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Subacute sensory neuronopathy associated with Merkel cell carcinoma with unknown primary: a case report with literature review

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Abstract

Paraneoplastic neurologic syndromes (PNSs) are a wide spectrum of neurologic diseases characterized by different clinical features, associated with a neoplasia, and triggered by an immune-mediated process. In most cases, it is possible to detect specific neuronal antibodies and the Hu protein is one of the most frequently recognized intracellular antigens in patients with PNSs. Small-cell lung cancer is the most common cancer associated with PNSs, followed by urological, gynecological and hematological malignancies. Otherwise, extra-pulmonary small-cell carcinomas, including Merkel cell carcinoma (MCC), have been rarely described as related to PNSs. In this article we report, for the first time in the published literature, a case of anti-Hu antibody-related subacute sensory neuronopathy in association with MCC.

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Fig. 1
Fig. 2

taken from the inguinal lymph node. Solid sheets of neoplastic cells with dark nuclei and high N/C ratio can be appreciated (A) in the hematoxylin and eosin staining. The tumoral cells resulted immunoreactive to cytokeratin AE1/AE3 (B) and neurofilaments (C), with a peculiar dot-like paranuclear pattern in both the stains. The cells appeared also positive for synaptophysin staining (D) with cytoplasmic reactivity

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Authors and Affiliations

  1. Neurology Unit, IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132, Milan, Italy

    Riccardo Milani, Miryam Cannizzaro, Federica Cerri & Massimo Filippi

  2. Department of Pathology, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Gianluigi Arrigoni & Federica Filipello

  3. Neurorehabilitation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Massimo Filippi

  4. Neurophysiology Service, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Massimo Filippi

  5. Neuroimaging Research Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Massimo Filippi

  6. Vita-Salute San Raffaele University, Milan, Italy

    Massimo Filippi

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  1. Riccardo Milani
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  2. Miryam Cannizzaro
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  3. Gianluigi Arrigoni
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  4. Federica Filipello
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  5. Federica Cerri
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Contributions

RM contributed to study design, literature review, data analyses and paper drafting. MC contributed to study design, data analyses and paper drafting. GA and FF contributed to data collection and paper drafting. FC contributed to study design and paper drafting. MF contributed to paper conception, supervised data analysis and interpretation, critically reviewed the paper.

Corresponding author

Correspondence to Massimo Filippi.

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No relevant competing interest was declared by the Authors.

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The patient gave explicit informed consent to report his clinical case.

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Milani, R., Cannizzaro, M., Arrigoni, G. et al. Subacute sensory neuronopathy associated with Merkel cell carcinoma with unknown primary: a case report with literature review. J Neurol 269, 4080–4088 (2022). https://doi.org/10.1007/s00415-022-11116-9

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  • DOI: https://doi.org/10.1007/s00415-022-11116-9

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