Ramsay hunt syndrome: case report

¹Otorhinolaryngology Service, Military Hospital Mohamed V, Rabat, Morocco

^&Corresponding author
Imane Ouhbi, Otorhinolaryngology Service, Military Hospital Mohamed V, Rabat, Morocco

Introduction    

The Ramsay Hunt syndrome (RHS) was described for the first time by the neurologist James Ramsay Hunt in 1907. It is an unusual disease that includes a herpes zoster oticus and an ipsilateral peripheral facial paralysis. It is caused by reactivation of the Varicella-Zoster virus (VZV) at the geniculate ganglion. This report highlights the importance of a complete clinical examination and precocious treatment in management of the Ramsay-Hunt syndrome.

Patient and observation     

A 27-year-old man presented with intense right-sided ear pain for two days. The patient did not complain of headache, dizziness, tinnitus, or hearing impairment. There was no history of trauma. Physical examination revealed right facial muscles weakness and incomplete eye closure at effort, consistent with a grade IV at House-Brackmann (HB) scale. We also noted a right-sided Charles Bell´s phenomenon. (Figure 1). The otoscopy showed a vesicular rash at the right concha extended to the external auditory meatus, with a slight swelling of the auricular area (Figure 2). Audiometric evaluation showed an abolished right stapedial reflex and a normal pure tone audiometry. Neurological examination showed no other cranial nerve lesion. Ophthalmological examination showed no signs on corneal lesion. The patient was treated with intravenous steroids (prednisolone 1mg/kg per day) with antivirals (acyclovir 15mg/kg per day) and antalgics. The follow-up showed gratifying results with the disappearance of the earache and vesicular rash, as well as the improvement of facial paralysis, becoming a grade II HB. The one-year follow-up showed no relapse of the syndrome.

Discussion     

Ramsay-Hunt syndrome is an uncommon disease, with incidence ranged from 0.3 to 18% of acute atraumatic facial palsies, the most common cause being Bell´s palsy [1]. RHS is characterized by vesicular eruptions on the external ear and severe ear pain (herpes zoster oticus) with ipsilateral peripheric facial paralysis. It is caused by reactivation of latent Varicella Zoster Virus in the geniculate ganglion. The neuropathogenesis of RHS is, according to Grose et al., the contamination of the ganglion by the virus during chickenpox, via the sensory branches of the facial nerve located on the ear and tongue. Upon reactivation, VZV travels back along the sensory branches of the facial nerve, causing, by a bystander effect, the inflammation of the adjacent motor branches of the facial nerve, leading to facial palsy [2]. Histopathological studies revealed perivascular, perineural, and intraneural aggregation of round cells in the facial nerve in patients with RHS. The VIII cranial nerve can be affected by VZV as well, causing vestibulocochlear symptoms [3]. There are complicated anastomoses between cranial nerves including facial, trigeminal, glossopharyngeal, vagal, or cervical nerves, which enable herpetic lesions to progress further. While skin lesions lead to the diagnosis of herpes zoster oticus, various neurological disturbances can occur such as tinnitus, hearing loss, nausea and vomiting, vertigo, and nystagmus [4].

Early diagnosis of RHS is a crucial factor to improve nerve damage in Ramsay Hunt syndrome. Diagnosis is mainly based on the history, clinical findings, and neurological examination. Polymerase Chain Reaction assays may be useful to detect herpes zoster virus DNA in biological fluid [5]. Magnetic Resonance Imaging after injection with gadolinium, although usually unnecessary, can also contribute to the diagnosis by showing intense enhancement of the geniculate ganglion [6]. Combination treatment with anti-viral agents and steroids is recommended for the treatment of RHS. The parenteral dose of acyclovir is 15 mg/kg per day and the oral dose is 800 mg 4 times a day. Murakami et al. [7] compared 48 patients with oral acyclovir versus 32 patients with intravenous acyclovir and observed that there was no significant difference between the two routes, except for the advantage of reducing hospital time for the oral acyclovir. When compared with idiopathic facial palsy (Bell´s palsy), RHS seems to have a more severe paralysis at onset and is less likely to recover completely [8]. Ryu´s study compared 202 patients with RHS and 155 patients with Bell´s palsy. Both groups were treated with oral steroids and antiviral agents. Ryu concluded that RHS showed poorer prognosis for recovery from facial weakness compared with Bell´s palsy [9]. Murakami´s study [7] investigated the importance of an early treatment and established that the sooner treatment was initiated, the better was the recovery. Early diagnosis and treatment of Ramsay Hunt syndrome is therefore crucial as prognosis of cranial nerve damage depends on the time at which acyclovir-corticosteroid therapy is started [10].

Conclusion     

Ramsay-Hunt syndrome is a rare but easily diagnosed disease, based on clinical symptoms and examination. Treatment recommended is the combination of anti-viral agents and steroids. Prognosis is good, but any delay of the care could lead to sequelae. This report emphasizes how an early on treatment can offer remarkable results with a prompt and complete recovery.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

IO has written the article. All the authors have read and agreed to the final manuscript.

Figures     

Figure 1: vesicle rash on the right concha with a slight swelling of the pavilion

Figure 2: facial palsy of the right side with incomplete eye closure and Charles-Bell phenomenon

References