Survival and outcome in 73 anti-Hu positive patients with paraneoplastic encephalomyelitis/sensory neuronopathy.
In a retrospective study, we determined clinical and serological findings, associated tumours, outcome and prognostic factors in 73 Hu-Ab positive patients detected in a Dutch reference laboratory. The most frequent signs and symptoms at presentation were sensory neuropathy (55 %), cerebellar degeneration (22 %), limbic encephalitis (15 %) and brainstem encephalitis (16 %). 23 % developed autonomic dysfunction including gastro-intestinal motility disorders in 14 %. In 85 % a tumour was detected, which was a lung tumour in 77 %. Signs, symptoms and associated tumours did not differ in six patients with additional neuronal antibodies (anti-amphiphysine, anti-CV2, anti-Ri). The overall 3 months, one-year and three-year survival rates from the time of diagnosis were 64 %, 40 % and 22 %. Rankin Scale Score (RS) at diagnosis and presence of tumour at the time of diagnosis predicted mortality with hazard ratios (95 % CI) of 2.6 (1.5-4.6) and 1.5 (1.1-2). The median delay between onset of symptoms and Hu-Ab diagnosis was 4 months. There was a negative association between delay RS at diagnosis (P=0.03). In a logistic regression analysis, only older age (OR=0.15; 0.02-0.63) and a higher RS at diagnosis (OR=0.29; 0.11-0.73) were associated with a lower probability of successful functional outcome. Adjusted for these factors, antitumour therapy showed a higher but statistically not significant probability of successful outcome (OR=3.5; 0.87-14.3). Our study underlines the importance of early diagnosis and start of antitumour treatment when the patient is still in a better functional state. The delay between onset of symptoms and diagnosis of PEM/SN suggests a window for improving outcome in these patients.
Department of Neuro-oncology, University Hospital Rotterdam, P. O. Box 5201, 3008 AE Rotterdam, The Netherlands. sillevis@neuh.azr.nl
Peter
P
Sillevis Smitt
Joost
J
Grefkens
Bertie
B
de Leeuw
Martin
M
van den Bent
Wim
W
van Putten
Herbert
H
Hooijkaas
Charles
C
Vecht
D000368:Aged; D000906:Antibodies / Q000097:blood* / Q000276:immunology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D018288:Carcinoma, Small Cell / Q000150:complications / Q000453:epidemiology / Q000276:immunology; D051959:ELAV Proteins; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D008175:Lung Neoplasms / Q000150:complications / Q000453:epidemiology / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology*; D009420:Nervous System / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D009460:Neurologic Examination; D009474:Neurons / Q000276:immunology; D020364:Paraneoplastic Polyneuropathy / Q000097:blood* / Q000453:epidemiology / Q000503:physiopathology; D020361:Paraneoplastic Syndromes, Nervous System / Q000097:blood* / Q000453:epidemiology / Q000503:physiopathology; D011237:Predictive Value of Tests; D011379:Prognosis; D016601:RNA-Binding Proteins / Q000276:immunology*; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
D000906:Antibodies; D001323:Autoantibodies; D051959:ELAV Proteins; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins