Opsoclonus myoclonus syndrome due to squamous cell carcinoma of the oesophagus

Abstract

We present the case of a 47-year-old woman with encephalopathy, ataxia and oscillopsia diagnosed with opsoclonus myoclonus syndrome (OMS). In adults, OMS in the context of encephalopathy is commonly paraneoplastic. A CT of the chest, abdomen and pelvis and a gastroscopy were performed and were normal. A fluorodeoxyglucose positron emission tomography CT scan demonstrated increased uptake in the mid-oesophagus and an endoscopic ultrasound examination demonstrated intramural thickening of 5 mm with no epithelial lesion. The patient was treated with three courses of intravenous immunoglobulin (IvIg) with improvement in her symptoms. A year following the initial presentation a repeat endoscopic examination demonstrated an ulcerated oesophageal carcinoma with regional lymph node involvement. The patient was treated with neo adjuvant chemotherapy prior to oesophagectomy. The patient's symptoms resolved within 6 months of tumour resection without the need for further IvIg. Unfortunately, after a further 6 months the patient developed liver metastases and died.

Figure 1

Radial endoscopic ultrasound image taken 7 months after the onset of symptoms and 5 months before the diagnosis of oesophageal cancer. An area of oesophageal thickening is seen at 11 o'clock directly beneath the epithelial layer. There was no macroscopic evidence of malignancy at the time of the endoscopy.

Figure 2

The endoscopic appearance of the oesophageal cancer immediately prior to surgery by which time there was mucosal invasion.