Atypical stiff-person syndrome with spinal MRI findings, amphiphysin autoantibodies, and immunosuppression.
In an atypical case of stiff-person syndrome (SPS), spinal T2-weighted MRI revealed a hyperintense lesion extending from C2 to C7 that corresponded with the clinical symptoms and signs. CSF showed lymphocytic pleocytosis and oligoclonal bands. Amphiphysin autoantibodies were detected in serum and CSF; however, unlike other reported cases, no malignancy occurred during a 3-year observation period. Methylprednisolone and cyclophosphamide pulse therapy led to a marked reduction of symptoms.
Schmierer
K
K
Department of Neurology, Charité University Hospital, Berlin, Germany.
Valdueza
J M
JM
Bender
A
A
DeCamilli
P
P
David
C
C
Solimena
M
M
Zschenderlein
R
R
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D001323:Autoantibodies / Q000097:blood; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage*; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology*; D013116:Spinal Cord / Q000276:immunology / Q000503:physiopathology*; D016750:Stiff-Person Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology*
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D009419:Nerve Tissue Proteins; C075637:amphiphysin; D008775:Methylprednisolone