An 83 y.o. male presents with pain in his face

What is wrong?

Our patient had Ramsay Hunt syndrome with vesicles over the ear and also involving the anterior 2/3 of the tongue.  This is an acute facial nerve infection caused by herpes zoster and associated with a vesicular rash of the ear canal and/or mucous membranes of the oropharynx. This was first described in 1907 by James Ramsay Hunt and he attributed it to infection by the herpesvirus 3(varicella-zoster or VZV).

Our pt also had involvement of the tongue.

It can present with facial palsy, but other cranial nerves may be involved including CN VIII, IX, V and VI. Tinnitus, deafness, vertigo, nystagmus or ataxia may result. VZV has caused retinitis in immunosuppressed patients. It is treated with intravitreal ganciclovir.

The primary infection with varicella is associated with chickenpox.  After chickenpox, VZV remains in the neurons of nerves and reactivation can result in a localized rash, known as herpes zoster.  When the virus is latent in the geniculate ganglion of CN VII reactivation results in Ramsay Hunt syndrome. It can occur without a rash and VZV has been detected by PCR in the tear fluid of patients with Bell’s palsy.

another example of Ramsay Hunt

Rare complications may include encephalitis, CN XII involvement or meningitis. The diagnosis is usually made clinically although VZV antigen can be detected by direct immunofluorescence assay  with a sensitivity of 90% and specificity close to 99%.

Since VZV vaccine is a live attenuated virus, herpes zoster may occur in  vaccine recipients.  Our  pt was treated for VZV and did well.  Thanks to Dr. Wilson for the case.

Goldani LX, Ferreira de Silva L, Dora J.  Ramsay Hunt syndrome in patients infected with human immunodeficiency virus.  Clin Exp Dermatol. 2009 ;34(8):e552-4.

Verm A, Scott I, Davis J. Necrotizing herpetic retinopathy associated with Ramsay Hunt syndrome. Arch Ophthalmol. 2002;120(7):989-990.

Uebe B, Sauerbrei A, Burdach, et al. Herpes zoster by reactivated vaccine varicella zoster virus in a helathy child. Eur J Pediatr. 2002;161:442-444.