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Patients with A20 haploinsufficiency (HA20) presenting with central nervous system (CNS) symptoms are rare, and available reports are limited. Here, we describe a patient with HA20, previously followed up as Behçet disease, who presented with CNS symptoms in adulthood. A 38-year-old Japanese male who had been followed up for incomplete Behçet disease at another hospital since 28 years of age presented to our hospital with acute-onset diplopia and persistent hiccups that were severe enough to cause vomiting.

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Persistent hiccups are rare but can serve as an early symptom of underlying conditions, including pulmonary infections and cerebrovascular disorders. This case highlights hiccups as a presenting symptom of bronchopneumonia in a hemodialysis patient and explores the effective use of chlorpromazine and Hange-koboku-to (HKT) as symptomatic therapies. Given the potential association of hiccups with neurological conditions, this case underscores the need for comprehensive diagnostic evaluation.

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  • * Using cerebrospinal fluid multiplex polymerase chain reaction (PCR) testing, doctors quickly identified VZV, leading to immediate antiviral treatment.
  • * This situation emphasizes the need to think about VZV in rare neurological cases and showcases how multiplex PCR can improve diagnosis, especially in cases without common symptoms.
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  • A young patient with neuromyelitis optica spectrum disorder experienced recurrent cardiac asystole and syncope following persistent hiccups, but successfully underwent cardioneuroablation (CNA) treatment, avoiding the need for a permanent pacemaker.
  • Previous cases of symptomatic bradycardia-arrhythmia related to area postrema syndrome (APS) showed a significant number required pacemaker implantation, highlighting the severity of the condition.
  • The findings suggest that CNA can effectively treat bradyarrhythmia associated with APS, and healthcare professionals should consider the potential link between these symptoms and APS in their clinical assessments.*
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