Minimal change nephrotic syndrome with stiff-person syndrome: is there a link?
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.
Ergün
Ihsan
I
Department of Nephrology, Ankara University School of Medicine, Ibni Sina Hospital, Ankara, Turkey. ihsanerg@yahoo.com
Akbostanci
M Cenk
MC
Canbakan
Başol
B
Koçer
Bilge
B
Ensari
Arzu
A
Nergizoglu
Gökhan
G
Keven
Kenan
K
D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D003975:Diazepam / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004576:Electromyography; D006801:Humans; D007111:Immunity, Cellular; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009125:Muscle Relaxants, Central / Q000627:therapeutic use; D009402:Nephrosis, Lipoid / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D016750:Stiff-Person Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000503:physiopathology; D016176:T-Lymphocyte Subsets / Q000276:immunology
D007166:Immunosuppressive Agents; D009125:Muscle Relaxants, Central; D003520:Cyclophosphamide; D011239:Prednisolone; D003975:Diazepam; D008775:Methylprednisolone