Thymoma-associated neuromyotonia with antibodies against voltage-gated potassium channels presenting as chronic intestinal pseudo-obstruction.
Chronic intestinal pseudo-obstruction can occur as a paraneoplastic disorder, and several cases have been reported in association with thymoma or small-cell lung cancer. Autoantibodies against voltage-gated potassium channels (VGKCs) are found in acquired neuromyotonia (Isaac's syndrome), and have been reported in one case of slow transit constipation without apparent neurological disease. We describe a patient with VGKC antibodies, acquired neuromyotonia and thymoma, who first presented with a severe slow-transit constipation and in whom the gastrointestinal symptoms responded well to plasmapheresis. We suggest that VGKC antibodies might be helpful in patients with possible paraneoplastic chronic intestinal pseudo-obstruction, and a positive result should stimulate the search for a thymoma or other tumour and raise the possibility of immunotherapy.
Viallard
Jean-François
JF
Service de Médecine Interne, Hôpital Haut-Lévêque, Pessac, France. jean-francois.viallard@chu-bordeaux.fr
Vincent
Angela
A
Moreau
Jean-François
JF
Parrens
Marie
M
Pellegrin
Jean-Luc
JL
Ellie
Emmanuel
E
D001323:Autoantibodies; D002908:Chronic Disease; D003248:Constipation / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000473:pathology*; D020386:Isaacs Syndrome / Q000150:complications* / Q000503:physiopathology; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000473:pathology; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D013945:Thymoma / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D013953:Thymus Neoplasms / Q000150:complications* / Q000473:pathology / Q000503:physiopathology
D001323:Autoantibodies; D024642:Potassium Channels, Voltage-Gated