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Intractable hiccup and nausea in neuromyelitis optica: a clinical predictor of acute spinal exacerbations

Objective: To demonstrate the occurrence of intractable hiccup and nausea (IHN) as first symptoms in Neuromyelitis optica (NMO) patients who developed cervical myelitis. Background: IHN are unique symptoms of NMO, which is a neurological disorder mainly characterized by optic neuritis and myelitis. Methods: We reviewed the medical records of 25 cases of relapsing NMO seen at the Ramos Mejia Hospital in Argentina during the period from 2006 to 2010. NMO was diagnosed by the Wingerchuk’s criteria. Indirect immunofluorescence with a substrate of monkey cerebellum was used to identify the distinctive NMO-IgG staining pattern. We selected all the cases that manifested IHN and analyzed their clinical profiles, MRI findings and the treatment response in one of them. IHN was defined as bouts lasting for more than 48 hours. Results: 4 out of 25 patients with NMO (16%) had one or more bouts of IHN. Three of them were women. The age at onset ranged from 14 to 31 years old. All patients were seropositive for anti-aquaporin 4 (AQP4) antibodies. IHN symptoms preceded transverse myelitis by a median of 4 weeks in three of them. No obvious differences in the clinical parameters were seen between the patients with and those without IHN except for the high prevalence of medulla lesions in the patients with IHN. None developed brainstem, cerebellar or others brain manifestations. MRI showed medullary lesions involved pericanal regions, extended into the fourth ventricle and connected to the cervical cord lesions. We describe one patient presented only with IHN and medullary MRI lesions involving dorsomedial structures including the area postrema that were successfully treated with high-dose IV methylprednisolone and who had no subsequent myelitis. Conclusions: These cases demonstrate that IHN could be a clinical marker for the early phase of an exacerbation of the disease. Brain lesions of NMO were reported to be localized in the periventricular and periaqueductal areas consistent with the distribution of AQP4. Thus, the development of IHN could be associated with anti- AQP4 antibody. The early initiation of the treatment can lead to a quick recovery and a favorable prognosis of this potential devastating disease.


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