K. Schmierer et al., ATYPICAL STIFF-PERSON SYNDROME WITH SPINAL MRI FINDINGS, AMPHIPHYSIN AUTOANTIBODIES, AND IMMUNOSUPPRESSION, Neurology, 51(1), 1998, pp. 250-252
In an atypical case of stiff-person syndrome (SPS), spinal T2-weighted
MRI revealed a hyperintense lesion extending from C2 to C7 that corre
sponded with the clinical symptoms and signs. CSF showed lymphocytic p
leocytosis and oligoclonal bands. Amphiphysin autoantibodies were dete
cted In serum and CSF; however, unlike other reported cases, no malign
ancy occurred during a 3-year observation period. Methylprednisolone a
nd cyclophosphamide pulse therapy led to a marked reduction of symptom
s.