Intestinal pseudo-obstruction, myasthenia gravis, and thymoma

Anderson, Neil E.; Hutchinson, David; Nicholson, G; Aitcheson, F.; Nixon, Joanna

doi:10.1212/wnl.47.4.985

Cited by 38 publications

(21 citation statements)

References 9 publications

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“…These myopathies or neuropathies can be either degenerative or inflammatory. Over recent years, observations have suggested that some cases of gastro-intestinal dysmotility are immune-mediated, based in part on the associations of CIPO with small-cell lung cancer and thymoma [7, 8, 14, 15], two tumours which frequently stimulate the immune system and result in paraneoplastic neurological disorders. In these cases, the antibodies detected in routine practice are often directed against intracellular antigens, such as Hu (ANNA-1), and are probably not pathogenic since there are few examples of a response to treatments that lower the antibody levels [16, 17].…”

Section: Discussionmentioning

confidence: 99%

“…Aetiologies include disease processes localised to the colon, such as Hirschsprung’s disease, or generalised motility disorders that affect multiple regions of the gastrointestinal tract [5]. CIPO can be a paraneoplastic disorder which appears to be secondary to an autoimmune process triggered by a cross-reaction between antigens shared by the tumour cells and myenteric plexus neurons, and several cases have been reported in association with thymoma or small-cell lung cancer [6,7,8]. CIPO has also been described during the course of idiopathic autonomic neuropathy characterised by the high frequency of circulating ganglionic AChR autoantibodies [9].…”

Section: Introductionmentioning

confidence: 99%

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Thymoma-Associated Neuromyotonia with Antibodies against Voltage-Gated Potassium Channels Presenting as Chronic Intestinal Pseudo-Obstruction

Viallard¹,

Vincent

Moreau

et al. 2005

Eur Neurol

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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 acquiredneuromyotonia (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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Thymoma-Associated Neuromyotonia with Antibodies against Voltage-Gated Potassium Channels Presenting as Chronic Intestinal Pseudo-Obstruction

Viallard¹,

Vincent

Moreau

et al. 2005

Eur Neurol

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“…In chronic EAAN, the density of neurons in paravertebral sympathetic ganglia appeared to be normal, but there was a reduction in the number of neurons in enteric plexus suggesting that some autonomic neurons not only lose synaptic nAChR but also sustain immunocytotoxic or apoptotic cell death. Inflammation and loss of ganglionic neurons have been documented in biopsied bowel of patients with paraneoplastic autoimmune autonomic neuropathy (Anderson et al 1996;Condom et al 1993;Lennon et al 1991). Enteric neurons may be particularly sensitive to an immunological attack directed against neuronal ganglionic nAChR because fast cholinergic transmission is important in both the extrinsic and intrinsic neural regulation of gut motility, and in both the motor and mechanosensory limbs of the enteric nervous system.…”

Section: Discussionmentioning

confidence: 99%

Experimental Autoimmune Autonomic Neuropathy

Vernino

Low

Lennon

2003

Journal of Neurophysiology

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. Antibodies specific for the neuronal ganglionic nicotinic acetylcholine receptor (nAChR) are found in high titer in serum of patients with subacute autonomic failure. This clinical disorder is known as autoimmune autonomic neuropathy (AAN). Rabbits immunized with a neuronal nAChR ␣3 subunit fusion protein produce ganglionic nAChR antibodies and develop autonomic failure (experimental AAN, or EAAN). We used quantitative measures of autonomic function to demonstrate that this animal model of neuronal nAChR autoimmunity recapitulates the cardinal autonomic features of AAN in humans. The severity of dysautonomia in the rabbit ranges from isolated cardiovagal impairment to severe panautonomic failure with fixed mydriasis, gastroparesis, dry eyes, impaired heart rate variability, hypotension, and low plasma catecholamines. The severity of autonomic failure correlates with serum antibody levels. Immunohistochemical staining of superior cervical ganglia and myenteric plexus neurons demonstrates intact presynaptic nerve terminals and intact postsynaptic neurons containing cytoplasmic nAChR, but lacking surface nAChR. These findings define the autonomic physiology and histopathology of this novel animal model and support the concept that AAN in humans is a disorder of ganglionic cholinergic synaptic transmission caused by ganglionic nAChR antibodies.

