Longitudinally extensive transverse myelitis associated with amphiphysin autoimmunity and breast cancer: a paraneoplastic accompaniment

Excerpt

Autoantibodies currently recognized in serum and spinal fluid as biomarkers of paraneoplastic neurological disorders include amphiphysin-IgG reactive with neuronal cytoplasmic proteins [1‐3]. Autoreactive cytotoxic T cells infiltrating both the tumor and the patient nervous system may play pathogenetic role in paraneoplastic autoimmunity, attacking target cells expressing neuronal antigens [1‐3]. Amphiphysin is a synaptic protein in the nerve terminals involved in endocytosis accessible to circulating antibodies [1‐3]. Sommer et al. [3] provided strong evidence for a direct pathogenetic role of amphiphysin in paraneoplastic stiff-person syndrome (SPS). Longitudinally extensive transverse myelitis (LETM) is a frequently devastating clinical syndrome which has come into focus for its association with neuromyelitis optica (NMO) with or without associated aquaporin-4 antibodies (AQP-4 Ab) [4]. We report the first case of AQP-4 Ab negative LETM, associated with anti-amphiphysin autoantibody in serum and CSF and histologically proven breast carcinoma. …