Chinese Paralytic Syndrome (CPS) is a recently described form of acute onset flaccid paralysis, primarily involving children in rural areas of Northern China. The review of over 3000 cases and personal examination of 88 patients indicates that this is a non-inflammatory motor neuropathy with distinct epidemiologic, clinical, physiologic, and pathologic features. These preliminary studies indicate that CPS differs from poliomyelitis and the demyelinative form of the Guillain-Barre Syndrome (GBS). In addition, serologic studies suggest an association with Campylobacter jejuni. To amplify our preliminary studies, the following specific aims are proposed. In collaboration with our Chinese colleagues, Aim 1 is designed to study prospectively and longitudinally, 50 patients and suitable controls in terms of clinical progression, electrophysiologic alterations, and serum antibody responses; to evaluate the pathophysiology of weakness and recovery; and to determine the incidence of the disease in a separate, defined population.
Aim 2 is designed to establish definitively how CPS differs from other forms of acute onset flaccid paralysis. Lastly, Aim 3 seeks to uncover underlying mechanisms such as the role of C. jejuni and host immunologic responses. Surveillance campaigns for poliomyelitis indicate that there are significant numbers of children with acute onset flaccid paralysis who do not have polio. It is our hypothesis that a disorder similar, if not identical, to CPS occurs with a high incidence in other developing nations, particularly in Latin America.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
1R01NS031528-01
Application #
3418463
Study Section
Neurology A Study Section (NEUA)
Project Start
1993-05-01
Project End
1996-04-30
Budget Start
1993-05-01
Budget End
1994-04-30
Support Year
1
Fiscal Year
1993
Total Cost
Indirect Cost
Name
Johns Hopkins University
Department
Type
Schools of Medicine
DUNS #
045911138
City
Baltimore
State
MD
Country
United States
Zip Code
21218
Jacobs, Bart C; Koga, Michiaki; van Rijs, Wouter et al. (2008) Subclass IgG to motor gangliosides related to infection and clinical course in Guillain-Barre syndrome. J Neuroimmunol 194:181-90
Nachamkin, I; Arzarte Barbosa, P; Barbosa, P Arzate et al. (2007) Patterns of Guillain-Barre syndrome in children: results from a Mexican population. Neurology 69:1665-71
Perera, Viraj N; Nachamkin, Irving; Ung, Huong et al. (2007) Molecular mimicry in Campylobacter jejuni: role of the lipo-oligosaccharide core oligosaccharide in inducing anti-ganglioside antibodies. FEMS Immunol Med Microbiol 50:27-36
Boffey, Judith; Odaka, Masaaki; Nicoll, Dawn et al. (2005) Characterisation of the immunoglobulin variable region gene usage encoding the murine anti-ganglioside antibody repertoire. J Neuroimmunol 165:92-103
Leonard 2nd, Edward E; Tompkins, Lucy S; Falkow, Stanley et al. (2004) Comparison of Campylobacter jejuni isolates implicated in Guillain-Barre syndrome and strains that cause enteritis by a DNA microarray. Infect Immun 72:1199-203
Boffey, Judith; Nicholl, Dawn; Wagner, Eric R et al. (2004) Innate murine B cells produce anti-disialosyl antibodies reactive with Campylobacter jejuni LPS and gangliosides that are polyreactive and encoded by a restricted set of unmutated V genes. J Neuroimmunol 152:98-111
Magira, Eleni E; Papaioakim, Miltiadis; Nachamkin, Irving et al. (2003) Differential distribution of HLA-DQ beta/DR beta epitopes in the two forms of Guillain-Barre syndrome, acute motor axonal neuropathy and acute inflammatory demyelinating polyneuropathy (AIDP): identification of DQ beta epitopes associated with susceptibil J Immunol 170:3074-80
Bowes, Tyrone; Wagner, Eric R; Boffey, Judith et al. (2002) Tolerance to self gangliosides is the major factor restricting the antibody response to lipopolysaccharide core oligosaccharides in Campylobacter jejuni strains associated with Guillain-Barre syndrome. Infect Immun 70:5008-18
Gong, Y; Tagawa, Y; Lunn, M P T et al. (2002) Localization of major gangliosides in the PNS: implications for immune neuropathies. Brain 125:2491-506
Nachamkin, Irving; Liu, Jirong; Li, Ming et al. (2002) Campylobacter jejuni from patients with Guillain-Barre syndrome preferentially expresses a GD(1a)-like epitope. Infect Immun 70:5299-303

Showing the most recent 10 out of 27 publications