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. 2009 Apr;80(4):412-6.
doi: 10.1136/jnnp.2007.138016. Epub 2008 Oct 17.

Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies

J Honnorat  1 , S Cartalat-Carel, D Ricard, J Ph Camdessanche, A F Carpentier, V Rogemond, F Chapuis, M Aguera, E Decullier, A M Duchemin, F Graus, J C Antoine
Affiliations

Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies

J Honnorat et al. J Neurol Neurosurg Psychiatry. 2009 Apr.

Abstract

Objective: Anti-Hu antibodies (Hu-Ab) and anti-CV2/CRMP5 antibodies (CV2/CRMP5-Ab) have been identified in association with paraneoplastic neurological disorders. However, it is not clear whether these antibodies are associated with specific neurological symptoms or are only markers of anti-cancer immune reaction.

Methods: To address this question, 37 patients with CV2/CRMP5-Ab and 324 patients with Hu-Ab were compared.

Results: Whereas the age and sex ratio were the same between the two groups, the distribution of neurological symptoms was not. Patients with CV2/CRMP5-Ab presented more frequently cerebellar ataxia, chorea, uveo/retinal symptoms and myasthenic syndrome (Lambert-Eaton myasthenic syndrome LEMS or myasthenia gravis). They also had a better Rankin score. In contrast, dysautonomia, brainstem encephalitis and peripheral neuropathy were more frequent in patients with Hu-Ab. Limbic encephalitis occurred similarly in both groups. Small-cell lung cancer was the most frequently associated tumour in both groups of patients, while malignant thymoma was observed only in patients with CV2/CRMP5-Ab. In particular, patients with CV2/CRMP5-Ab and thymoma developed myasthenic syndrome more frequently, while patients with SCLC developed neuropathies more frequently. Chorea and myasthenic syndrome were only seen in patients with CV2/CRMP5-Ab. The median survival time was significantly longer in patients with CV2/CRMP5-Ab, and this effect was not dependent on the type of tumour.

Interpretation: The data demonstrate that in patients with paraneoplastic neurological syndromes, the neurological symptoms and survival vary with both the type of associated onco-neural antibody and the type of tumour.

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Figures

Figure 1
Figure 1
Survival curve for all patients with CV2/CRMP5-Ab (CV2), Hu-Ab (Hu) or both antibodies (Hu+CV2)
Figure 2
Figure 2
Survival curve for patients with SCLC and CV2/CRMP5-Ab (CV2), Hu-Ab (Hu) or both antibodies (Hu+CV2)
See this image and copyright information in PMC

References

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