Severe Gastroparesis in Neuromyelitis Optica: Response to Corticosteroids

Abstract

Purpose: Introduction: Gastroparesis has been rarely associated with demyelinating lesions involving the medulla. We describe a case of severe gastroparesis associated with neuromyelitis optica (Devic's disease) involving the medulla. The patient was refractory to all antiemetics and prokinetics. Her gastrointestinal and neurological symptoms responded rapidly and dramatically to corticosteroids. Case Report: A 41 year old female with a history of optic neuritis presented with severe nausea, vomiting, and intractable hiccups of 2 months duration. She lost 40 lbs. Physical examination was unremarkable other than signs of recent weight loss. Laboratory evaluation was normal for complete blood count, comprehensive metabolic panel, and thyroid function. Negative evaluation included upper endoscopy, small bowel follow through x-ray, CT scan of the abdomen and gallbladder ultrasound. Hepatobiliary scan showed no gallbladder emptying, consistent with gallbladder dyskinesia. Gastric emptying scan revealed markedly delayed emptying with a half-time emptying of 532 minutes (normal < 90 minutes), consistent with severe gastroparesis. She did not respond to proton pump inhibitors, metoclopramide, erythromycin, domperidone, ondansetron, scopolamine, prochlorperazine, promethazine, or baclofen. She then developed ataxia and diploplia, and was found to have nystagmus, upgoing toes and clonus. An MRI of her brain revealed demyelinating lesions of the medulla. NMO antibody was positive, consistent with neruomyelitis optica (Devic's disease). She was given methylprednisolone 1 g twice daily for 3 days. Her neurological and gastrointestinal symptoms resolved within days. Subsequent relapse of her demyelinating lesions involving the spine, but not the medulla, was not associated with gastrointestinal symptoms. Discussion: Demyelinating lesions involving the medulla can be associated with various gastrointestinal manifestations since the medulla houses the dorsal motor nucleus of the vagus nerve. Our patient had severe gastroparesis, nausea, vomiting, hiccups and gallbladder dyskinesia, due to interference with the vomiting center of the brainstem and vagal motor innervation of the stomach and gallbladder. She was refractory to antiemetics and prokinetics, but responded dramatically to corticosteroids. The fact that relapses of neuromyelitis optica not involving the medulla were not associated with gastrointestinal symptoms, indicates that the symptoms were due to medullary involvement, not the disease itself. Gastroenterologists should be aware of this association and its prompt response to corticosteroids.