Open Access Case report

Paraneoplastic cerebellar degeneration associated with lymphoepithelial carcinoma of the tonsil

Christian Henke1*, Johannes Rieger2, Sylvia Hartmann3, Marcus Middendorp4, Helmuth Steinmetz1 and Ulf Ziemann15

Author Affiliations

1 Department of Neurology, Goethe University Frankfurt, Theodor-Stern-Kai 7, 60590 Frankfurt, Germany

2 Institute of Neurooncology, Goethe University Frankfurt, Senckenberganlage 31, 60325 Frankfurtm, Germany

3 Institute of Pathology, Goethe University Frankfurt, Senckenberganlage 31, 60325 Frankfurtm, Germany

4 Department of Nuclear Medicine, Goethe University Frankfurt, Senckenberganlage 31, 60325 Frankfurtm, Germany

5 Department of Neurology and Stroke, Hertie Institute for Clinical Brain Research, Eberhard-Karls-University Tübingen, 72076 Tübingen, Germany

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BMC Neurology 2013, 13:147  doi:10.1186/1471-2377-13-147

Published: 17 October 2013

Abstract

Background

Paraneoplastic cerebellar degeneration (PCD) is a classical tumor-associated, immune-mediated disease typically associated with gynecological malignancies, small-cell lung-cancer or lymphoma.

Case presentation

Here we present the case of a 38-year old male with an over 12 months rapidly progressive cerebellar syndrome. Extensive diagnostic workup revealed selective hypermetabolism of the right tonsil in whole-body PET. Histological examination after tonsillectomy demonstrated a lymphoepithelial carcinoma of the tonsil and the tongue base strongly suggesting a paraneoplastic cause of the cerebellar syndrome. To the best of our knowledge this is the first case of an association of a lymphoepithelial carcinoma, a rare pharyngeal tumor, with PCD.

Conclusions

In cases of classical paraneoplastic syndromes an extensive search for neoplasms should be performed including whole-body PET to detect tumors early in the course of the disease.

Keywords:
Lymphoepithelioma; Paraneoplastic syndrome; PET; Subacute cerebellar degeneration; Tonsil