DiscussionRamsay Hunt Syndrome: The Basics • Epidemiology: In one large case series, accounted for 12% of facial nerve paralysis3 • Presentation: Classic triad of otalgia, facial nerve palsy and vesicular eruption of the auricle and auditory canal1; see Fig. 1 for additional cutaneous and mucocutaneous manifestations • Pathophysiology: Caused by the reactivation of varicella zoster virus in the geniculate ganglion and often affects nerves in close proximity, such as CN VIII, by mechanisms of both VZV neuritis and inflammatory edema5, commonly causing vertigo, tinnitus and hearing loss2 (Fig. 2) • Diagnosis: Clinical alone; LP has no role in diagnosis, though CSF is abnormal in about 60% of patients with RHS6. • Treatment: Combination antiviral and steroid therapy; despite lack of evidence for their use in this syndrome when systematic reviews were undertaken8,9. Eye cares are an important adjunctive therapy.

• Prognosis: Recovery tends to be less favorable than Bell’s Palsy3

Case of Disseminated Varicella Zoster in Patient with AIDS Jessica Tischendorf, MD and Prabhav Kenkre, MD

Department of Medicine, University of Wisconsin Hospital and Clinics, Madison, WI

Table 1. Pertinent lab results.

Case Report

BackgroundVaricella zoster virus (VZV) infection has two principle manifestations: primary disease, causing “chickenpox” and reactivation of latent disease, known as herpes zoster (HZ) or “shingles”. The most common manifestation of HZ is a dermatomal rash with acute neuritis; however, disease can be complicated by ophthalmic, otic and in about 3% of cases, neurologic involvement4. Complicated disease is more common in immunocompromised patients. Here, we discuss a case of disseminated varicella zoster principally manifesting as Ramsay Hunt Syndrome (RHS) in a patient with Acquired Immunodeficiency Syndrome (AIDS).

Initial Presentation EM is a 57 year old man with AIDS chronically non-adherent to antiretroviral therapy who presented with two months of headache and subjective fever that was followed by right sided otalgia and facial droop two days prior to admission. On further questioning, he noted onset of a diffuse rash several weeks prior that was improving. Evaluation Exam: Edematous right pinna with purulent drainage in the conchal bowl and upper and lower right facial palsy. Diffuse erythematous follicular based papules, some with scarring and crusting over the entire body surface.

MRI: abnormal enhancement of geniculate ganglion and right facial nerve; nodular leptomeningeal enhancement concerning for disseminated infection or lymphoproliferative disorder.

Diagnosis: His cranial nerve findings and geniculate ganglion enhancement were consistent with RHS, and diffuse rash suggested disseminated zoster. Flow cytometry was performed on CSF, which was normal, excluding CNS lymphoma as a cause for his symptoms, abnormal imaging and CSF pleocytosis.

In addition to identification of RHS, EM had evidence of otitis externa, likely a bacterial superinfection of initial rash.

References1. Yawn BP, Saddier P, Wollan P, St. Sauver JL, Kurland MJ, Sy LS. A population-based study of the incidence and complication

rates of herpes zoster before zoster vaccine introduction. Mayo Clin Proc. 2007;82(11):1341-9.2. Robillard RB. Ramsay Hunt facial paralysis: Clinical analyses of 185 cases. Otolaryngology and head and neck surgery

1986;95(3):292-7.3. Hunt JR. On herpetic inflammation of the geniculate ganglion: A new syndrome and its complications. J Nerv Ment Dis

1907;34:73.4. Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. Journal of Neurology, Neurosurgery and Psychiatry 2001;71(2):149-545. Devaleenal DB, Ahilasamy N, Solomon S, Kumarsamy N. Ramsay hunt syndrome in a person with HIV disease. Indian J

Otolaryngol. Head Neck Surg 2008;60:171-3.6. Haanpää M, et al. CSF and MRI findings in patients with acute herpes zoster. Neurology 1998;51:1405-11.7. Uscategui T, Doree C, Chamberlain IJ, Burton MJ. Corticosteroids as adjuvant to antiviral treatment in Ramsay Hunt syndrome

(herpes zoster oticus with facial palsy) in adults. Cochrane Database of Systematic Reviews 2008, Issue 3. Art. No.: CD006852. DOI: 10.1002/14651858.CD006852.pub2.

