A case of area postrema syndrome associated with sick sinus syndrome in an elderly patient with neuromyelitis optica spectrum disorder: Case report

Abstract

Hiccups (singultus) are one of the most challenging symptoms to diagnose a cause. Causes range from the gastrointestinal and cardiovascular systems to the neurological system. 1 It is essential to remember that area postrema syndrome (APS), particularly in cases caused by neuromyelitis optica spectrum disorders (NMOSD), is an important differential diagnosis. 2 Persistent and intractable hiccups are a representative symptom in patients with NMOSD and associated with damage to areas abundant with the aquaporin protein. [3][4][5] However, intractable hiccups caused by APS with NMOSD rarely are associated with sick sinus syndrome (SSS). 6,7 | C A S EA 77-year-old man presented with an acute onset of hiccups and reported vomiting 8 days prior to admission. Hiccups occurred about 10 times per minute and persisted over 48 hours. An electrocardiogram (ECG) did not show any abnormal findings. A gastrointestinal endoscope revealed an esophageal hiatal hernia and atrophic gastritis, but there was no clear cause of the hiccups. Intravenous administration of metoclopramide, haloperidol, diazepam, and chlorpromazine did not relieve the hiccups. At this point, he was transferred to our hospital, where he lost consciousness because of sinus arrests lasting 13 seconds. He was diagnosed with SSS (Figure 1A

Neuromyelitis Optica Spectrum Disorder with Sick Sinus Syndrome: Two Cases and a Literature Review

“…Only a few patients with NMOSD who presented with SSS due to medullary lesions have been reported. We have reviewed all previously reported NMOSD cases with SSS in a total of eight articles after searching PubMed and Medline [4][5][6][7][8][9][10][11]. Table 1 summarizes the eight previous reports of NMOSD patients with SSS and our report of two patients (patients no.…”

Sick sinus syndrome as the initial manifestation of neuromyelitis optica spectrum disorder: a case report

“…However, only a few patients with NMOSD who presented with SSS due to medullary lesions have been reported. Table 1 summarizes the six previously reported NMOSD patients presenting SSS associated with medullary lesions, including our patient [ 4 – 8 ]. In our patient, frequent episodes of syncope were among the initial symptoms.…”

“…The patients reported by Tsouris and Okada et al [ 6 , 7 ] were admitted with a chief complaint of syncope, while syncope was noticed early after admission in the patients reported by Endo and Komaki et al [ 4 , 5 ]. Bigi et al [ 8 ] reported a young patient with area postrema involvement who displayed vomiting, hiccups, and sinus bradycardia.…”

“…There are some reports of sick sinus syndrome (SSS) caused by lesions in the medulla oblongata [ 3 ]. Potentially fatal bradyarrhythmia can occur in patients with NMOSD involving the area postrema, but there are few reports of SSS associated with NMOSD [ 4 – 8 ]. Here, we report a patient with NMOSD who developed refractory nausea, vomiting and SSS as the initial manifestation.…”