The clinical analysis of 60 cases of paraneoplastic neurological syndrome

Abstract

Objective To explore the clinical features of paraneoplastic neurological syndrome (PNS). Methods Sixty PNS patients in Peking Union Medical College Hospital from January 2008 to December 2012 according to the international diagnostic criteria for PNS recommended by Graus were diagnosed and their clinical features were retrospectively analyzed. Results Sixty PNS patients had 11 different clinical syndromes, including 29/60 (48.3%) classical syndromes and 27/60 (45.0%) non-classical syndromes. The most common classical syndromes were limbic encephalitis (11/60, 18.3%) and subacute cerebellar degeneration (10/60, 16.7%), while the most common non-classical syndrome was subacute/chronic sensorimotor neuropathies. Thirty-three of sixty (55.0%) PNS patients were found with underlying tumors, the most common of which was lung cancer (13/33, 39.4%), and 46.2% (6/13) of lung cancer was small cell lung cancer. And 19/60 (31.7%) PNS patients had well characterized onconeuronal antibodies in their serum/cerebral spinal fluid, and anti-Hu and anti-Yo antibodies were most commonly seen (16/19). No partially characterized onconeuronal antibody was found. Eight of sixty (13.3%) patients were found with neuronal cell-surface antibodies, all of which were anti-N-methyl-D-aspartate receptor antibodies. Eleven of thirty-one followed-up patients died, including 7 (7/15) definite PNS and 4 (4/16) possible PNS, without significant difference between groups (χ2=0.782 P=0.380). The 1-, 3- and 5-year survival rates were 84.0%, 61.0% and 54.0% respectively. Conclusions PNS patients with either classical or non-classical syndromes should be screened for onconeural antibodies and underlying tumors. Those patients without underlying tumor should be closely followed up. Key words: Paraneoplastic syndromes, nervous system; Antibodies, neoplasm; Retrospective studies