8. Uscategui T, Doree C, Chamberlain IJ, Burton MJ. Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database of Systematic Reviews 2008, Issue 4. Art. No.: CD006851. DOI: 10.1002/14651858.CD006851.pub2.

9. Vafai A and Berger M. Zoster in patients infected with HIV: A review. Am J Med Sci 2001;321(6):372-80.10.Hung et al. Herpes zoster in HIV-1-infected patients in the era of highly active antiretroviral therapy: a prospective observational

study. International Journal of STD & AIDS 2005;16(10):673-6.11.Blank LJ, Polydefkis MJ, Moore RD, Gebo KA. Herpes zoster among persons living with HIV in the current antiretroviral therapy

era. J Acqui Immune Defic Syndr 2012;61(2):203-7.

Herpes Zoster and HIV Infection • Age-adjusted relative risk of HZ in HIV patients was 16.9 in 1992 study10

• Recurrent infections occur in 10-27% of HIV infected patients, compared to 1-4% of immunocompetent patients11

• Those with lower CD4 count are at higher risk and antiretroviral therapy appears to be protective12

• Complicated disease, particularly ocular and neurologic, is more common (see Table 2)

Hospital Course EM was initiated on IV antibiotics for his otitis externa and treated initially with IV acyclovir and corticosteroids for RHS. Steroids were discontinued after several days due to challenging glycemic control. He was discharged to home on culture directed antibiotics for otitis externa and valacyclovir to complete three week course for disseminated zoster.

Site Study ResultBlood HIV-1 RNA 275 copies/mL

Absolute CD4 count

45 / uLCSF Cell count 51 nucleated cells (79% lymphocytes),

550 RBCs, glucose 117, protein 60Varicella zoster PCR

Detected

Table 1. Pertinent Labs

Table 2. Selected neurologic complications of HZ and associated manifestations. Note these are often not accompanied by rash.

Encephalitis Altered mental status, focal neurologic findings, seizures

Meningitis Fever, headache, stiff neck, photophobia

Myelitis Paresis of extremities, incontinence, sensory deficits

Acute ascending polyradiculitisProgressive, symmetric muscle weakness with decreased or absent deep tendon reflexes

Hemiplegia May complicate cranial neuropathy, due to midbrain involvement

Peripheral motor neuropathy Occurs in distribution of dermatomal rash

Cervical zoster Arm weakness, may cause diaphragmatic paralysis

Herpes zoster ophthalmicus Involvement of CN V, can result in vision loss

Optic neuritis May result in permanent visual field loss

Herpes zoster oticus Otalgia, ear rash, facial nerve palsy

Acute retinal necrosis Blurring of vision, rapid vision loss

Post-herpetic neuralgia Dermatomal distribution pain that persists

Zoster sine herpete Dermatomal pain in the absence of rash

VasculopathyIschemic or hemorrhagic stroke, spinal cord infarction, aneurysm

Figure 1. Exam features of Ramsay Hunt Syndrome. Image courtesy of: C J Sweeney, 2001

Figure 2. Anatomic relationship of geniculate ganglion and facial nerve to surrounding structures. Image courtesy of Duke Medicine, web.duke.edu

Take Home Points• Ramsay Hunt Syndrome, a rare manifestation of HZ, is characterized by otalgia, facial nerve palsy and vesicular eruption of the ear • HZ can be complicated by neurologic involvement; these cases are more common in immunocompromised patients • Patients with neurologic HZ disease should be monitored very closely and treated with antivirals given the potentially devastating consequences; eye cares are also important