Rhombencephalitis associated with varicella-zoster virus masquerading as Guillain-Barré syndrome

Although rare, rhombencephalitis or inflammation of the brainstem and cerebellum has significantly associated morbidity and mortality.

We describe the case of a 64-year-old previously healthy, immunocompetent man who presented with acute bulbar symptoms (difficulty swallowing, change in phonation, and drooling). His symptoms progressed in severity and he ultimately required intubation due to declining lung function and inability to manage his secretions. Clinical examination revealed bulbar dysfunction without involvement of other cranial nerves. A lumbar puncture revealed albuminocytological dissociation: elevated protein (9.8 g/L), nonerythroid cell count (13 cells/µL) with predominantly lymphocytic count. He received a five-day course of intravenous immunoglobulin for presumed Guillain-Barré syndrome. Initial magnetic resonance imaging (MRI) showed mild microangiopathic changes in the brain with no parenchymal, leptomeningeal, or cranial nerve enhancement. Cerebrospinal fluid (CSF) analysis was positive for varicella zoster virus (VZV). He was treated with intravenous Acyclovir 10 mg/kg three times daily for 14 days with no initial improvement and underwent a tracheostomy. Subsequent MRI was consistent with rhombencephalitis.

VZV rhombencephalitis with cranial neuropathies represents a rare and potentially fatal condition. Early recognition and investigation with lumbar puncture and imaging are critical for establishing the diagnosis and initiating treatment.