Area Postrema Syndrome-Onset with neuromyelitis optic spectrum disorder (NMOSD) and coincidence Quadrigeminal plate lipoma (PP-29)

Document Type : Poster Presentation

Author
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Abstract
A 40- year- old male patient without any significant past or family history admitted due to intractable and intermittent nausea, vomiting and hiccups attacks. The patient's symptoms started 3-4 months ago and underwent frequent endoscopy to check for nausea and vomiting but unremarkable.  On examination, congenital ptosis, increased tendon reflexes, decreased abdominal skin reflexes, bilateral Babinski sign, and ataxia when walking.  Mental status examination revealed clear consciousness.
Brain MRI was performed. There is fat signal intensity lesion in size 10 * 5.4 mm in right aspect of quadrigeminal cistern cistern, suggestive of lipoma. (Fig. 1-2). Brain MRI with and without contrast revealed foci of high T2/FLAIR signal intensity lesions in periventricular, subcortical and juxtacortical rejoins along with anterior aspect of medulla and left brachium. (Figs. 3-8) After contrast injection lesion in anterior aspect of medulla shows diffuse enhancement. (Figs.9) In upper cervical MRI increase signal intensity in anterior aspect of medulla with extension to superior cervical cord down to C2 level but in lower cervical it is clear (Figs10-12). Serum NMO and MOG Antibody testing were negative. Pulse Methyl Prednisolone 1gr /day started for 5 days and then continue Rituximab therapy. The clinical features of patients improved after 2-3 weeks. 
Discussion
Area postrema syndrome (APS) is one of the core clinical features of neuromyelitis optic spectrum disorder (NMOSD). APS-onset NMOSD with quadrigeminal lipoma mass effect has not been previously reported in literature. APS is mostly associate with NMO and rarely in myelin oligo dendrocyte glycoprotein antibody disease. We herein report a case of APS-onset NMOSD-onset with incidental large quadrigeminal cistern lipoma.
Conclusion
An early diagnosis and treatment result in the resolution of APS-related symptoms and prevent further progression of the disease.

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  • Receive Date 24 December 2024
  • Publish Date 01 October 2024