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Case Reports
. 2014 Feb 15;337(1-2):235-7.
doi: 10.1016/j.jns.2013.12.015. Epub 2013 Dec 15.

Evidence of T-cell Mediated Neuronal Injury in Stiff-Person Syndrome With Anti-Amphiphysin Antibodies

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Case Reports

Evidence of T-cell Mediated Neuronal Injury in Stiff-Person Syndrome With Anti-Amphiphysin Antibodies

Mervyn Q W Poh et al. J Neurol Sci. .

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.

Keywords: Amphiphysin; Amphiphysin antibodies; Cytotoxic T-cell; Neuronophagia; Paraneoplastic; Stiff-person syndrome.

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