Ramsay Hunt syndrome in a patient with human immunodeficiency virus infection.

Ramsay Hunt syndrome (RHS) is a rare, severe complication of varicella-zoster virus reactivation in the geniculate ganglion, characterized by hearing loss, pain, and vesicles in the ear or mouth along with ipsilateral facial palsy. Although it is rare, it is more commonly found with immunodeficiency conditions. We report a case of a 35-year-old human immunodeficiency virus positive male, having CD4+ count of 336/μl, who presented with RHS and had vertigo, painful vesicular eruptions on the right ear and unilateral sensorineural hearing loss. He was treated immediately with valacyclovir and prednisolone, and the complete recovery was achieved at 6 months after the onset.

INTRODUCTION

Ramsay Hunt syndrome (RHS), also called herpes zoster oticus, is a rare, severe complication of varicella-zoster virus (VZV) reactivation in the geniculate ganglion. The classic triad consists of otalgia, vesicles in the auditory canal and ipsilateral facial paralysis.[1] It is the second most common cause of atraumatic peripheral facial paralysis.[2] In mild cases, it may or may not be associated with any neurological signs, but in its severest form, it is associated with sensorineural hearing loss, disturbance of vestibular function and even viral encephalitis. Without treatment, full recovery of the facial paralysis occurs in only about 20% of cases; the prognosis is better if treatment is started within 72 h.[34] We report a case of RHS in a 35-year-old human immunodeficiency virus (HIV) positive male, having CD4+ count of 336/μl, who was treated immediately with valacyclovir and prednisolone leading to a complete recovery.

CASE REPORT

A 35-year-old male, a known case of HIV infection for the last 4 years, presented to us with complaints of right earache and pain over the right side of the face for the last 9 days which was followed by painful erythematous eruption over the right ear and inability to close the right eye. The patient took some oral analgesics and a topical antibiotic from a local practitioner for the same, but there was no relief in symptoms. The patient also gave a history of tinnitus and vertigo, but there was no history of ear discharge, nasal discharge, headache, seizure, or weakness of the limbs. The patient was HIV positive with CD4+ count of 336/μl and had been on antiretroviral therapy with zidovudine, lamivudine, and nevirapine for the last 2 years.

On examination, the right ear had a few vesicles along with crusted erosions on the concha of the right pinna [Figure 1]. There was no discharge, and the tympanic membrane was intact. Bell's phenomenon was present on the right side along with loss of nasolabial fold [Figure 2].

Figure 1

Herpetic lesions in the right ear

Figure 2

Facial nerve palsy with Bell's phenomenon and loss of nasolabial fold

The patient was diagnosed as a case of RHS and started on tablet valacyclovir 1 g three times a day for 7 days, tapering dose of tablet prednisolone, acetaminophen, physiotherapy for facial nerve palsy and ocular care. After 10 days, the vesicles and crusting over the right ear had cleared, and there was mild improvement in Bell's palsy and vertigo. The patient was advised to continue physiotherapy and was advised regular follow-up. After 6 months of physiotherapy, the facial nerve function had improved considerably, and the patient was able to completely close his eyes, and there was no facial asymmetry.

DISCUSSION

The Center for Disease Control estimates that 32% of people in the USA will have herpes zoster (HZ) during their lifetime with the risk increasing with the progression of age.[5] RHS has a much lower incidence, presenting in only 0.2% of all HZ cases.[6] The risk of developing HZ depends on the decline in cell-mediated immunity. Herpes zoster is one of the AIDS-defining illnesses. Other factors are old age, an immunosuppressive therapy used in malignancies, transplantation, and autoimmune disorders. HIV-infected patients have twenty times more risk of developing HZ than their age-matched seronegative persons and about 30% of HIV persons develop zoster at least once within 12 years after the diagnosis of HIV.[7]

HZ is also a common manifestation in immune reconstitution inflammatory syndrome (IRIS) and usually manifests after 2–3 months of initiation of highly active antiretroviral therapy (HAART), but late onset HZ has also been reported even after 3 years of HAART initiation.[8] Our case may also be a manifestation of IRIS as the patient had been on HAART for the last 2 years.

RHS is diagnosed clinically and is based on unilateral facial weakness plus vesicular lesions in the ipsilateral ear, hard palate, or anterior two-third of the tongue. Facial weakness is identified by facial drooping, a widened palpebral fissure, and decreased smile on the affected side. Otalgia or vertigo completes the triad picture of RHS.[1]

The use of CSF analysis or MRI adds no additional diagnostic value. The gold standard for diagnosing VZV reactivation is polymerase chain reaction of skin, saliva, or middle ear fluid samples but this is rarely done clinically.

Treatment of RHS follows the general treatment goal of VZV infection. In the normal host, the primary goal of treatment is to reduce acute pain and postherpetic neuralgia. Several controlled studies verified that high-dose oral acyclovir of valacyclovir within the first 72 h speeds resolution of the lesions and seems to reduce the risk of prolonged pain.[9] For the severely immunocompromised host, intravenous acyclovir has been shown to prevent disease progression. For mildly to moderately immunocompromised persons, however, oral valacyclovir or famciclovir might be acceptable alternatives to intravenous treatment. The management of IRIS associated HZ is similar and monosegmental zoster can be treated with oral acyclovir, valacyclovir, or famciclovir for 7–10 days but complicated, multisegmental or facial zoster should be treated intravenously for 10–14 days. As our patient had a monosegmental zoster, we treated him with oral valacyclovir for 7 days with good response.

RCTs have shown that adjunct corticosteroids resulted in quicker healing of the rash and decreased incidence and severity of pain during HZ. Yet, there was no reduction in the incidence or severity of postherpetic neuralgia in these studies.[10] Steroids reduce the inflammation in the facial canal. Early administration of acyclovir-prednisone has proved to reduce nerve degeneration by nerve excitability testing. Hearing recovery is also better in these patients.[4] Apart from these, eye patches, taping the eye closed, artificial tears and oral analgesics are also advised to prevent ocular complications.

If untreated, the course of RHS can be complicated with ocular complaints like corneal ulcers if eyelid closure is impaired, permanent facial paralysis, postherpetic neuralgia, or long-term ipsilateral hearing loss. The prognosis of IRIS associated HZ is similar and tends to resolve completely without complications with treatment, but chronic courses with ulcerating forms and involvement of other organs may be seen as may be pneumonia or central nervous system involvement.

RHS is a rare disease that can present with vague symptoms and atypical presentations. A high index of suspicion and close follow-up is essential as early intervention with antivirals and corticosteroids has shown to significantly improve outcomes in these patients.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.