Document Detail


Minimal change nephrotic syndrome with stiff-person syndrome: is there a link?
MedLine Citation:
PMID:  15983949     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Stiff-person syndrome is a rare, likely immune-mediated neurological disorder characterized by painful spasms and progressive symmetric rigidity of the axial and proximal limb muscles. Rigidity of truncal muscles and continuous contraction of the agonist and antagonist muscles caused by involuntary motor-unit firing at rest are the hallmarks of stiff-person syndrome. Immunosuppressive therapy has induced remission in patients with stiff-person syndrome. We report a patient with stiff-person syndrome with minimal change nephrotic syndrome (MCNS). The pathophysiologic states of stiff-person syndrome and MCNS are unclear. T-Cell-dependent mechanisms are highly suspected for the pathogenesis of both. The diagnosis of stiff-person syndrome was made on the basis of clinical and laboratory findings, and both MCNS and stiff-person syndrome resolved completely with immunosuppressive therapy. To our knowledge, this is the first case of stiff-person syndrome in association with MCNS in the literature.
Authors:
Ihsan Ergün; M Cenk Akbostanci; Başol Canbakan; Bilge Koçer; Arzu Ensari; Gökhan Nergizoglu; Kenan Keven
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of kidney diseases : the official journal of the National Kidney Foundation     Volume:  46     ISSN:  1523-6838     ISO Abbreviation:  Am. J. Kidney Dis.     Publication Date:  2005 Jul 
Date Detail:
Created Date:  2005-06-28     Completed Date:  2005-11-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8110075     Medline TA:  Am J Kidney Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e11-4     Citation Subset:  IM    
Affiliation:
Department of Nephrology, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, Turkey. ihsanerg@yahoo.com
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MeSH Terms
Descriptor/Qualifier:
Adult
Cyclophosphamide / therapeutic use
Diazepam / therapeutic use
Drug Therapy, Combination
Electromyography
Humans
Immunity, Cellular
Immunosuppressive Agents / therapeutic use
Male
Methylprednisolone / therapeutic use
Muscle Relaxants, Central / therapeutic use
Nephrosis, Lipoid / complications*,  drug therapy,  immunology
Nephrotic Syndrome / drug therapy,  etiology*,  immunology
Prednisolone / therapeutic use
Remission Induction
Stiff-Person Syndrome / diagnosis,  drug therapy,  etiology*,  immunology,  physiopathology
T-Lymphocyte Subsets / immunology
Chemical
Reg. No./Substance:
0/Immunosuppressive Agents; 0/Muscle Relaxants, Central; 439-14-5/Diazepam; 50-18-0/Cyclophosphamide; 50-24-8/Prednisolone; 83-43-2/Methylprednisolone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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