Ramsay Hunt Syndrome Associated with Central Nervous System Involvement in an Adult

Abstract

Ramsay Hunt syndrome associated with varicella zoster virus reactivation affecting the central nervous system is rare. We describe a 55-year-old diabetic female who presented with gait ataxia, right peripheral facial palsy, and painful vesicular lesions involving her right ear. Later, she developed dysmetria, fluctuating diplopia, and dysarthria. Varicella zoster virus was detected in the cerebrospinal fluid by polymerase chain reaction. She was diagnosed with Ramsay Hunt syndrome associated with spread to the central nervous system. Her facial palsy completely resolved within 48 hours of treatment with intravenous acyclovir 10 mg/kg every 8 hours. However, cerebellar symptoms did not improve until a tapering course of steroid therapy was initiated.

Brainstem and Cerebellar Involvement in Ramsay Hunt Syndrome

“…Immunocompromised patients due to immunosuppressant agents, chemotherapy, or other etiologies have an increased risk of disseminated VZV infection including encephalitis [13]. Immunosuppressants impair leukocyte adhesion and migration into the CNS, potentially suppressing the natural defense against VZV encephalitis [1]. e patient, in this case, had recently completed a course of chemotherapy for squamous cell laryngeal cancer (4 months prior to presentation) which likely led to an immunocompromised state, predisposing him to the development of VZV reactivation.…”

“…Diagnosis in the cases of concomitant RHS and VZV encephalitis generally consists of the clinical features, brain MRI, and CSF analysis, all of which were used in this case to establish a diagnosis [1,15]. However, the sensitivity of these tests has not been well established in the literature.…”

“…Prognosis is controversial but seems to be related to the time span between onset and initiation of therapy. Facial paralysis recovery rates vary between studies but range from 10 to 75% recovery rate [1,16,17]. e wide variation in prognosis is likely due to the speed of treatment along with clinical status at the time of presentation.…”

“…Varicella zoster virus (VZV) is a human herpes virus that, during the primary infection, causes chickenpox [1]. After the primary infection, the virus lies dormant in the ganglion of the sensory neurons.…”

“…VZV involvement of the geniculate ganglion is known as Ramsay Hunt syndrome (RHS), characterized by ipsilateral facial paralysis, otic pain, and herpetic lesions of the external auditory canal and auricle [3]. To the best of our knowledge, there have been 3 prior cases of RHS complicated exclusively by cerebellitis [1,4,5] and 3 prior cases of RHS complicated exclusively by rhombencephalitis [6][7][8]. While isolated involvement of the brainstem, cerebellum, and spinal cord has been discussed in the literature, no reports to our knowledge have described concurrent brainstem and cerebellar involvement [9].…”