The term “area postrema syndrome” refers to an episode of otherwise unexplained nausea and vomiting or hippus. It is considered a core clinical characteristic of neuromyelitis optica, but may be present in other neurologic conditions such us multiple sclerosis or posterior fossa tumors.
We present the case of a patient suffering a first demyelinating event presenting as “area postrema syndrome”.
A 10 year-old girl with no significant past medical history developed acutely incoercible nausea and vomiting. One week later she complained about double vision and instability. She had no previous history of other neurological symptoms, preceding illness or fever. Her examination was consistent with multidirectional nystagmus, marked dysmetria of the upper and lower extremities and incapacitating ataxia.
She was admitted to the hospital for further evaluation and management. Blood test results were within normal range. Extensive infectious and autoimmunity work up including aquaporin-4 and anti-MOG antibodies were negative. A brain MRI revealed multiple T2-hyperintensities in supratentorial subcortical areas, posterior fossa and brainstem; with gadolinium enhancement along the periaqueductal region. CSF analysis showed normal cell count, protein and glucose levels, with negative cultures and cytology. IgG oligoclonal bands (OCB) with elevated IgG-index were found.
The patient was treated with methylprednisolone and experienced rapid improvement. She was diagnosed with pediatric multiple sclerosis (MS) and started on interferon ß1a.
We conclude that patients suffering isolated and unexplained incoercible nausea and/or vomiting should be considered to have a neurologic substrate, and demyelinating disorders must be excluded. Based on this case report we show that clinical suspicion for an early diagnosis is mandatory to start a modifying therapy as soon as possible in MS since new relapses are expected [1-4].
- Waldman A, Ghezzi A, Bar-Or A (2014) Multiple sclerosis in children: an update on clinical diagnosis, therapeutic strategies, and research. Lancet Neurol 13: 936-948. [Crossref]
- O’Mahony J, Shroff M, Banwell B (2013) Mimics and Rare Presentations of Pediatric Demyelination. Neuroimag Clin N Am 23: 321-336. [Crossref]
- Narula S, Hopkins S, Banwell B (2015) Treatment of Pediatric Multiple Sclerosis. Curr Treat Options Neurol 17: 1-10.
- Wingerchuk M, Banwell B, Bennett JL, Cabre P, Carroll W, et al. (2015) International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 85:177-189. [Crossref]
University of Rome "G.Marconi"
Received date: August 12, 2016
Accepted date: September 16, 2016
Published date: September 19, 2016
© 2016 Sara Vila Bedmar. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Sara Vila Bedmar (2016) Pediatric multiple sclerosis presenting as area postrema syndrome. Clin Case Rep Rev 2: DOI: 10.15761/CCRR.1000274.
Sara Vila Bedmar
Department of Neurology (S.V.-B.), Hospital Universitario 12 de Octubre, Madrid, Spain and Department of Neurology (B.N., S.A., E.W.), University of California San Francisco, USA.
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