Abstract Details

Title Isolated Area Postrema Syndrome as a Rare Presentation of Neurosarcoidosis: A Case Report

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 095

Objective NA

Background Area postrema syndrome (APS) is characterized by intractable nausea, vomiting, and/or hiccups. While APS is a core clinical criterion for neuromyelitis optica spectrum disorder, it has rarely been reported in other inflammatory / infiltrative neurological conditions.

Design/Methods NA

Results A 42-year-old female with a history of migraine presented with intractable vomiting. She underwent extensive gastroenterological work up which was unrevealing. Five months later, she developed vertigo, new headache characteristics, and left leg sensory changes. Neurologic examination revealed symmetrically decreased reflexes and diminished sensation to light touch of the left leg. Full neuroaxis MRI, performed eight months after onset, demonstrated a T2 FLAIR hyperintense / T1 hypointense brainstem lesion affecting the area postrema, multifocal leptomeningeal enhancement throughout the brain and entire spine, and intramedullary enhancement in the right dorsolateral cord at C7-T1. Opening pressure was 35 cmH₂O and cerebrospinal fluid studies were notable for elevated protein of 123 and 62 nucleated cells with 96% lymphocytes. Serum demyelinating panel was negative. Infectious work up, including testing for tuberculosis and histoplasmosis, was negative. PET CT demonstrated metabolically active cervical, mediastinal, and hilar lymph nodes. Lymph node aspirate demonstrated non-caseating granulomas and she was diagnosed with probable neurosarcoidosis. She received 5 days of high-dose methylprednisolone which led to resolution of her vomiting. She was subsequently started on a prednisone taper, methotrexate, and infliximab with ongoing clinical stability. Repeat neuroimaging 3 months later showed resolution of the T2 FLAIR brainstem lesion and significant interval improvement in leptomeningeal disease.

Conclusions We present the first case report of APS as a presenting feature of neurosarcoidosis. This patient’s diagnosis and subsequent treatment were considerably delayed. We recommend early neurological imaging and extensive inflammatory, infectious, and malignant evaluation for APS in cases of intractable nausea, vomiting, and/or hiccups where no systemic cause is identified.

Authors/Disclosures
Taylor M. Lakusta-Wong, MD PRESENTER	Dr. Lakusta-Wong has nothing to disclose.
Chantal Roy-Hewitson, MD (University of Vermont Medical Center)	Dr. Roy-Hewitson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Pharma. Dr. Roy-Hewitson has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Pharma.

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