Seronegative paraneoplastic cerebellar degeneration: the PNS Euronetwork experience.
OBJECTIVE
To describe the characteristics of patients presenting a paraneoplastic cerebellar degeneration without classical onconeural antibodies (seronegative PCD).


METHODS
Thirty-nine seronegative PCD patients from the Paraneoplastic Neurological Syndrome Euronetwork were retrospectively analyzed and compared with 180 patients with PCD associated with classical onconeural antibodies (seropositive PCD).


RESULTS
No patient had anti-CASPR2 or anti-mGluR1 antibodies. No significant difference between the clinical characteristics of seronegative and seropositive PCD patients was observed. Yet the frequency of associated tumors was different. Lymphoma was more frequent in seronegative than in seropositive women (24% vs. 2%, P = 0.002) whilst gynecological cancer were less frequent (38% vs. 74%, P = 0.002). In comparison with seropositive men, seronegative men more frequently had a non-small-cell lung cancer (27% vs. 6%, P = 0.08) or a genitourinary cancer (22% vs. 0%, P = 0.04) but less frequently a small-cell lung cancer (23% vs. 74%, P = 0.002). Seronegative and seropositive PCD patients with similar tumors had a similar overall survival.


CONCLUSIONS
The clinical characteristics of seronegative and seropositive PCD are similar but the spectrum of associated tumors is different. The immunological scenario of seronegative PCD seems to be different from that of limbic encephalitis with only few patients harboring anti-neuropile antibodies.
French Reference Center on Paraneoplastic Neurological Syndrome, Department of Neuro-oncology, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Lyon Neuroscience Research Center INSERM U1028/CNRS UMR 5292, Lyon, France; Université de Lyon - Université Claude Bernard Lyon 1, Lyon, France.
F
F
Ducray
G
G
Demarquay
F
F
Graus
E
E
Decullier
J-C
JC
Antoine
B
B
Giometto
D
D
Psimaras
J-Y
JY
Delattre
A F
AF
Carpentier
J
J
Honnorat
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies / Q000097:blood*; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000097:blood; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology*; D020362:Paraneoplastic Cerebellar Degeneration / Q000097:blood* / Q000276:immunology*; D018091:Receptors, AMPA / Q000276:immunology*; D012189:Retrospective Studies; D012737:Sex Factors; D055815:Young Adult
D000906:Antibodies; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D018091:Receptors, AMPA; C403049:CNTNAP2 protein, human
onconeural antibodies; paraneoplastic cerebellar degeneration