Ramsay Hunt syndrome is a rare condition characterised by herpetic lesions and peripheral facial nerve palsy. The condition is difficult to diagnose as it can mimic several other neurological illnesses. This article provides diagnosis guidance and treatment management for such self-limiting conditions.  

What is Ramsay Hunt Syndrome (RHS)?

Ramsay Hunt syndrome (Herpes Zoster Oticus) is a clinical diagnosis that occurs as a late complication of varicella-zoster virus infection. The condition leads to a herpetic inflammation of the geniculate ganglion which disrupts the motor functions of the seventh cranial nerve. 

Based on the pathological process taking place in the geniculate ganglion, the condition can be categorised into four types:

• The disease affects the sensory portion of the seventh cranial nerve
• Disease involving the sensory and motor divisions of the seventh cranial nerve
• Disease involving the sensory and motor divisions of the seventh cranial nerve with auditory symptoms
• Disease involving the sensory and motor divisions of the seventh cranial nerve with auditory and vestibular symptoms

Causes

Varicella-zoster virus (HHV-3; human alphaherpesvirus 3) is the causative agent of Ramsay Hunt syndrome. Initial infection with the virus results in chickenpox. But upon clearing of chickenpox, the virus remains dormant in the cranial nerve or dorsal root ganglia, which may reactivate during stress or immunocompromised conditions. Reactivation of the virus can cause Ramsay Hunt syndrome.

The different stages during the development of Ramsay Hunt syndrome are enumerated below:

• Initial infection - Initial infection with the varicella-zoster virus causes chickenpox - disseminated vesicular rash accompanied by fever.
• Acute phase - During the acute phase of infection, the varicella-zoster virus spreads through respiratory droplets.
• Dormant phase - Once viremia and exanthem resolve, varicella-zoster virus remains dormant in cranial nerves and dorsal root ganglia.
• Reactivation of the virus - Reactivation of the virus occurs during periods of physiological stress or immunocompromise. In Ramsay Hunt syndrome, the varicella-zoster virus reactivates in the geniculate ganglion and affects the seventh cranial nerve, causing facial paralysis and rash.

Diagnosis

Diagnosis of Ramsay Hunt syndrome is entirely clinical relying on the patient’s medical history, clinical findings, and neurological examination. Patients usually present with the classical triad of

• Ipsilateral facial paralysis
• Otalgia
• Vesicles on the auricle

However, in some patients, the early course of the disease may only involve pain and facial paralysis or rash may be absent.

Usually, the condition involves prodromal signs of pain, fever, and fatigue, for 1 to 3 days. This is followed by the onset of facial paralysis and widespread rash (exanthem). Facial palsy takes 1 to 3 days to reach its nadir, with rapid disease progression often resulting in more severe paralysis.

The erythematous vesicular rash is painful and can spread across the auricle, auditory canal, scalp cheek, tongue, or palate. The rash initiates as erythematous papules, which turn into vesicles, that ultimately rupture and crust within a week. The rash usually appears after the palsy.

Several other symptoms can be seen during the acute phase of the syndrome. This includes:

• Hyperacusis
• Dysgeusia
• Nasal obstruction
• Epiphora
• Xerophthalmia
• Drooling
• Dysarthria
• Smile asymmetry
• Vertigo, tinnitus, hearing loss
• Hoarseness, dysphagia
• Facial numbness

Laboratory tests to confirm the clinical diagnosis involves evaluating virus antibodies. Polymerase chain reaction (PCR) assays can help detect virus DNA in exudates from the geniculate zone of the ear. PCR can also be performed on tears or blood mononuclear cells. However, performing PCR on exudates from the geniculate zone of the ear is more sensitive.

Analysis of cerebrospinal fluid and brain magnetic resonance imaging has limited diagnostic or prognostic value for Ramsay Hunt syndrome.

Treatment

The treatment is targeted at reducing pain, decreasing the disease duration, and preventing complications.

1. The treatment for Ramsay Hunt syndrome involves the use of antiviral agents and steroids.
2. A combination of acyclovir and prednisone in the early stages of the condition is found to be effective. Early treatment can also prevent hearing loss.
3. Acyclovir and famciclovir help in improving acute pain, and aid recovery of lesions from herpes zoster. They also prevent postherpetic neuralgia.
4. The anti-inflammatory action of steroids helps reduce inflammation and oedema in the nerves. This also aids in the accelerated recovery of the affected nerves.
5. Acyclovir–prednisone is reported to prevent nerve degeneration and enhance the recovery rate of facial nerve palsy.

Disclaimer- The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of M3 India.