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J Neurol Neurosurg Psychiatry. 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.

Author information

  • 1Centre de Référence Maladies Rares Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France. jerome.honnorat@chu-lyon.fr

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.

PMID:
18931014
PMCID:
PMC2664637
DOI:
10.1136/jnnp.2007.138016
[PubMed - indexed for MEDLINE]
Free PMC Article
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