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


CONCLUSIONS
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.
Department of Internal Medicine Maxima Medical Centre Ds Theodor Fliednerstr 1; 5631BM; Eindhoven The Netherlands.
M A W
MA
Hermans
Department of Neurology Canisius Wilhelmina Hospital Weg door Jonkerbos 100, 6532SZ, Nijmegen The Netherlands.
B M L
BM
Stelten
Department of Internal Medicine Maxima Medical Centre Ds Theodor Fliednerstr 1; 5631BM; Eindhoven The Netherlands.
H R
HR
Haak
Department of Internal Medicine Erasmus University Medical Centre Gravendijkwal 230, 3015CE, Rotterdam The Netherlands.
W W
WW
de Herder
Department of Internal Medicine Maxima Medical Centre Ds Theodor Fliednerstr 1; 5631BM; Eindhoven The Netherlands.
M W
MW
Dercksen