BRAINSTEM SYMPTOMS AS THE FIRST MANIFESTATION OF NEUROMYELITIS OPTICA SPECTRUM DISORDERS.

Background: Brainstem symptoms can be the first manifestation of neuromyelitis optica (NMO) and related spectrum disorders (NMOSD). They can exhibit as a cervical spinal cord lesion extending to the medullary region, or as a brainstem-restricted lesion. These lesions commonly cause intractable nausea, vomiting, and/or hiccups, but oculomotor symptoms or ataxia are infrequent. Objectives: To report the prevalence of brainstem symptoms and lesions on magnetic resonance imaging (MRI) as the first attack of NMOSD. Methods: A retrospective study involving 83 NMO/NMOSD patients followed at the Hospital das Clínicas, University of Sao Paulo (Sao Paulo, Brazil). A cell-based assay was used to detect aquaporin-4 (AQP4)-antibodies. Results: Of the 83 patients included, 67.5% (56/83) had NMO and 32.5% (27/83) had NMOSD. Brainstem symptoms were the first clinical symptom of NMOSD in 31.3% (26/83) of patients. There were brainstem symptoms as the first attack without an extension of a cervical spinal cord lesion in 16.8% (14/83) of individuals, and as an extension of LETM in 14.5% (12/83) of patients. At least one attack with brainstem symptoms during the disease course was present in 51.8% (43/83) of the total number of patients. Conclusions: Brainstem symptoms as the sole manifestation of a first NMOSD attack may not be uncommon. Patients with intractable nausea, vomiting, and/or hiccups, especially those with brainstem lesions shown on an MRI, should be tested for AQP4-antibodies. [ABSTRACT FROM AUTHOR]