Area Postrema Syndrome as the Onset of Neuromyelitis Optica in a Pediatric Patient: Case Report

Abstract

Area postrema syndrome, characterized by nausea, emesis, and persistent hiccups can be the initial manifestation of demyelinating diseases of the central nervous system.

We report a 4-year-old girl who presented with intractable vomiting that was noted to have signs of demyelination in the area postrema. MRI of the spine revealed longitudinally extensive myelitis in the cervical and thoracic spine. Neuromyelitis optica spectrum disorder (NMOSD) was suspected. Serum anti-aquaporin 4 antibodies were positive. Oligoclonal bands in cerebrospinal fluid were negative.

NMOSDs are extremely rare in the pediatric population, this being the second case reported in Colombia. It is crucial to consider the possibility of such neurological disorders in the setting of intractable vomiting.

Keywords

neuromyelitis optica spectrum disorder - area postrema - myelitis - spinal cord inflammation

Publication History

Received: 20 August 2023

Accepted: 09 June 2024

Article published online:
07 August 2024

• References

• 1 Camara-Lemarroy CR, Burton JM. Area postrema syndrome: a short history of a pearl in demyelinating diseases. Mult Scler 2019; 25 (03) 325-329
• 2 Zeiner PS, Brandhofe A, Müller-Eschner M, Steinmetz H, Pfeilschifter W. Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis. Ann Clin Transl Neurol 2018; 5 (12) 1534-1542
• 3 Wingerchuk DM, Lucchinetti CF. Neuromyelitis optica spectrum disorder. N Engl J Med 2022; 387 (07) 631-639
• 4 Pabón-Moreno A, Gaitán-Quintero GA, Beltrán-Carrascal EJ. et al. Síndrome del área postrema. Med Int Mex. 2021; 37 (04) 613-618
• 5 Zhou C, Liao L, Sun R. et al. Area postrema syndrome as initial manifestation in neuromyelitis optica spectrum disorder patients: a retrospective study. Rev Neurol (Paris) 2021; 177 (04) 400-406
• 6 Chitnis T, Ness J, Krupp L. et al. Clinical features of neuromyelitis optica in children: US Network of Pediatric MS Centers report. Neurology 2016; 86 (03) 245-252
• 7 Cabrera Ávial RM, González Delgado MJ, Perdomo García MA, Hanrryr Murillo MA, Vargas MA. Neuromielitis óptica, en paciente pediátrico. Pediatr. 2021; 54 (04) 167-170
• 8 Paul S, Mondal GP, Bhattacharyya R, Ghosh KC, Bhat IA. Neuromyelitis optica spectrum disorders. J Neurol Sci 2021; 420: 117225
• 9 Wingerchuk DM, Banwell B, Bennett JL. et al; International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015; 85 (02) 177-189
• 10 Dubey D, Pittock SJ, Krecke KN, Flanagan EP. Association of extension of cervical cord lesion and area postrema syndrome with neuromyelitis optica spectrum disorder. JAMA Neurol 2017; 74 (03) 359-361
• 11 Tenembaum S, Chitnis T, Nakashima I. et al. Neuromyelitis optica spectrum disorders in children and adolescents. Neurology 2016; 87 (9 Suppl 2) S59-S66
• 12 Tomari Y, Igata Y, Chong PF. et al. The etiology and outcome of area postrema syndrome in childhood: two cases and a literature review. Pediatr Neurol 2024; 152: 11-15
• 13 Tenembaum S, Yeh EA. Guthy-Jackson Foundation International Clinical Consortium (GJCF-ICC). Pediatric NMOSD: a review and position statement on approach to work-up and diagnosis. Front Pediatr 2020; 8: 339
• 14 Bulut E, Karakaya J, Salama S, Levy M, Huisman TAGM, Izbudak I. Brain MRI findings in pediatric-onset neuromyelitis optica spectrum disorder: challenges in differentiation from acute disseminated encephalomyelitis. AJNR Am J Neuroradiol 2019; 40 (04) 726-731
• 15 Kunchok A, Chen JJ, McKeon A, Mills JR, Flanagan EP, Pittock SJ. Coexistence of myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies in adult and pediatric patients. JAMA Neurol 2020; 77 (02) 257-259
• 16 Ferilli MAN, Paparella R, Morandini I. et al. Pediatric neuromyelitis optica spectrum disorder: case series and literature review. Life (Basel) 2021; 12 (01) 19