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Antibody Transfection into Neurons as a Tool to Study Disease Pathogenesis
Published on: September 26, 2012
Amphiphysin-IgG autoimmune neuropathy: A recognizable clinicopathologic syndrome
Divyanshu Dubey1, Jiraporn Jitprapaikulsan1, Hongyan Bi1
1From the Department of Neurology (D.D., J.J., H.B., R.V.D.C., A.M., S.J.P., J.K.E., C.J.K.), Laboratory Medicine and Pathology (D.D., J.J., A.M., S.J.P., J.R.M., C.J.K.), Center for MS and Autoimmune Neurology (S.J.P.), Mayo Clinic, Rochester, MN; Beijing Friendship Hospital (H.B.), Capital Medical University, China; and Siriraj Hospital, Mahidol University (J.J.), Bangkok, Thailand.
Objective:
To define the clinicopathologic features of amphiphysin-immunoglobulin G (IgG)-mediated neuropathy.
Methods:
Patients examined at our institution from January 1, 1995, to September 30, 2018, with amphiphysin-IgG by indirect immunofluorescence and Western blot, were reviewed. Their phenotypes were compared to cases of coexisting collapsin response-mediator protein-5 (CRMP5)-IgG or anti-neuronal nuclear antibody type 1 (ANNA1-IgG) and CRMP5-IgG autoimmunity. Improvement in modified Rankin Scale (mRS) (≥1) on follow-up was considered a favorable outcome. Amphiphysin RNA expression was assessed in healthy nerves.
Results:
Fifty-three amphiphysin-IgG-positive cases were identified. Of 33 (60%) patients with neuropathy, 21 had amphiphysin-IgG alone, and 12 had coexisting autoantibodies (ANNA1-IgG, n = 8; CRMP5-IgG, n = 2; ANNA1-IgG and CRMP5-IgG, n = 2). The neuropathies in isolated amphiphysin-IgG autoimmunity included polyradiculoneuropathy (62%), diffuse sensory neuronopathy (35%), and facial neuropathy with gastroparesis (3%). Among these, pain (80%), breast cancer (63%), and CNS (57%) involvements commonly coexisted, and neuropathy frequently prompted breast cancer diagnosis (76%). Stiff-person spectrum disorder was the most common CNS accompaniment (45%). Nerve biopsies showed axonal loss (n = 6/6), subperineurial edema (n = 4/6), and CD4 predominant inflammation (n = 2/6). Median mRS score at last follow-up was 3.5; 58% of patients were immunotherapy-responsive. Patients with amphiphysin-IgG alone had more favorable immunotherapy response than patients with CRMP5-IgG polyneuropathy (n = 45) (44% vs 16%, p = 0.028, odds ratio 4.2, 95% confidence interval 1.1 to 15.5). Only 1/9 (11%) patients with amphiphysin-IgG with coexisting CRMP5-IgG or ANNA1-IgG had immunotherapy response. RNA amphiphysin expression occurred at low levels in nerve.
Conclusion:
Amphiphysin-IgG autoimmune neuropathy has a recognizable phenotype, is frequently immune responsive, and can prompt early diagnosis of breast cancer.
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