Two patients with a neuroendocrine tumour of the small intestine and paraneoplastic myasthenia gravis

Summary This paper reports on two patients with a long-standing diagnosis of an ENETS stage IV neuroendocrine tumour (NET) of the small intestine who developed neurological symptoms. The first patient only had bulbar symptoms and tested positive for acetylcholine receptor antibodies. The second pati...

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Bibliographic Details
Published in:Endocrinology, diabetes & metabolism case reports diabetes & metabolism case reports, 2014-05, Vol.2014, p.140013-140013
Main Authors: Hermans, M A W, Stelten, B M L, Haak, H R, de Herder, W W, Dercksen, M W
Format: Article
Language:English
Subjects:
5HIAA
Adult
Anti-acetylcholine antibodies
Cholinesterase inhibitors
Chromogranin A
Diarrhoea
Diplopia
Duodenum
Dysarthria
Dysphagia
Electromyography
Flushing
Gastroenterology
Glucocorticoids
Male
Myasthaenia
Netherlands
Neuroendocrine tumour
Neuroendocrinology
Neurology
Octreotide
Oncology
Paraneoplastic syndromes
Prednisolone
Pyridostigmine
Somatostatin analogues
Unique/Unexpected Symptoms or Presentations of a Disease
White
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Summary:Summary This paper reports on two patients with a long-standing diagnosis of an ENETS stage IV neuroendocrine tumour (NET) of the small intestine who developed neurological symptoms. The first patient only had bulbar symptoms and tested positive for acetylcholine receptor antibodies. The second patient had more classical symptoms of fatigable diplopia and muscle weakness of the legs, but no detectable antibodies. The diagnosis of paraneoplastical myasthenia gravis (MG) was postulated. Both patients were treated with pyridostigmine for MG and octreotide for the NETs. Interestingly, treatment of the NETs resulted in improvement of myasthenic symptoms. Paraneoplastic MG has been described to occur with certain malignancies, mainly thymoma. Herein, we prove that the association with gastrointestinal NETs, however, rare, is also one to be considered by clinicians dealing with either of these diseases. The pathogenesis has yet to be elucidated. Learning points NETs are rare malignancies with a wide variety of symptoms. Paraneoplastic MG can occur with various types of malignancies. Herein, we provide evidence of paraneoplastic MG in association with a grade IV NET of the small intestine. Treatment of the NETs resulted in remission of myasthenic symptoms in one patient.
ISSN:2052-0573
2052-0573
DOI:10.1530/EDM-14-0013