TY - JOUR
T1 - GFAP IgG associated inflammatory polyneuropathy
AU - Paul, Pritikanta
AU - McKeon, Andrew
AU - Pittock, Sean J.
AU - Klein, Christopher J.
AU - Shah, Shailee
AU - Toledano, Michel
AU - Mills, John R.
AU - Dubey, Divyanshu
N1 - Publisher Copyright:
© 2020 Elsevier B.V.
PY - 2020/6/15
Y1 - 2020/6/15
N2 - Background: GFAP (glial fibrillary acidic protein)-IgG is predominantly associated with meningoencephalomyelitis, and neuropathy presentations are rare. Methods: We reviewed clinical, electrodiagnostic and histopathological presentations of GFAP-IgG associated peripheral neuropathy. Results: We identified six cases, five of whom had peripheral neuropathy as the initial presentation. Acute/subacute polyradicluoneuropathy or proximal nerve involvement was a common presentation. Three had demyelinating neuropathies on electrophysiological studies. Nerve biopsies (n = 2) demonstrated T-cell predominant perivascular inflammatory collections, and all patients with clinical follow up responded favorably to immunotherapy. Conclusion: GFAP neuropathy represents a potentially treatable immune-mediated neuropathy and can occur with or without co-existing meningoencephalomyelitis.
AB - Background: GFAP (glial fibrillary acidic protein)-IgG is predominantly associated with meningoencephalomyelitis, and neuropathy presentations are rare. Methods: We reviewed clinical, electrodiagnostic and histopathological presentations of GFAP-IgG associated peripheral neuropathy. Results: We identified six cases, five of whom had peripheral neuropathy as the initial presentation. Acute/subacute polyradicluoneuropathy or proximal nerve involvement was a common presentation. Three had demyelinating neuropathies on electrophysiological studies. Nerve biopsies (n = 2) demonstrated T-cell predominant perivascular inflammatory collections, and all patients with clinical follow up responded favorably to immunotherapy. Conclusion: GFAP neuropathy represents a potentially treatable immune-mediated neuropathy and can occur with or without co-existing meningoencephalomyelitis.
KW - Antibodies
KW - GFAP
KW - Peripheral neuropathy
KW - Polyradiculoneuropathy
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U2 - 10.1016/j.jneuroim.2020.577233
DO - 10.1016/j.jneuroim.2020.577233
M3 - Article
C2 - 32272393
AN - SCOPUS:85082718830
SN - 0165-5728
VL - 343
JO - Journal of neuroimmunology
JF - Journal of neuroimmunology
M1 - 577233
ER -