Acute-onset chronic inflammatory demyelinating polyneuropathy with focal segmental glomerulosclerosis

• Published in: Journal of the neurological sciences Vol. 341; no. 1; pp. 139 - 143
• Main Authors: Quek, Amy May Lin, Soon, Derek, Chan, Yee Cheun, Thamboo, Thomas Paulraj, Yuki, Nobuhiro
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15.06.2014
• Subjects: Autoimmune disease; Chronic inflammatory demyelinating polyneuropathy; Electric Stimulation; Female; Focal segmental glomerulosclerosis; Glomerulosclerosis, Focal Segmental - complications; Glomerulosclerosis, Focal Segmental - pathology; Guillain–Barré syndrome; Humans; Middle Aged; Nephrotic syndrome; Neural Conduction - physiology; Neurology; Peripheral Nerves - physiopathology; Peripheral neuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - complications; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - pathology; Reaction Time - physiology; Time Factors; Autoimmune disease; Guillain–Barré syndrome; Nephrotic syndrome; Focal segmental glomerulosclerosis; Peripheral neuropathy; Chronic inflammatory demyelinating polyneuropathy
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2014.03.040

Abstract Inflammatory neuropathies have been reported to occur in association with nephrotic syndrome. Their underlying immuno-pathogenic mechanisms remain unknown. A 50-year-old woman concurrently presented with acute-onset chronic inflammatory demyelinating polyneuropathy and nephrotic syndrome secondary to focal segmental glomerulosclerosis. Both neuropathy and proteinuria improved after plasma exchange and steroids. Literature review of cases of concurrent inflammatory neuropathies and nephrotic syndrome revealed similar neuro-renal presentations. This neuro-renal condition may be mediated by autoantibodies targeting myelin and podocytes.