Intrathecal synthesis of anti-Hu antibodies distinguishes patients with paraneoplastic peripheral neuropathy and encephalitis.

Philipp Schwenkenbecher,Lisa Priya Chacko,Refik Pul,Kaweh Pars,Martin Stangel,Kurt-Wolfram Sühs,Thomas Skripuletz,Ulrich Wurster

doi:10.1186/s12883-016-0657-5

Philipp Schwenkenbecher, Lisa Priya Chacko + Show 6 more

Open Access

https://doi.org/10.1186/s12883-016-0657-5

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Journal: BMC Neurology	Publication Date: Aug 11, 2016
Citations: 27	License type: cc-by

Affiliation: Hannover Medical School

Abstract

BackgroundParaneoplastic syndromes are serious immune caused diseases of the peripheral and/or central nervous system associated with malignant neoplasm. Symptoms develop when antibodies against antigens expressed by tumor cells cross-react with neuronal proteins. Antineuronal antibodies are usually examined in patient’s sera while examination of the cerebrospinal fluid (CSF) often fails. Furthermore, the few previous reports describing CSF data summarized different antineuronal antibodies and/or regarded patients with different neurological symptoms as one group.MethodsWe retrospectively evaluated data of 18 patients with paraneoplastic syndromes due to anti-Hu antibodies. The study aimed to differentiate patients with peripheral neuropathy and encephalitis by cerebrospinal fluid (CSF) parameters including anti-Hu antibody titers.ResultsOur results confirm previous observations that serum titers of anti-Hu antibodies and standard CSF values do not differ between patients with neuropathy and encephalitis. However, analysis of CSF anti-Hu titers and calculating the intrathecal synthesis helped to discriminate between both groups.ConclusionIn conclusion, our results indicate that patients even with one defined antineuronal antibody need to be regarded separately depending on the involved location of the nervous system. We recommend incorporation of anti-Hu analyses in the CSF and calculating the intrathecal synthesis in patients with anti-Hu syndrome.

Highlights

Paraneoplastic syndromes are serious immune caused diseases of the peripheral and/or central nervous system associated with malignant neoplasm
Patient’s characteristics A total of 25 patients with neurological symptoms confirmative with a paraneoplastic syndrome were tested positive for anti-Hu antibodies in serum by using the immunoblot technique
By using immunohistochemistry none of the latter seven patients showed an anti-neuronal staining in the cerebellar tissue, and these patients were considered negative for anti-Hu antibodies

Summary

Introduction

Paraneoplastic syndromes are serious immune caused diseases of the peripheral and/or central nervous system associated with malignant neoplasm. Symptoms develop when antibodies against antigens expressed by tumor cells cross-react with neuronal proteins. Antineuronal antibodies are usually examined in patient’s sera while examination of the cerebrospinal fluid (CSF) often fails. The few previous reports describing CSF data summarized different antineuronal antibodies and/or regarded patients with different neurological symptoms as one group. Paraneoplastic neurological syndromes (PNS) are rare neurological disorders associated with malignant neoplasm [1]. It is widely accepted that antibodies against ectopic antigens expressed by tumor cells crossreact with antigens of the nervous system and initiate an immune cascade leading to neurodegeneration [2]. The anti-Hu antibody has been first described in 1985 in patients with small cell

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