Brainstem encephalitis and anti-GQ1b antibody syndrome triggered by varicella-zoster virus presenting as intractable hiccups: a first reported case.

BACKGROUND: Varicella-zoster virus (VZV) reactivation without rash (zoster sine herpete, ZSH) is a known cause of neurological disease. However, its association with an overlap syndrome of brainstem encephalitis and anti-GQ1b antibody syndrome is exceedingly rare, and its presentation with intractable hiccups as the initial symptom has not been previously reported. CASE PRESENTATION: We describe a 35-year-old male whose illness began with isolated intractable hiccups, which progressed to include brainstem signs such as cranial neuropathies and autonomic failure. Diagnosis was confirmed by a triad of findings: CSF next-generation sequencing positive for VZV, serum anti-GQ1b/GM4 antibodies, and a corresponding medullary lesion on MRI. Despite aggressive treatment with intravenous acyclovir and immunoglobulin, his condition deteriorated rapidly, culminating in fatal respiratory and circulatory failure. CONCLUSIONS: This case suggests that intractable hiccups can be the initial sign of a life-threatening neurologic emergency. It underscores the need for high clinical suspicion of ZSH and overlapping anti-ganglioside antibody syndromes, even in the absence of a rash. Therefore, early and concurrent testing for VZV and anti-ganglioside antibodies is warranted to guide the timely intensive combined therapy, as this approach could be critical to altering the disease course.