Brain, Vol 120, Issue 6 923-928, Copyright © 1997 by Oxford University Press
S Alamowitch, F Graus, M Uchuya, R Rene, E Bescansa and JY Delattre
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.
ARTICLES
Limbic encephalitis and small cell lung cancer. Clinical and immunological features
Department of Neurology, Hopital de la Salpetriere, Paris, France.
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