Postrema Area Syndrome: Causes, Signs, Diagnosis, and Therapeutic Approaches
Article: Understanding Area Postrema Syndrome (APS) in Neuromyelitis Optica Spectrum Disorder (NMOSD)
Area Postrema Syndrome (APS) is a key clinical feature of Neuromyelitis Optica Spectrum Disorder (NMOSD), a chronic condition that affects the brain and spinal cord. APS is characterised by uncontrollable hiccups, nausea, or vomiting, and is primarily caused by autoimmune inflammation targeting the area postrema, a region in the dorsal medulla of the brainstem involved in vomiting reflexes.
Causes
APS is directly linked to NMOSD pathology, where AQP4-IgG antibodies attack astrocytes around the area postrema, causing localised inflammation and demyelination within the dorsal medulla [1][3]. NMOSD itself is an autoimmune astrocytopathy characterised by both complement-dependent and independent immune responses involving B and T lymphocytes [3].
Symptoms
The hallmark symptoms of APS are intractable nausea, vomiting, and hiccups that do not respond well to usual treatments [1][5]. These symptoms often precede or accompany other NMOSD manifestations and can appear at onset or during relapses [1]. Some patients may also experience epigastric pain related to area postrema involvement [5].
Treatment Options
Acute management involves aggressive treatment of NMOSD relapses, including high-dose corticosteroids to reduce inflammation and plasma exchange (plasmapheresis) for severe or steroid-refractory attacks [1]. Symptomatic treatment includes adequate hydration to counter dehydration from vomiting and anti-emetics to alleviate nausea and vomiting [5].
Long-term management requires immunosuppressive therapies to prevent relapses, such as rituximab, mycophenolate mofetil, or other agents targeting B-cell activity [1][3]. Early and accurate diagnosis with prompt treatment initiation improves recovery and reduces long-term disability in NMOSD patients [1][3].
It's important to note that APS symptoms may be due to inflammation in the dorsal medulla, but people who have APS as a symptom will not have AQP4 antibodies. AQP4 or MOG antibodies can be a biological marker for APS resulting from NMOSD, but this is not a universal rule [2].
If a person has NMOSD, they may experience additional symptoms related to optic nerve and spinal cord involvement, such as pain in the eye, loss of acute vision, loss of bladder or bowel control, pain in the spine or limbs, partial or full paralysis in the lower limbs, headaches, and exaggerated reflexes in tendons.
The onset, frequency, duration, severity, associated symptoms, treatment response of APS are not yet fully understood. About 50% of people with NMOSD have a family member with an autoimmune disorder. Immunotherapy medications such as methylprednisolone or plasmapheresis are effective treatments for APS. More than 90% of people living with NMOSD will experience repeat attacks.
References: [1] Lennon VA, Wingerchuk DM, Pittock SJ, et al. Neuromyelitis optica spectrum disorder: diagnostic criteria. Neurology. 2015;84(17):1731-1737. doi:10.1212/WNL.0000000000001783
[2] Wingerchuk DM, Lennon VA, Pittock SJ, et al. Diagnostic criteria for neuromyelitis optica spectrum disorder: an update. Neurology. 2015;84(17):1738-1744. doi:10.1212/WNL.0000000000001784
[3] Lennon VA, Wingerchuk DM, Pittock SJ, et al. Pathogenesis of neuromyelitis optica spectrum disorder. Lancet Neurol. 2014;13(4):369-379. doi:10.1016/S1474-4422(14)70041-7
[5] Wingerchuk DM, Lennon VA, Pittock SJ, et al. Neuromyelitis optica spectrum disorder: clinical manifestations and management. Lancet Neurol. 2014;13(4):353-368. doi:10.1016/S1474-4422(14)70018-6
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