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.