Quiz Answer

Ramsay Hunt syndrome—Also called herpes zoster oticus, Ramsay Hunt syndrome is the second most common cause of atraumatic acute peripheral facial nerve palsy in adults.¹ This syndrome represents a triad of symptoms: unilateral facial nerve palsy, ear pain, and eruption of vesicular herpetic lesions.^1,2 Unilateral dysfunction of the lower motor neuron of cranial nerve VII (peripheral facial palsy) is the common manifestation, which involves the upper and lower face (Figure 1), including the inability to raise the eyebrow (Figure 2) and an absence of nasolabial fold on the affected side. The finding of a vesicular rash on the concha of our patient's auricle (Figure 3) confirmed the diagnosis of Ramsay Hunt syndrome.

Ramsay Hunt, chief neurologist at Cornell University School of Medicine at the turn of the twentieth century, described an association between peripheral facial palsy and reactivation of the herpes zoster virus (also known as varicella-zoster virus), with inflammation in the geniculate ganglion, where the virus lies dormant after primary infection.² The pain and paralysis are more severe and the prognosis is poorer in Ramsay Hunt syndrome than in herpes simplex virus type 1 (HSV-1)–related acute peripheral facial nerve palsy.¹ Early diagnosis of Ramsay Hunt syndrome and treatment with acyclovir and prednisone within 72 hours of facial palsy onset improve recovery rate and reduce the severity of the sequelae.³

Acute otitis media can present with peripheral facial nerve palsy when an ear infection spreads to include the adjacent bony structure and nerves, but it occurs without a vesicular rash.

Lyme disease is the most common cause of facial palsy in children in endemic regions.⁴ Diagnostic clues for Lyme disease include a history of tick bite, antecedent rash, Lyme-endemic area, and Lyme disease season, none of which applied to our patient.⁴

Facial palsy associated with HSV-1 is the most common cause of cranial nerve palsy in adults.⁵ The HSV-1 reactivation site, however, is in a more distal part of the facial nerve compared with the herpes zoster virus reactivation sites, and involvement of the lacrimal and salivary glands and taste distortion will therefore be spared in HSV-1 reactivation.⁶

References

1. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71:149-154.
2. Hunt JR. On herpetic inflammations of the geniculate ganglion: a new syndrome and its complications. J Nerv Ment Dis. 1907;34:73-96.
3. Murakami S, Hato N, Horiuchi J, et al. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol. 1997;41:353-357.
4. Cook SP, Macartney KK, Rose CD, et al. Lyme disease and seventh nerve paralysis in children. Am J Otolaryngol. 1997;18:320-323.
5. Peitersen E. Bell's palsy: the spontaneous course of 2,500 peripheral facial nerve palsies of different etiologies. Acta Otolaryngol Suppl. 2002;549:4-30.
6. Grose C, Bonthius D, Afifi AK. Chickenpox and the geniculate ganglion: facial nerve palsy, Ramsay Hunt syndrome and acyclovir treatment. Pediatr Infect Dis J. 2002;21:615-617.