Anti-Amphiphysin Stiff-Person Spectrum Disorders: A Systematic Review and Case Series Featuring an Unusual Isolated Upper-Limb Presentation.

Authors

Andrew Leslie Lee, Divyani Garg, Jie Ping Schee, Alfand Marl F Dy Closas, Jeffrey Wei Yang Tee, Emilie Sing Yee Wong, Khean Jin Goh, Mee Hoong See, Mei-Ling Sharon Tai, Ai Huey Tan, Shen-Yang Lim

Published in

Movement disorders clinical practice. Aug 14, 2026. Epub Aug 14, 2026.

Abstract

Anti-amphiphysin stiff-person spectrum disorders (SPSD) are paraneoplastic syndromes with few Asian reports and limited clinico-demographic data. We present two Asian cases alongside the largest pooled cohort analysis to date (n = 64).
Two breast adenocarcinoma-associated cases are presented. Case 1 (Chinese Malaysian) describes an unusual upper-limb stiff-limb syndrome (SLS) resolving fully following early tumor resection and intravenous immunoglobulin (IVIG). Case 2 (India) describes stiff-person syndrome (SPS) with relief from IVIG, but died from cancer progression.
Analysis of 64 cases of anti-amphiphysin SPSD revealed a stiffness hierarchy: lower limbs (37/45 = 82.2%) were most frequently affected, followed by upper limbs (57.8%), paraspinal muscles (55.6%), neck (28.9%), abdomen (26.7%), thorax (8.9%), and face (2.2%). Malignancy occurred in 93.8% (n = 60/64), the majority involving breast (73.4%, n = 47/64) or lung (14.1%, n = 9/64). SPSD symptoms preceded cancer detection in 80.0% (n = 32/40) by a median of 8 (range:1-60) months. Overall, the majority of cases (25/44 = 56.8%) had good functional outcomes (modified Rankin Scale ≤2). Good outcomes were documented in most (19/29 = 65.5%) patients receiving "triple" (symptomatic, immunomodulatory, oncological) therapy and those with oncological remission reported (10/13 = 76.9%).
Properly managed, anti-amphiphysin SPSD has good outcomes in the majority of patients, particularly when the underlying cancer is successfully treated. Atypical presentations, including upper-limb SLS, warrant high diagnostic suspicion.

PMID:
42601572
Bibliographic data and abstract were imported from PubMed on 15 Aug 2026.

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