title: Evidence of T-cell mediated neuronal injury in stiff-person syndrome with anti-amphiphysin antibodies.
abstract: Paraneoplastic stiff-person syndrome (SPS) has been associated with antibodies against amphiphysin. Current evidence supports a pathogenic role for anti-amphiphysin antibodies. A 74-year-old female was diagnosed with amphiphysin-associated paraneoplastic stiff-person syndrome and associated encephalomyelitis. She had initial response to IVIG, however her symptoms worsened after two months and were resistant to further treatment. Subsequently the patient died and a post-mortem was performed. Neuropathology revealed perivascular and parenchymal lymphocytic infiltrates, with neuronophagia mediated by CD8+ T cells and microglia in brainstem, spinal cord, and mesial temporal lobe structures. These findings suggest a pathogenic role of cytotoxic CD8+ T-cells, with potential implication for therapy of future patients.
authors.affiliation: Department of Neurology, Prince of Wales Hospital, Barker St., Randwick, Australia. Electronic address: mervyn.poh@gmail.com.
authors.forename: Mervyn Q W
authors.initials: MQ
authors.lastname: Poh
authors.affiliation: Neuroscience Research Australia, Barker St., Randwick, Australia; Prince of Wales Clinical School, University of New South Wales, Barker St., Randwick, Australia.
authors.forename: Neil G
authors.initials: NG
authors.lastname: Simon
authors.affiliation: Neuropathology Department, Royal Prince Alfred Hospital and the University of Sydney, Sydney, Australia.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Buckland
authors.affiliation: Department of Anatomical Pathology, Prince of Wales Hospital, Barker St., Randwick, Australia.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Salisbury
authors.affiliation: Department of Neurology, Prince of Wales Hospital, Barker St., Randwick, Australia; Neuroscience Research Australia, Barker St., Randwick, Australia; Prince of Wales Clinical School, University of New South Wales, Barker St., Randwick, Australia.
authors.forename: Shaun
authors.initials: S
authors.lastname: Watson
mesh_terms: D000368:Aged; D000906:Antibodies / Q000097:blood*; D015703:Antigens, CD / Q000378:metabolism; D005260:Female; D006801:Humans; D009419:Nerve Tissue Proteins / Q000276:immunology*; D009474:Neurons / Q000473:pathology*; D016750:Stiff-Person Syndrome / Q000097:blood / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000473:pathology*
chemical_list: D000906:Antibodies; D015703:Antigens, CD; D009419:Nerve Tissue Proteins; C075637:amphiphysin
keywords: Amphiphysin; Amphiphysin antibodies; Cytotoxic T-cell; Neuronophagia; Paraneoplastic; Stiff-person syndrome