NMOSD testing
Credit: Getty Images

A team of researchers from China reported the case of 4 patients with neuromyelitis optica spectrum disorder (NMOSD) whose first symptom was area postrema syndrome (APS). All 4 patients were initially misdiagnosed and mistreated. 

“The clinical manifestations of NMOSD are complex and diverse,” the authors wrote. “Early identification of the APS and timely therapy can prevent visual and physical disabilities,” they said. It is of great importance “to identify specific and sensitive serum and imaging markers for predicting the prognosis and recurrence of the disease,” they added.

Read more about the prognosis of NMSOD


Continue Reading

The study is published in the European Journal of Medical Research.

All 4 patients described in the current study presented with intractable nausea and vomiting. Three of them were admitted to the gastroenterology department. 

The disease of 2 of the patients could not be diagnosed properly, which led to their condition worsening. They were then admitted to the intensive care unit of the hospital to be put on life support. 

One patient was treated with intravenous immunoglobulin, methylprednisolone, and plasma exchange. However, this did not improve their condition. They were then treated with low-dose rituximab. However, their disease relapsed.

All 4 patients were eventually diagnosed with NMSOD and treated properly before being discharged from the hospital.

“The treatment course can be appropriately extended for patients with older onset and APS to reduce disease recurrence,” the researchers concluded. 

NMSOD is a rare chronic inflammatory disease of the central nervous system characterized by repeated episodes of severe optic neuritis and/or myelitis. In very severe cases, the disease can lead to respiratory failure, coma, and cardiac arrest.

APS occurs as the result of inflammation or lesions on the brain stem and is often associated with NMSOD. Symptoms of APS include nausea, uncontrollable vomiting, and/or hiccups typically lasting more than 48 hours.

Reference

Liu T, Li L, Guo X, Li Q, Jia D, Ma L. Clinical analysis of neuromyelitis optica spectrum disease with area postrema syndrome as the initial symptom. Eur J Med Res. Published online December 29, 2022. doi:10.1186/s40001-022-00949-9