Ramsay Hunt syndrome management: A multimodal approach

INTRODUCTION

Ramsay Hunt Syndrome (RHS), also known as geniculate zoster, geniculate neuralgia, nervus intermediate neuralgia, or herpes zoster oticus,[^1,2] is an infectious disease that arises in the geniculate ganglion of cranial nerve V and XII can affect both immunocompromised and immunocompetent patients. J. Ramsay Hunt first described it in 1907 caused by the varicella-zoster virus (VZV), which now represents the second most common cause of atraumatic peripheral facial palsy.[³] The main clinical symptoms (Classical Triad) include ipsilateral facial palsy, inner ear dysfunction, periauricular pain, and herpetiform vesicles on the pinna.[⁴] The RHS affects the elderly population above 55 years with 20% high females predilection and among children (<12 years) with immunocompromised status accounts for <10% of the diagnosed cases with an incidence of 2.7/100,000.[^5,6] Although various clinical etiologies have been associated with this syndrome, including direct anesthesia of facial nerve, neurotoxicity of local anesthetic solution, viral reactivation, demyelination, edema, vasospasm, ascending infection, and trauma, viral reactivation is proposed to be the most likely one.[⁷] There are also reported literature cases of reactivation of VZV following dental procedures such as

periodontal surgeries and fitting of a crown, treatment of carious teeth, extraction of a tooth,[⁷] root canal therapy, and adjustment of dentures in immune-compromised patients.[^1,5] Other underlying systemic illnesses, particularly hematologic malignancies such as leukemia and lymphoma maybe associated with VZV reactivation.

CASE REPORT

A 55-year-old male patient was referred from the ear, nose, and throat department of Chettinad Health City to the Oral Medicine and Radiology Department for a dental opinion. With a chief complaint of multiple eruptions in his face, scalp, mouth along with facial deviation present for 4 days. On probing more into the history, the patient revealed continuous, radiating pain to his right side of the face, ear, and oral cavity for the past 2 days and further reported experiencing low-grade fever accompanied with facial disfigurement. The patient gave a history of experiencing an episode of chickenpox during his childhood. The patient’s medical history revealed that he had diabetes with poorly controlled glycemic status for the past 5 years.

On extraoral examination, multiple vesicles were noted over the external auditory meatus, multiple macules with crustations over the right side of the scalp, face, zygoma further extended to the lower lip [Figure 1a and b]. There was the absence of wrinkles on the right forehead, the inability of the patient to close his right eye completely [Figure 2], and mild drooping of the right commissure of the lip. Intraoral examination revealed multiple vesicular eruptions with a clear fluid discharge on the right buccal mucosa [Figure 3]. Hence, the provisional diagnosis of RHS and differential diagnosis of Bells palsy, neurosarcoidosis, and Parry Romberg syndrome were given. Laboratory investigation with a hemogram, serum glucose levels, serum electrolytes, and a peripheral blood smear was done. Except for the increase in serum glucose level, other test results were within the normal limits. The patient was subjected to a Tzanck smear test, which revealed multinucleated giant cells, thus based on the above findings, a final diagnosis of RHS was derived. The patient was prescribed acyclovir 800 mg five times a day, Tablet acetaminophen 500 mg q6 h tablet prednisolone 20 mg od and vitamin supplement twice daily for 7 days and were asked to report of a review. The patient was told to rest and stay hydrated. After 2 months, during which the complete regression of lesions was noted, he returned for follow-up. Later, the patient was under physiotherapy and transcutaneous electrical nerve stimulation (TENS) therapy to regain muscular activity post-facial paralysis; the degree of facial paralysis was Grade III, using the House-Brackmann Scale [Table 1].

Figure 1. (a) Crustations were evident along the right side of zygoma further extended to the lower lip; (b) scalp and face involving tragus of ear

Figure 2. Inability to close eye completely

Figure 3. Intra oral vesicles seen in buccal mucosa

Table 1. House Brackmann Scale for grading facial palsy

DISCUSSION

RHS affects the facial nerve and the geniculate ganglion in the auditory canal, where it remains dormant in the sensory dorsal root ganglion cells. The primary infection causes an induction of the VZV-specific memory T-cells. This cell immunity declines over time below a theoretical “zoster threshold” leads to an increased risk of herpes zoster infection, and the average period of immunity against varicella following infection is 20 years. The pathogenesis for the reactivation of VZV is unknown. Any factor affecting the cell-mediated immunity may play an essential role in the reactivation of VZV.[⁸] The most common prodromal symptoms include fever, pain, vomiting, hearing loss, and fatigue for the first 3 days, followed by the signs such as nystagmus, tinnitus, change in taste along with anesthesia of two-thirds of the tongue, and presence of vesicles in the tympanic membrane accompanied with rash finally leads to pustules that dry and crust were sometimes leaving extraoral scars. When the same lesion extends, intra-orally presence of vesicles unilaterally involving the oral mucosa is seen, which may or may not affect the palate and tongue. Laboratory confirmation is based on repeated complement fixation tests showing increased antibody titer. The detection of VZV is confirmed in saliva, tears, middle ear fluid, and blood mononuclear cell with the help of a PCR test. The recurrence ranging from 2.3% to 8.0% overall and age, stress, immunocompromised status, and immunosuppressive drugs are the known factors for virus reactivation.[⁹] In our case, males were affected; other factors such as age and uncontrolled diabetes made them vulnerable to the virus’s reactivation.

CONCLUSION

RHS is a rare clinical entity that, when diagnosed early, can decrease complications and the morbidity associated with it. Hence, dentists should be aware of the syndrome and examine for the signs and symptoms of intraoral herpetic lesions whenever there are signs of facial palsy. A multi-disciplinary approach among oral physicians, medical physicians, and physiotherapists is needed for prompt management and treatment to minimize postherpetic complications and ensure complete recovery.