1st Case Conference of the Year

Sheryl Kho, M.D.

PGY 3

July 22, 2009

Chief Complaint

17 yo AA boy

“I can’t move my face.”

History of Present Illness

6 days PTA- Woke up unable to move R side of face

No fever, rash, HA, constitutional sxs, trauma

Went to ED- Dx: Bell’s PalsyRx: Acyclovir + Prednisone

History of Present Illness

3 days PTA- persistent facial paralysis with pins and needle sensation

+ HA-frontal, +photophobia, +phonophobia

+vomiting+pain in R ear

+hyperacusis+tingling sensation on his

tongue

Past Surgical Hx

S/p I&D Pilonidal abscess- 2 wks ago

Rx: Augmentin x 7 days

Past Medical Hx

Varicella @ 5yoOccasional cold sores on upper lipIUTDPPD negative- 1 year ago

Adolescent Hx (HEADSSS)

Lived in the BronxNo travelDenies tick bites, animal exposureJunior in HS, worked as a lifeguard

during summerDenies sexual activityDenies use of illicit drugs or alcohol

Physical Exam

VS: T 38.2C, HR 110bpm, RR 20/min, BP 127/75, SaO2 100%

AAO, c/o frontal HAHEENT: NCAT, PERRLA, +crusted lesion in R ear canal,

TM intact B/L, +2 crusted sores on R upper lip, MMM, clear OP, supple neck, no Brudzinski, no Kernig,+nuchal rigidity

Lungs: CTA B/L, no WRR, no retractionsHeart: RRR, normal S1/S2, no mrg

Physical Exam

Abd: +BS, soft, NT, ND, no HSMExt: FROMx4, no cyanosis, no edema, 2+pulses, good

cap refillNeuro: AAO, unable to close R eye, +drooping R side of

face with flattening of ipsilateral nasolabial fold, unable to wrinkle R side of forehead, unable to puff out R cheek, +asymmetric smile

Differential Diagnosis? Infectious

AOM, COM, cholesteatoma Meningitis Bell’s Palsy Lyme Disease Viral Syndrome Mumps Herpes zoster oticus

Neuro/CNS Mass/Tumor ie. Acoustic neuroma,

glomus tumor,facial ner neuroma Stroke Bleed

Others Vertigo Trigeminal Neuralgia TMJ Disorders Dental Pain Persistent Idiopathic Facial Pain

Toxins Tetanus

Iatrogenic Surgical Embolization Nerve block

Idiopathic Autoimmune syndrome Myasthenia gravis Multiple sclerosis Sarcoidosis Amyloidosis

Systemic DM Alcoholic neuropathy Hyperthyroidism Pregnancy

Trauma Birth trauma Temporal bone fracture Facial trauma

Diagnostic Workup?

CBC, BCxCMPCSF, CSF CxLyme titersCT scanWound Cx

Diagnostic Workup

CBC- 4.4>13<172,000 N65 L19 M16CMP- normal TP: 7.5 Alb: 4UA-normalCT Brain- negativeLyme titers negative IgM, IgG

Diagnostic Workup

Lumbar PunctureClear CSFWBC: 32, L91, RBC: 25CSF protein 45, CSF glucose 47Gm stain: no organisms, no cellsCSF culture pendingCSF PCR HSV negativeCSF Viral Cx negative

Management

Started on Ceftriaxone and AcyclovirPrednisone taperHA and neck stiffness resolved w/in 24hrCTX d/c’d once CSF cx negative

Further Diagnostic Workup

VZV cultured from R ear canal lesionsHIV ELISA: positiveCD4 count: 28 AIDSHIV Viral load: 414,555

Further Management

IV Acyclovir continuedPrednisone PO x 7 daysBactrim and Zithromax- prophylaxis for M.

avium, Pneumocystis jiroveciEfavirenz, Emtricitabine and Tenofovir

started 6 wks after acute illness

What happened later?

6 months after start HAARTViral load: undetectableCD4 count: 220Continues with sensitivity to sound and R facial

paralysis

Ramsay Hunt Syndrome

Ramsay Hunt Syndrome

1907: described by James Ramsay Hunt“Geniculate neuralgia”, “nervus

intermedius neuralgia”Facial paralysisInner ear dysfunctionPeriauricular painHerpetiform vesicles of the pinna (herpes

zoster oticus)

Ramsay Hunt Syndrome

Primary infection with VZV (HHV 3)Latent in the geniculate ganglion of CN VIIVZV reactivation, zoster: decline in cell

mediated immunity ie. HIV

Pathophysiology of RHS

Geniculate ganglion of CN VIIPetrous portion of the temporal bone lies

the ear apparatus (inner ear)CNVII courses through the inner and

middle earInflammation causes facial paresis,

vertigo, otalgia, hyperacusis

Anatomy of the Facial Nerve

Anatomy of the Facial Nerve

Anatomy of the Facial Nerve

Epidemiology of RHS

RareComplete recovery rate <50%Self limitingMorbidity: facial weakness

History Taking

Pain deep in the earVertigoTinnitusFacial paresis

Rash, blisters, herpetic lesions

Physical Examination

PainPeripheral facial nerve paralysis with

herpetic lesionsAnt 2/3 of tongueSoft palateext auditory canalPinna

Ipsilateral hearing loss, balance problemsNeuro exam

Physical Examination

Diagnostic Workup

CBC with differentialESRSerum electrolytesViral Studies

Serologic testsVZV PCR on tear samplesViral cxs

Imaging studiesMRI, CT scan

AudiometryCSF studies (controversial)

RHS in HIV Patients

Normal children: 0.74/1000>70% in HIV, CA 7-20x greater risk than children with

leukemiaRecurrence: 53% (1.7-5%)Persistence of skin lesions: 14%

Bell’s PalsyIdiopathic facial paralysis (IFP)Virally mediated, exact mechanism unknownAffects CN VIIReactivation of HSV60-75% of acute facial palsiesSudden paresis of facial muscles on one side,

absence of CNS dse <48hrs20-30 pxs/100,000Paresis in the morning, worsens thru the day

Otalgia, facial pain, hyperacusis, decreased tears, NO SKIN LESIONS

Herpes Zoster Ophthalmicus

Primary infection: chickenpoxLatent in the trigeminal ganglionAffects the first division of CN VPE:

Treatment of RHS

Acyclovir + prednisoneRemains controversial

Thank you…