Pritikanta Paul1, Andrew McKeon2, Sean J Pittock2, Christopher J Klein2, Shailee Shah3, Michel Toledano1, John R Mills4, Divyanshu Dubey5. 1. Department of Neurology, Mayo Clinic, Rochester, MN, United States of America. 2. Department of Neurology, Mayo Clinic, Rochester, MN, United States of America; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United States of America; Center of Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, United States of America. 3. Department of Neurology, Mayo Clinic, Rochester, MN, United States of America; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United States of America; Center of Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, United States of America; Northwestern Feinberg School of Medicine, Chicago, IL, United States of America. 4. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United States of America. 5. Department of Neurology, Mayo Clinic, Rochester, MN, United States of America; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United States of America. Electronic address: Dubey.Divyanshu@mayo.edu.
Abstract
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.
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:GFAPneuropathy represents a potentially treatable immune-mediated neuropathy and can occur with or without co-existing meningoencephalomyelitis.