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“…Pseudo-achalasia, gastroparesis and intestinal pseudo-obstruction have all been linked to the autoantibody anti-Hu. 5,7,13,16,17 The autoantibodies found in patients with paraneoplastic GI dysmotility are different from those thought to be responsible for primary achalasia in that the former may affect neurons throughout the GI track. Therefore, many patients have a pan-dysmotility, although usually one of the above disorders will present as the predominant symptom.…”

Section: Clinical Characteristics/differential Diagnosis/serological mentioning

confidence: 99%

GI Dysmotility: A Case Report

Osley¹,

Leung²

2010

TMF

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A 75-year-old male, with a medical history of diabetes, hypertension, coronary artery disease, status post coronary artery bypass graft, and left-sided breast cancer, status post left breast mastectomy, was transferred from an outside hospital with complaints of a month of constipation, nausea and vomiting. The patient presented to an OSH a month prior with recent onset of constipation, with no bowel movements for 10 days, changed from his usual habit of daily bowel movements. He initially responded to lactulose with a bowel movement and was discharged on a regimen of stool softeners and laxatives; however, upon returning home, he was again unable to move his bowels despite his bowel regimen and developed diffuse abdominal pain, nausea and vomiting secondary to distension. An outpatient esophagogastroduodenoscopy (EGD) showed that the patient had a normal esophagus and a large amount of retained food in the stomach, concerning for gastroparesis. This finding was thought to be secondary to his diabetes, despite his well-controlled blood sugars and a hemoglobin A1c of 7.0 %. After two further weeks of constipation, the patient was readmitted to the OSH with abdominal pain, intractable nausea and vomiting, as well as a 20-25 lbs weight loss since his symptoms began. Imaging showed colonic gaseous distention, with the cecum dilated to 9.5 cm, and an un-prepped sigmoidoscopy was performed, showing no inflammation, polyps or masses; of note, a screening colonoscopy done 4 months prior identified and removed multiple benign polyps. A CT scan of his abdomen and pelvis also did not show any obstructing colonic masses. He was presumed to have colonic pseudo-obstruction, or Ogilvie Syndrome. Erythromycin was started without effect. He was subsequently given neostigmine, which was also unsuccessful in relieving his symptoms. The patient was then referred to another OSH for further workup. A 4-day gut motility study showed a pan-GI dysmotility disorder. Furthermore, a gastric emptying study revealed markedly delayed gastric emptying of both solid and liquid foods, but defecography was entirely normal. Non-contrast CTs of the chest, abdomen and pelvis again did not identify any masses or bowel obstruction, but did show enlarged precarinal lymph nodes and prostatomegaly, as well as small thyroid nodules bilaterally. As the patient continued to fail to have a bowel movement and could not tolerate a diet, he was started on total parenteral nutrition and was transferred to Thomas Jefferson University Hospital (TJUH) for further evaluation.On presentation to TJUH, the patient complained of occasional diffuse abdominal pain and distention, as well as nausea. He had not had any vomiting since he stopped eating when he was started on TPN. He denied any swallowing difficulty but did complain of constantly spitting up clear sputum. He had not had a bowel movement in more than 4 weeks since his initial response to lactulose at the first OSH. He had lost 30 lbs in the 4 weeks since his symptoms began. His review of systems was other wise...

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Intestinal pseudo-obstruction, myasthenia gravis, and thymoma

Cited by 38 publications

References 9 publications

Thymoma-Associated Neuromyotonia with Antibodies against Voltage-Gated Potassium Channels Presenting as Chronic Intestinal Pseudo-Obstruction

Thymoma-Associated Neuromyotonia with Antibodies against Voltage-Gated Potassium Channels Presenting as Chronic Intestinal Pseudo-Obstruction

Experimental Autoimmune Autonomic Neuropathy

GI Dysmotility: A Case Report

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