(Ramsay Hunt Syndrome)
Ramsay Hunt syndrome
Ramsay Hunt syndrome (herpes zoster facial paralysis) differs from Bellís palsy because it is associated with VZV (as shown by rising titers of
antibodies to VZV) and the presence of skin vesicles on the pinna, retroauricular area, face, or mouth. Compared with Bellís palsy, Ramsay Hunt
syndrome generally causes more severe symptoms and patients have a higher risk of developing complete nerve degeneration.10
Varicella, or chickenpox, is the manifestation of the primary infection by VZV (HSV, varicellae) in a nonimmune host. Herpes zoster is the
manifestation of this same virus in a partially immune host. Serologic and epidemiologic data strongly suggest that VZV represents the reactivation of
a latent virus rather than reinfection. After the primary infection the virus probably travels to the dorsal root to extramedullary CN ganglia where it
remains dormant until it is reactivated. Reactivation generally occurs during a period of decreased cell-mediated immunity. VZV is the second most
common cause of facial paralysis. The incidence of herpes zoster in patients with peripheral facial palsy is 4.5% to 9%.47 A Mayo Clinic study172
estimated the annual incidence of herpes zoster as 130 cases per 100,000. The incidence increased dramatically in patients older than age 60; 10%
of this population had identifiable risk factors for decreased cell-mediated immunity, including carcinoma, trauma, radiotherapy, or chemotherapy.
The increased incidence in the elderly is explained by an age-related decrease in cellular immune response to VZV.35
Compared with Bellís palsy, the severity of the paralysis is worse and the prognosis poorer in herpes zoster oticus. Peitersen163 reported full
recovery in only 22% of patients, and Devriese46 found complete recovery in only 16%. As in Bellís palsy, the recovery is in part predicted by the
severity of the paralysis. Complete recovery occurred in only 10% of patients after complete loss of facial function and in about 66% after
The timing of the appearance of the vestibular eruption may have prognostic significance. In most cases, eruption and paralysis occur
simultaneously. In approximately 25% of cases the eruption precedes the paralysis; these patients have a higher likelihood of recovery.47 Patients
with Ramsay Hunt syndrome also are more likely than patients with Bellís palsy to have associated CN symptoms, including hyperacusis, hearing
loss, and pain.
Severe ocular complications can occur with herpes zoster ophthalmicus. These complications include uveitis, keratoconjunctivitis, optic neuritis, and
glaucoma and are almost always associated with involvement of the ophthalmic division of the trigeminal nerve. Herpes zoster ophthalmicus may be
difficult to differentiate from the localized skin rash associated with HSV. Although both conditions may cause keratitis, differentiation between them
is extremely important because topical corticosteroids are used to manage herpes zoster but are contraindicated in HSV. Ophthalmologic
consultation for biomicroscopy, staining, cytology, and viral isolation studies may differentiate these two conditions.127 Adour7 stated that aside from
concerns about ophthalmic involvement, the development of skin vesicles before or after initiation of prednisone does not contraindicate
Management of patients with herpes zoster, including cephalic zoster, is systemic corticosteroids.177 A specific benefit of corticosteroid therapy is a
reduction of postherpetic neuralgia. The usefulness of corticosteroids in fostering the recovery of facial paralysis is controversial; however, early
institution of corticosteroids appears to relieve acute pain, reduce vertigo, and decrease the incidence of postherpetic neuralgia.
The antiviral agent acyclovir also has been recommended to treat herpes zoster facial paralysis.11,97 Acyclovir is a nucleotide analog that interferes
with herpes virus DNA polymerase and inhibits DNA replication. The drug is preferentially taken up by HSV-infected cells, making the drug nearly
nontoxic to noninfected cells. Early return of facial function after acyclovir management has been reported by some authors,11,97 but no beneficial
effects were reported by others.190 At the least, management with acyclovir appears to lessen pain and promote resolution of the vesicles.