Researchers from India reported the rare case of a patient with neuromyelitis optica spectrum disorder (NMOSD) with aquaporin-4 (AQP4) antibody who presented with area postrema syndrome (APS) that later progressed to myelitis. 

“Diagnosis of NMOSD is based on International Consensus Diagnostic Criteria within the exclusion of common differentials such as multiple sclerosis and myelin oligodendrocyte glycoprotein antibody-associated disease,” the researchers wrote. “Less commonly, NMOSD with AQP4-IgG can present with APS and [progress] to myelitis.”

They said these patients should be treated with intravenous prednisolone, plasma exchange, and immunotherapy. The patients should be followed-up long-term to ensure that any relapses are detected in a timely manner, they wrote.

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The case was that of a man, aged 52 years, who presented with multiple episodes of hiccups and vomiting, a hallmark symptom of APS. He also had decreased bladder sensation and urinary retention. In addition, he had developed weakness and reduced sensation in both legs and muscle spasms in his hands and legs in the last 2 weeks.

Read more about the differential diagnosis of NMOSD

Blood tests revealed the presence of anti-AQP4 antibody. Spinal magnetic resonance imaging (MRI) showed T2 hyperintensities and acute transverse myelitis. Brain magnetic resonance spectroscopy showed mild demyelination. 

Based on his symptoms and other investigations, he was diagnosed with NMOSD and treated with intravenous methylprednisolone, which led to a 30% improvement in the weakness in his legs. He was also given 7 cycles of plasma exchange and physiotherapy.

He was discharged from the hospital after 14  days and mycophenolate mofetil was added to his treatment regimen with a monthly pulse of intravenous methylprednisolone. He was also given baclofen, carbamazepine, and diazepam to reduce recurrent painful muscle spasms. His neurological symptoms were significantly improved.

The study is published in the journal Clinical Case Reports.

NMOSD is a rare chronic inflammatory disease affecting the brain and spinal cord. One of the core clinical features of the disease is APS, characterized by intractable nausea, vomiting, and hiccups lasting more than 48 hours.


Jagannath P, Suhail KM, Aslam S SM, et al. Neuromyelitis optica spectrum disorder with AQP4-IgG presenting as area postrema syndrome and progressing to myelitis: a rare case report. Clin Case Rep. Published online July 4, 2023. doi:10.1002/ccr3.7636