornblath, G. M. McKhann, J. W. Griffin

Research output: Contribution to journalArticle

Abstract

We investigated the possible mechanisms of paralysis and recovery in a patient with the acute motor axonal neuropathy (AMAN) pattern of the Guillain-Barre syndrome. The AMAN pattern of GBS is characterized clinically by acute paralysis without sensory involvement and electrodiagnostically by low compound motor action potential amplitudes, suggesting axonal damage, without evidence of demyelination. Many AMAN patients have serologic or culture evidence of recent Campylobacter jejuni infection. Pathologically, the most severe cases are characterized by wallerian-like degeneration of motor axons affecting the ventral roots as well as peripheral nerves, but some fatal cases have only minor changes in the roots and peripheral nerves, and some paralyzed patients with the characteristic electrodiagnostic findings of AMAN recover rapidly. The mechanism of paralysis and recovery in such cases has been uncertain. A 64-year-old woman with culture-proven Campylobacter upsaliensis diarrhea developed typical features of AMAN. She improved quickly following plasmapheresis. Her serum contained IgG anti-GM1 antibodies. The lipopolysaccharide of the organism bound peanut agglutinin. This binding was blocked by cholera toxin, suggesting that the organism contained the Gal(β1-3)GalNAc epitope of GM1 in its lipopolysaccharide. Motor-point biopsy showed denervated neuromuscular junctions and reduced fiber numbers in intramuscular nerves. In contrast, the aural nerve biopsy was normal and skin biopsy showed normal dermal and epidermal innervation. In AMAN the paralysis may reflect degeneration of motor nerve terminals and intramuscular axons. In addition, the anti-GM1 antibodies, which can bind at nodes of Ranvier, might produce failure of conduction. These processes are potentially reversible and likely to underlie the capacity for rapid recovery that characterizes some cases of AMAN.

Original languageEnglish (US)
Pages (from-to)717-724
Number of pages8
JournalNeurology
Volume48
Issue number3
StatePublished - Mar 1997

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Campylobacter Infections
Nerve Degeneration
Paralysis
Peripheral Nerves
Biopsy
Campylobacter upsaliensis
Lipopolysaccharides
Anti-Idiotypic Antibodies
Ranvier's Nodes
Wallerian Degeneration
Peanut Agglutinin
Skin
Campylobacter jejuni
Guillain-Barre Syndrome
Plasmapheresis
Spinal Nerve Roots
Neuromuscular Junction
Cholera Toxin
Presynaptic Terminals
Demyelinating Diseases

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Ho, T. W., Hsieh, S. T., Nachamkin, I., Willison, H. J., Sheikh, K., Kiehlbauch, J., ... Griffin, J. W. (1997). Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection. Neurology, 48(3), 717-724.

Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection. / Ho, T. W.; Hsieh, S. T.; Nachamkin, I.; Willison, H. J.; Sheikh, K.; Kiehlbauch, J.; Flanigan, K.; McArthur, Justin Charles; Cornblath, David; McKhann, G. M.; Griffin, J. W.

In: Neurology, Vol. 48, No. 3, 03.1997, p. 717-724.

Research output: Contribution to journalArticle

Ho, TW, Hsieh, ST, Nachamkin, I, Willison, HJ, Sheikh, K, Kiehlbauch, J, Flanigan, K, McArthur, JC, Cornblath, D, McKhann, GM & Griffin, JW 1997, 'Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection', Neurology, vol. 48, no. 3, pp. 717-724.
Ho, T. W. ; Hsieh, S. T. ; Nachamkin, I. ; Willison, H. J. ; Sheikh, K. ; Kiehlbauch, J. ; Flanigan, K. ; McArthur, Justin Charles ; Cornblath, David ; McKhann, G. M. ; Griffin, J. W. / Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection. In: Neurology. 1997 ; Vol. 48, No. 3. pp. 717-724.
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AU - Sheikh, K.

AU - Kiehlbauch, J.

AU - Flanigan, K.

AU - McArthur, Justin Charles

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