Background: Ramsay Hunt Syndrome with cerebellar encephalitis is rare in adult. Case Report: We describe a 55-year-old diabetic female who presented with truncal ataxia, right peripheral facial palsy and right ear pain associated with erythematous vesicular lesions in her external auditory canal. Later, she developed dysmetria, fluctuating diplopia and dysarthria. No facial lesions were identified and lesions were limited to the external auditory canal. Cerebral spinal fluid tested positive for varicella zoster virus polymerase chain reaction. She was diagnosed with Ramsay Hunt Syndrome with spread to the central nervous system and treated with acyclovir intravenous therapy (10 mg/kg every 8 hours). Her facial palsy completely resolved within 48 hours of acyclovir treatment, however, vesicular lesions, imbalance and cerebellar symptoms remained; a tapering course of high dose prednisone was then added.

Discussion: Prognosis for facial palsy is poor in Ramsay Hunt Syndrome: Only 10% of patients will have complete resolution of their facial palsy. Improvement of facial palsy may be a good marker for response to treatment. Conclusion: Varicella zoster virus reactivation affecting the central nervous system in adults is rare. Knowledge of Ramsay Hunt syndrome with brainstem and/or cerebellar involvement is important for diagnosis and for consideration of antiviral and prednisone treatment.