Limbic encephalitis and small cell lung cancer. Clinical and immunological features.
Paraneoplastic limbic encephalitis (LE) is considered a particular manifestation of paraneoplastic encephalomyelitis (PEM), a remote effect of cancer almost always associated with anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinoma (SCLC). In order to define the frequency of anti-Hu antibodies in LE with SCLC and to analyse possible clinical differences between patients with and without anti-Hu antibodies, the charts of 16 patients with LE and SCLC were reviewed. Eight patients (50%) had anti-Hu antibodies (anti-Hu+) whereas eight patients (50%) had no detectable anti-neuronal antibodies (anti-Hu-). The clinical and laboratory features of LE and time to diagnosis of SCLC were similar in the anti-Hu+ and anti-Hu- groups. Involvement of other areas of the nervous system compatible with the diagnosis of PEM was observed in seven (87.5%) patients of the anti-Hu+ group but in only one (12.5%) of the anti-Hu- group (P = 0.012). Five patients, including four of the anti-Hu- group, had a partial improvement of the LE after treatment of the SCLC. Another anti-Hu- patient improved spontaneously. Six patients of the anti-Hu+ group died from the neurological disorder, whereas in the anti-Hu- group the cause of death was progression of the SCLC in the three patients who died. The results of this study indicate that the absence of anti-Hu antibodies does not rule out the presence of an underlying SCLC in patients with a clinical diagnosis of LE. Patients with LE and SCLC who are without anti-Hu antibodies are less likely to develop PEM and seem to improve more often after treatment of the cancer than those who present anti-Hu antibodies.
Alamowitch
S
S
Department of Neurology, Hôpital de la Salpêtrière, Paris, France.
Graus
F
F
Uchuya
M
M
Reñé
R
R
Bescansa
E
E
Delattre
J Y
JY
D000888:Antibodies, Anti-Idiotypic / Q000097:blood / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D018288:Carcinoma, Small Cell / Q000150:complications / Q000276:immunology* / Q000401:mortality; D002555:Cerebrospinal Fluid / Q000166:cytology; D051959:ELAV Proteins; D004660:Encephalitis / Q000175:diagnosis / Q000209:etiology / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015151:Immunoblotting; D007165:Immunosuppression Therapy; D008032:Limbic System; D008175:Lung Neoplasms / Q000150:complications / Q000276:immunology* / Q000401:mortality; D008214:Lymphocytes / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000209:etiology / Q000276:immunology*; D016601:RNA-Binding Proteins / Q000097:blood / Q000276:immunology; D012189:Retrospective Studies
D000888:Antibodies, Anti-Idiotypic; D001323:Autoantibodies; D051959:ELAV Proteins; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins