Abstract General Information
Recurrent Area Postrema Syndrome in an AQP4-IgG Positive Patient
Case presentation: Female patient, mulatto, 34 years-old, reported the occurrence of hiccups and uncontrollable vomiting, lasting 20 days, followed by complete recovery. After 3 years in remission, she had a 2nd attack with symptoms of paresis and paresthesia in lower limbs; followed by the 3rd attack, after 7 months, with uncontrollable hiccups and paresthesia in lower limbs. After 3 months, she had a new exacerbation, with left hemiparesis and right optic neuritis. In this event, treatment with intravenous methylprednisolone and plasmapheresis was performed, with partial recovery of symptoms. MRI of the brain showed lesions in the brainstem, cervical bulb transition, and extensive damage to the optic nerve; and spinal MRI showed the presence of longitudinally extensive transverse myelitis, with gadolinium uptake. AQP4 antibody was positive by the CBA method. Since then, she has had 7 new outbreaks, with APS recurrence on 2 of these occasions, accompanied by Lhermitte's sign on one occasion and isolated on the other. The last recorded EDSS, 18 years after the first outbreak, was 6.5.
Discussion: We describe the case of a patient with NMOSD who evolved with 4 APS attacks and a significant EDSS in the course of the disease. Area postrema syndrome (APS) is one of the central clinical manifestations of neuromyelitis optica spectrum disorder (NMOSD), but the prevalence of recurrent APS in NMOSD is not well elucidated in literature, with only 1 case report presenting 2 episodes of the syndrome available at PubMed platform. Some studies indicate that the presence of this condition predicts a worse prognosis in patients with NMOSD.
Final comments: The recurrence of APS in patients with NMOSD is a condition rarely reported in the literature. The analysis of more cases like the one described above may provide information about its value as a prognostic factor for the disease.
Larissa Ferrarez, Clara Catharino Pinhati, Ana Cristina Cotta Queiroz, Beatriz Silva Lombardi, Cleison Sanches Silva, Denison Pedrosa, Emerson Costa Oliveira, Felipe Baptista Brunheroto, Gabriel Coelho, Grazielle Fialho de Souza, Hugo Brito, Juliana Santiago Amaral, Juliana Monção, Márcia Prates, Natália Cirino Talim, Paulo Pereira Christo, Pedro L. Bessa dos Reis, Thales Ponsá, Marco Aurélio Lana-Peixoto