IntroductionOpsoclonus-myoclonus syndrome is a rare autoimmune syndrome usually seen in children and very rarely in adults. It typically presents with a triad of opsoclonus, myoclonus and ataxia, and is most often associated with a tumor or after an infection or vaccination. Around half of all adult cases are paraneoplastic in origin, and isolated case reports include associations with lung, breast and ovarian cancers. To the best of our knowledge, this is the first-ever reported case of paraneoplastic opsoclonus-myoclonus syndrome occurring in association with a nasopharyngeal carcinoma.Case presentationA 50-year-old British Caucasian woman presented with left-sided otalgia and subjective hearing loss. Over the coming weeks she developed subacute confusion and dizziness, leading to recurrent falls. Her clinical examination revealed opsoclonus, myoclonus and signs of cerebellar dysfunction. Subsequent magnetic resonance imaging revealed a left-sided nasopharyngeal carcinoma, which was confirmed on biopsy. A tapering dose of steroids and a five-day course of intravenous immunoglobulins, followed by a combination of chemo-radiotherapy for the nasopharyngeal carcinoma, led to a significant clinical improvement. At six months follow-up she had no signs of focal neurological deficit, apart from the inability to tandem walk. We believe that the typical clinical features, presence of a tumor and response to treatment support a paraneoplastic aetiology.ConclusionsWe show that a nasopharyngeal carcinoma can be associated with adult onset opsoclonus-myoclonus syndrome. Both neurologists and otorhinolaryngologists must be aware of such a presentation. Prognosis of the syndrome depends on early and adequate management of the tumor, therefore prompt identification of the syndrome and the underlying tumor is essential.
ObjectiveTo report a patient with late-onset seropositive myasthenia gravis in whom further investigations disclosed a carcinoid tumour, a rarely reported association.ResultsAn 83-year-old woman presented with a six-month history of double vision, drooping of her right eyelid, and dysphagia. Similar symptoms had occurred two years previously but remitted spontaneously after a few weeks. Acetylcholine receptor antibody assay was strongly positive, confirming the clinical diagnosis of myasthenia gravis. Symptomatic treatment with pyridostigmine and then disease-modifying therapy with intravenous immunoglobulin produced clinical benefit. Computed tomography of the thorax showed a lobulated soft tissue mass (2.8 × 4.0 cm) lying in the anterior mediastinum adjacent to the right heart border. Transthoracic biopsy of this lesion showed features of a carcinoid tumour.ConclusionCarcinoid tumours have rarely been reported in association with myasthenia gravis: we have identified only two previously reported cases (one from Liverpool). It remains unclear whether this represents a chance concurrence of two rare disorders or a paraneoplastic phenomenon.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.