Progressive Encephalomyelitis with Rigidity and Myoclonus: A Syndrome with Diverse Clinical Features and Antibody Responses

Shugaiv E., Leite M. I., Sehitoglu E., Woodhall M., Cavus F., Waters P., ...Daha Fazla

EUROPEAN NEUROLOGY, sa.5, ss.257-262, 2013 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Basım Tarihi: 2013
  • Doi Numarası: 10.1159/000342237
  • Dergi Adı: EUROPEAN NEUROLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.257-262
  • Anahtar Kelimeler: Progressive encephalomyelitis with rigidity and myoclonus, Autoimmune encephalitis, Paraneoplastic, Protein macroarray, Antibody, Autoimmunity, LIMBIC ENCEPHALITIS, PROTEINS, DISEASES, GLYCINE
  • İstanbul Üniversitesi Adresli: Evet

Özet

Background/Aims: To better characterize progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome and identify novel PERM phenotypes. Methods: The clinical features and antibody status of PERM patients were investigated using immunoblots, cell-based assays, RIA, protein macroarray and ELISA. Results: Two patients with supratentorial involvement showed abnormal PET or EEG findings. One patient was discovered to have renal cell carcinoma, and protein macroarray revealed Ma3-antibodies. Another patient with leucine-rich, glioma-inactivated 1 (LGI1) and glutamic acid decarboxylase (GAD) antibodies showed a good response to immunotherapy. Conclusion: The heterogeneity of the immunological features suggests that PERM is caused by diverse pathogenic mechanisms. Seropositivity to well-characterized neuronal cell surface antigens might indicate a good treatment response. Copyright (C) 2013 S. Karger AG, Basel