Area postrema syndrome: an atypical presentation of primary Sjogren's

Authors

  • Sonam Tanwar Department of Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India
  • Rahul Jadhav Department of Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India
  • Jyotsna Oak Department of Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India
  • Tushar Raut Department of Neurology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20240552

Keywords:

Area postrema syndrome, Longitudinal extensive transverse myelitis, NMOSD, Primary Sjogren’s syndrome, AQP4-Ab

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune condition characterized by antibodies against serum aquaporin-4 (AQP4), primarily affecting the optic nerves and spinal cord. This case report sheds light on the diagnostic challenges of area postrema syndrome (APS) as the initial manifestation of Sjogren's syndrome, often misidentified due to its diverse symptomatology. APS, seen in 10% of NMOSD cases, manifests as persistent nausea, vomiting, and uncontrollable hiccups. Left untreated, APS can progress to optic neuritis and longitudinally extensive myelitis, emphasizing the need for early intervention with corticosteroid therapy and immunomodulators. NMOSD commonly coexists with other autoimmune diseases like Sjogren's syndrome and systemic lupus erythematosus. Recent evidence also highlights NMOSD's impact on peripheral organs, particularly skeletal muscles, with elevated creatine kinase levels during acute phases. Understanding the relationship between NMOSD and autoimmune diseases is vital for accurate diagnosis and management, especially in cases of seronegative NMOSD and recurrent attacks, emphasizing comprehensive clinical evaluations and a broader diagnostic approach.

References

Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-89.

Shosha E, Dubey D, Palace J, Nakashima I, Jacob A, Fujihara K, et al. Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD. Neurology. 2018;91(17):1642-51.

Shahmohammadi S, Doosti R, Shahmohammadi A, Mohammadianinejad SE, Sahraian MA, Azimi AR, et al. Autoimmune diseases associated with neuromyelitis optica spectrum disorders: a literature review. Multiple sclerosis and related disorders. Elsevier B.V.; 2019: 350-63.

Wang X, Shi Z, Zhao Z, Chen H, Lang Y, Kong L, et al. The causal relationship between neuromyelitis optica spectrum disorder and other autoimmune diseases. Front Immunol. 2022;13.

Wingerchuk DM, Weinshenker BG. The emerging relationship between neuromyelitis optica and systemic rheumatologic autoimmune disease. Mult Scler. 2012;18(1):5-10.

Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome. Medicine. 2004;83(5):280-91.

Tzarouchi LC, Tsifetaki N, Konitsiotis S, Zikou A, Astrakas L, Drosos A, et al. CNS involvement in primary sjögren syndrome: assessment of gray and white matter changes with MRI and Voxel-based morphometry. Am J Roentgenol. 2011;197(5):1207-12.

Pittock SJ, Lennon VA, De Seze J, Vermersch P, Homburger HA, Wingerchuk DM, et al. Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol. 2008;65(1):78-83.

Gökçay F, Çelebisoy N, Gökçay A, Kabasakal Y, Öder G. Primary Sjögrens syndrome presenting as neuromyelitis optica. Pediatr Neurol. 2007;36(1):58-60.

Qiao L, Wang Q, Fei Y, Zhang W, Xu Y, Zhang Y, et al. The clinical characteristics of primary Sjogren’s syndrome with neuromyelitis optica spectrum disorder in China: A STROBE-compliant article. Medicine. 2015;94(28).

Javed A, Balabanov R, Arnason BGW, Kelly TJ, Sweiss NJ, Pytel P, et al. Minor salivary gland inflammation in Devic’s disease and longitudinally extensive myelitis. Mult Scler. 2008;14(6):809-14.

Zhang B, Zhong Y, Wang Y, Dai Y, Qiu W, Zhang L, et al. Neuromyelitis optica spectrum disorders without and with autoimmune diseases. BMC Neurol. 2014;14(1).

Chen HX, Zhang Q, Lian ZY, Liu J, Shi ZY, Miao XH, et al. Muscle damage in patients with neuromyelitis optica spectrum disorder. Neurol Neuroimmunol Neuroinflamm. 2017;4(6).

He D, Li Y, Dai Q, Zhang Y, Xu Z, Li Y, et al. Myopathy associated with neuromyelitis optica spectrum disorders. Int J Neurosci. 2016;126(10):863-6.

Guo Y, Lennon VA, Bogdan BF, Grouse CK, Topel J, Milone M, et al. Autoimmune aquaporin-4 myopathy in neuromyelitis optica spectrum. JAMA Neurol. 2014;71(8):1025-9.

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Published

2024-02-28

How to Cite

Tanwar, S., Jadhav, R., Oak, J., & Raut, T. (2024). Area postrema syndrome: an atypical presentation of primary Sjogren’s. International Journal of Research in Medical Sciences, 12(3), 981–984. https://doi.org/10.18203/2320-6012.ijrms20240552

Issue

Section

Case Reports