Abstract
Introduction: Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. Methods: A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Subsequently he also developed dysarthria, diplopia, xerostomia, fatigability and progressive anorexia. He underwent a full diagnostic workup and was found to have a high titer of voltage-gated calcium channel antibodies in serum and cerebrospinal fluid, neurophysiological findings compatible with Lambert-Eaton myasthenia and neurological signs compatible with cerebellar degeneration. Results: A positron emission tomography study revealed a hypermetabolic lesion in the axilla, subsequently biopsied and consistent with Merkel cell carcinoma. Conclusions: In most previous reports, neurological symptoms preceded the Merkel cell carcinoma diagnosis, and the primary localization was in lymph nodes. This tumor should be considered in patients with paraneoplastic syndrome, and particularly Lambert-Eaton myasthenia after exclusion of small cell lung carcinoma.
| Original language | English |
|---|---|
| Journal | Muscle and Nerve |
| DOIs | |
| Publication status | E-pub ahead of print - 2017 |
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Keywords
- Cerebellar degeneration
- Lambert-Eaton myasthenia
- Merkel cell carcinoma
- Neuroendocrine neoplasm
- Paraneoplastic syndrome
- Voltage-gated calcium channel antibodies
ASJC Scopus subject areas
- Physiology
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Physiology (medical)
Cite this
Paraneoplastic cerebellar degeneration and lambert-eaton myasthenia in a patient with merkel cell carcinoma and voltage-gated calcium channel antibodies. / Pavolucci, Lucia; Giannini, Giulia; Giannoccaro, Maria Pia; Foschini, Maria Pia; Lang, Bethan; Avoni, Patrizia; Tinuper, Paolo; Vincent, Angela; Liguori, Rocco.
In: Muscle and Nerve, 2017.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Paraneoplastic cerebellar degeneration and lambert-eaton myasthenia in a patient with merkel cell carcinoma and voltage-gated calcium channel antibodies
AU - Pavolucci, Lucia
AU - Giannini, Giulia
AU - Giannoccaro, Maria Pia
AU - Foschini, Maria Pia
AU - Lang, Bethan
AU - Avoni, Patrizia
AU - Tinuper, Paolo
AU - Vincent, Angela
AU - Liguori, Rocco
N1 - Ricercatori distaccati presso IRCCS a seguito Convenzione esclusiva con Università di Bologna (Avoni Patrizia, Tinuper Paolo, Liguori Rocco)
PY - 2017
Y1 - 2017
N2 - Introduction: Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. Methods: A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Subsequently he also developed dysarthria, diplopia, xerostomia, fatigability and progressive anorexia. He underwent a full diagnostic workup and was found to have a high titer of voltage-gated calcium channel antibodies in serum and cerebrospinal fluid, neurophysiological findings compatible with Lambert-Eaton myasthenia and neurological signs compatible with cerebellar degeneration. Results: A positron emission tomography study revealed a hypermetabolic lesion in the axilla, subsequently biopsied and consistent with Merkel cell carcinoma. Conclusions: In most previous reports, neurological symptoms preceded the Merkel cell carcinoma diagnosis, and the primary localization was in lymph nodes. This tumor should be considered in patients with paraneoplastic syndrome, and particularly Lambert-Eaton myasthenia after exclusion of small cell lung carcinoma.
AB - Introduction: Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. Methods: A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Subsequently he also developed dysarthria, diplopia, xerostomia, fatigability and progressive anorexia. He underwent a full diagnostic workup and was found to have a high titer of voltage-gated calcium channel antibodies in serum and cerebrospinal fluid, neurophysiological findings compatible with Lambert-Eaton myasthenia and neurological signs compatible with cerebellar degeneration. Results: A positron emission tomography study revealed a hypermetabolic lesion in the axilla, subsequently biopsied and consistent with Merkel cell carcinoma. Conclusions: In most previous reports, neurological symptoms preceded the Merkel cell carcinoma diagnosis, and the primary localization was in lymph nodes. This tumor should be considered in patients with paraneoplastic syndrome, and particularly Lambert-Eaton myasthenia after exclusion of small cell lung carcinoma.
KW - Cerebellar degeneration
KW - Lambert-Eaton myasthenia
KW - Merkel cell carcinoma
KW - Neuroendocrine neoplasm
KW - Paraneoplastic syndrome
KW - Voltage-gated calcium channel antibodies
UR - http://www.scopus.com/inward/record.url?scp=85016580909&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85016580909&partnerID=8YFLogxK
U2 - 10.1002/mus.25530
DO - 10.1002/mus.25530
M3 - Article
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
ER -