Management of Ramsay Hunt Syndrome in an Acute Palliative Care Setting

INTRODUCTION

James Ramsay Hunt was an American physician and scientist in 1907, who described a series of cases of a syndrome of lower motor neuron facial paralysis, otalgia, and auricular vesicles (herpes zoster oticus), which he hypothesized was due to infection of the geniculate ganglion, accompanied by other neurological disturbances like tinnitus, hyperacusis, hearing loss, vomiting, lacrimation, vertigo, loss of taste on the anterior tongue, and nystagmus, depending on the location of infection and inflammation.[12] The generally accepted cause of Ramsay-Hunt syndrome is the reactivation of the Varicella Zoster Virus (VZV). The seventh cranial nerve is typically involved due to inflammation of the geniculate ganglion. The VZV infection may also affect cranial nerves VIII, IX, V, and VI due to the proximity of their pathway alignments in the temporal bone.[3] Hunt himself classified the disease into four subgroups according to the extent of the pathological processes taking place in the geniculate ganglion.[4] The symptoms are unrepresentative since the herpetic lesions are not always present (zoster sine herpete) and might mimic several other neurologic illnesses.[5]

Here, we have described a case report of a 63-year-old male who developed Ramsay Hunt syndrome in an acute palliative care setting.

Presenting concerns

A 63-year-old man known case of carcinoma of gall bladder with liver metastases, post surgery and chemotherapy with no scope for further disease modifying treatment, was referred to palliative care unit for best supportive care. He presented to Palliative Medicine outpatient with 3 days history of ipsilateral facial pain of neuropathic character, otalgia, diffuse vesciculo-papular rash over ophthalmic and maxillary divisions of left trigeminal nerve distribution of face and ear with associated secondary bacterial infection, and unilateral facial edema. On central nervous system examination, paresthesia was noted over left side of face with mild facial asymmetry. He was diagnosed to have Herpes Zoster (HZ) with superadded bacterial infection and was treated with tablet Valacyclovir 500 mg four times a day, Acyclovir cream for local application, Acyclovir eye ointment for prophylactic treatment of Herpetic Keratitis, low dose of Prednisolone, oral Amoxicillin and Clindamycin for 7 days, and Pregabalin 150 mg per day. After completion of 7 days of treatment, the rash and vesicles completely resolved and pain and symptoms improved [Figure 1].

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Figure 1

Herpes Zoster infection refore and after treatment

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DISCUSSION

Ramsay Hunt syndrome (RHS), also called Herpes Zoste Oticus, is a rare but severe complication of VZV reactivation. The classic triad consists of otalgia, vesicles in the auditory canal, and ipsilateral facial paralysis.[6] Taste perception, hearing (tinnitus, hyperacusis), and lacrimation are affected in selected patients. Multiple cranial nerves are involved with frequent involvement of cranial nerves V, IX, and X.[678] The RHS diagnosis is purely clinical; but in some cases, a blood test for VZV antibodies may be useful. When other cranial nerves are affected, magnetic resonance imaging may be necessary to exclude intracerebral pathology. Various studies show expected likelihood of full recovery of facial muscle control to be 27.3% to 67.3% if the treatment is started within 72 hours of symptoms onset The data on recovery rate for individual cranial nerve involvements are not well established.[910] However, if the treatment is delayed for more than 72 hours, the chances of complete recovery drop to about 50%. Post-herpetic neuralgia has been reported in up to 50% patients.[11] The main prognostic factor that determines outcomes is the severity of the initial symptoms.[12] Possible complications includes: Corneal abrasions and ulcers, secondary bacterial infections (cellulitis), postherpetic neuralgia, permanent facial paralysis, long term ipsilateral hearing loss, and tinnitus. Occasionally, the virus may spread to other nerves, or even to the brain and spinal cord, causing confusion, drowsiness (lethargy), headaches, motor weakness, neuropathic pain.[13] Antivirals and corticosteroids are the current mainstay of treatment although more randomized controlled trials are needed. Acyclovir, Valacyclovir, and Famcyclovir are the drugs approved by Food and Drug Administration (FDA) as first-line therapies for HZ. These have been shown to reduce the duration of acute HZ symptoms and associated long-term nerve damage. Steroids by their potent anti-inflammatory effect has been argued to enhance recovery in RHS by reducing the inflammation and edema of the facial nerve, thus reducing damage.[14] though Cochrane review showed no evidence for the use of corticosteroids in Ramsay Hunt syndrome.[15] Vaccination against VZV is an interesting new development that might reduce the incidence of VZV associated disease altogether.[1617]

In our case study, we found that timely use of antivirals, corticosteroids along with antibiotics for superadded bacterial infection improved HZ infection and prevented further complications.

CONCLUSION

• HZ with superadded bacterial infection involving head and neck region poses a potentially life-threatening complication and can compromise both length and quality of life

• Early identification and prompt intervention with antivirals and corticosteroids has shown to improve outcomes significantly and prevent complications.