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The Unnerving Case of the Hiccups
The Unnerving Case of the Hiccups
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Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease that causes severe inflammation and damage to the central nervous system. Unlike multiple sclerosis, it has distinct features and requires prompt recognition because delayed treatment can lead to major disability.<br /><br />This case describes a 77-year-old man who first came to the emergency department with intractable hiccups, nausea, and vomiting. These symptoms resolved with baclofen and metoclopramide, but they were likely an early sign of area postrema syndrome, a core feature of NMOSD that is often missed. Two weeks later, he returned with rapidly worsening lower-extremity weakness, urinary retention, and saddle anesthesia. His neurologic exam showed marked paraparesis and severe sensory loss below the T2 level.<br /><br />MRI of the spine showed a long, continuous lesion from C7 to the conus medullaris, and brain MRI showed involvement of the area postrema, findings strongly suggestive of NMOSD. Cerebrospinal fluid analysis showed marked neutrophilic pleocytosis and elevated protein, with no evidence of infection. He was treated immediately with high-dose intravenous methylprednisolone and plasma exchange. Despite treatment, his weakness progressed overnight to complete paraplegia. He completed seven sessions of plasmapheresis and five days of steroids, then was placed on a prednisone taper with plans for long-term immunosuppression. Serum aquaporin-4 antibodies later returned strongly positive, confirming NMOSD.<br /><br />The case highlights how area postrema symptoms such as unexplained hiccups, nausea, and vomiting can be an early clue to NMOSD. Early diagnosis and treatment are critical to reduce irreversible neurologic injury.
Asset Subtitle
Colin Tsui
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Author List
Amanda Frugoli, Colin Tsui, Mostafa Hammoudi, Rahul Jalota, Simon Akl, Yalda Chalabi
Category
Clinical Vignettes
Concept
Neuromyelitis Optica Spectrum Disorder
Concept
Area Postrema Syndrome
Concept
Myelitis
Concept
Central Nervous System Demyelination
Concept
Spinal Cord Lesion
Distinguished
Non-Finalist
Presenter Organization
Community Memorial Health System
Presenting Author
Colin Tsui
Track
Adult
Keywords
neuromyelitis optica spectrum disorder
NMOSD
area postrema syndrome
aquaporin-4 antibodies
longitudinally extensive transverse myelitis
paraparesis
plasma exchange
intravenous methylprednisolone
urinary retention
central nervous system inflammation
Neuromyelitis Optica Spectrum Disorder
Area Postrema Syndrome
Myelitis
Central Nervous System Demyelination
Spinal Cord Lesion
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