Paraneoplastic neurological syndromes - patients' cohort profile in the Czech Republic.

Abstract

Reported paraneoplastic neurological syndromes (PNS) are rare disabling neurological diseases with supposed autoimmune pathogenesis. The aims of this study were to evaluate frequency, clinical course and therapeutic response in the cohort of PNS positive patients (n=10) in the Czech Republic for the first time. Second, we determined the presence and distribution of oligoclonal IgG bands (OB IgG) in PNS and compared the clinical and laboratory features of OB IgG positive and negative patients. A total of 2355 suspicious serum and/or CSF samples were screened by immunofluorescence and immunohistochemistry with definite confirmation by Western blot. OB IgG were detected by isoelectric focusing and immunoenzymatic staining and clinical status was scored according to modified Rankin scale (RS). Four patients had anti-Yo antibody, ovarian cancer and the score in range (2-5) on RS. Five patients had anti-Hu antibody, small cell lung cancer (SCLC), prostate cancer and the score between 1-4 grade on RS. One patient with SCLC and anti-Ri antibody had grade 2. Five of 10 patients with PNS had positive OB IgG and average value 4.2 on RS comparing with negative OB IgG patients with average value 2.6. Finally, we add well-defined cohort of PNS patients to emerging European profile of PNS and conclude that the presence of OB IgG in PNS seems to reflect enhanced immune response with more severe neurological damage and clinical course.