Stiff-person syndrome with amphiphysin antibodies: distinctive features of a rare disease.

ABSTRACT:

Background

Stiff-person syndrome (SPS), formerly Stiff-man syndrome, is a rare autoimmune disease usually exhibiting severe spasms and thoracolumbar stiffness, with very elevated glutamic acid decarboxylase antibodies (GAD Ab). A paraneoplastic variant, less well characterized, is associated with amphiphysin antibodies (amphiphysin Ab). The objective of this study was to identify distinctive clinical features of amphiphysin Ab-associated SPS.

Methods

Records associated with 845 sera tested in the Yale SPS project were examined, and 621 patients with clinically suspected SPS were included in the study. Clinical characteristics were assessed with correction for multiple comparisons.

Results

In all, 116 patients had GAD antibodies and 11 patients had amphiphysin Ab; some

SUBMITTER: Murinson BB 

PROVIDER: S-EPMC2676978 | biostudies-literature | 2008 Dec

REPOSITORIES: biostudies-literature