Abstract
A 40-year-old male developed swallowing difficulties, loss of strength, and imbalance. On admission, the patient exhibited bifacial, extremity weakness, ataxia, impaired sensation, and areflexia. Electrophysiology and nerve biopsy suggested demyelination. Spinal fluid revealed increased protein content. Plasmapheresis showed benefit, but neuropathy relapsed. At second recurrence, urine analysis showed heavy proteinuria. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). Methylprednisolone and oral cyclophosphamide were given. Long-term steroids and immunoglobulin showed steady benefit. Concurrence of chronic inflammatory demyelinating polyneuropathy and FSGS suggests synergistic cellular and humoral autoimmune mechanisms related to either cross-reactivity within antigenic targets or mimicry between neural and renal epitopes.
References
Romanick Schmiedl S, Kiprov DD, Chalmers A, Miller RG. Extraneural manifestations of chronic inflammatory demyelinating polyradiculoneuropathy. Am J Med. 1990;89:531–4.
Koller H, Schroeter M, Kieseier BC, Hartung HP. Chronic inflammatory demyelinating polyneuropathy-update on pathogenesis, diagnostic criteria and therapy. Curr Opin Neurol. 2005;18:273–8.
Olbricht CJ, Stark E, Helmchen U, et al. Glomerulonephritis associated with inflammatory demyelinating polyradiculoneuropathy: a case report and review of the literature. Nephron. 1993;64:139–41.
Henderson RD, Healy HG, Mc Combe PA, Lander CM. Chronic inflammatory demyelinating polyradiculoneuropathy and severe peripheral oedema: a renal explanation. J Clin Neurosci. 2000;72:148–9.
Smyth S, Menkes DL. Coincident membranous glomerulonephritis and chronic inflammatory demyelinating polyradiculoneuropathy: questioning the autoimmunity hypothesis. Muscle Nerve. 2008;37:130–5.
Acknowledgments
The authors thank Drs K. Susuki, M. Odaka, and K. Funakoshi (Department of Neurology, Dokkyo University School of Medicine, Tochigi, Japan) for performing antiganglioside antibody assay.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Girolami, F., Galassi, G., Furci, L. et al. Coincident chronic inflammatory demyelinating polyneuropathy and focal segmental glomerulosclerosis: a common autoimmunity?. Clin Exp Nephrol 14, 294–295 (2010). https://doi.org/10.1007/s10157-009-0259-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10157-009-0259-2